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Ding P, Zhang L, Pei S, Qu Z, Kong X, Wang Z, Wang J, Fang Y. Nomogram construction for overall survival in breast angiosarcoma based on clinicopathological features: a population-based cohort study. Discov Oncol 2025; 16:351. [PMID: 40100579 PMCID: PMC11920551 DOI: 10.1007/s12672-025-02118-w] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/13/2024] [Accepted: 03/11/2025] [Indexed: 03/20/2025] Open
Abstract
BACKGROUND Breast angiosarcoma (BAS) is a rare, aggressive malignancy with a poor prognosis, often challenging to assess due to its unique biology. This study aimed to develop a nomogram to predict 3- and 5-year overall survival (OS) for BAS patients using key clinicopathological factors. METHODS Data from 450 BAS patients diagnosed between 2000 and 2021 were extracted from SEER database. Key variables, including age, tumor size, tumor grade, and distant metastasis status, were identified through univariate and multivariate Cox regression analyses. These factors were incorporated into a nomogram for OS prediction. The model was validated internally and externally using the concordance index (C-index), calibration curves, and decision curve analysis (DCA) to assess its predictive accuracy and clinical utility. RESULTS The nomogram demonstrated good predictive accuracy, with a C-index of 0.68 in the training set and 0.72 in the test set. ROC analysis indicated strong short-term predictive power, with AUC values of 0.81 and 0.75 for 1-year survival in the training and test sets, respectively, though predictive performance declined over time. DCA showed substantial clinical benefit for 12-month predictions, which diminished over longer time frames. The model effectively distinguished high-risk BAS patients and provided individualized survival estimates, supporting its potential use in clinical decision-making. CONCLUSION This study presents the first BAS nomogram for OS prediction, showing robust short-term accuracy. The long-term utility is limited by heterogeneity and sample size, highlighting the need for external validation to confirm generalizability and clinical applicability.
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Affiliation(s)
- Peikai Ding
- Department of Breast Surgical Oncology, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100021, China
| | - Luxiao Zhang
- Department of Breast Surgical Oncology, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100021, China
| | - Shengbin Pei
- Department of Breast Surgical Oncology, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100021, China
| | - Zheng Qu
- Department of Breast Surgical Oncology, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100021, China
| | - Xiangyi Kong
- Department of Breast Surgical Oncology, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100021, China
| | - Zhongzhao Wang
- Department of Breast Surgical Oncology, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100021, China
| | - Jing Wang
- Department of Breast Surgical Oncology, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100021, China.
| | - Yi Fang
- Department of Breast Surgical Oncology, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100021, China.
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Almushayqih M, Chami R, Malik A, Chavhan GB. Imaging features of pediatric angiosarcomas: clinical, pathologic, and radiological review. Pediatr Radiol 2024; 54:1873-1883. [PMID: 39223383 DOI: 10.1007/s00247-024-06041-0] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/25/2024] [Revised: 08/16/2024] [Accepted: 08/21/2024] [Indexed: 09/04/2024]
Abstract
BACKGROUND Angiosarcomas are rare malignant vascular tumors and there is scarcity of data on their imaging features. OBJECTIVE To review and illustrate the imaging, clinical, and pathologic features of angiosarcoma in children. MATERIALS AND METHODS A list of pathologically proven angiosarcoma seen between Nov 1992 and Jan 2023 was obtained from a pathology database and picture archiving and communication system. Those with pre-treatment imaging available on our PACS were included in the study. Imaging studies were reviewed by two readers in consensus. RESULTS A total of six children (two males and four females; median age of 8.8 years; range 2.9 years to 15.5 years) had angiosarcoma during the study period. Organ of origin included breast (n = 2), liver (n = 2), spleen (n = 1), and paranasal sinuses (n = 1). The patient with splenic angiosarcoma had Li-Fraumeni syndrome. Five patients had a single lesion while one had multifocal lesions. The tumors were large with a median diameter of 12.9 cm (range 2.7 cm to 24 cm). Most tumors were heterogeneous on T2-weighted imaging with hemorrhage and necrosis and showed heterogeneous enhancement. Three had well-defined borders and three had infiltrative borders. None of the tumors showed calcifications. Two tumors in the liver showed gradual non-centripetal progressive diffuse enhancement on dynamic imaging. One patient had metastases at presentation and four patients subsequently developed metastases on follow-up. Five patients underwent surgical resection and chemotherapy; one patient with a liver lesion underwent arterial embolization followed by liver transplant. Three patients died at the last follow-up. CONCLUSION The imaging features of angiosarcomas are nonspecific, but the tumors are large heterogeneously enhancing masses with hemorrhage and necrosis. Hepatic angiosarcomas may show non-centripetal progressive and heterogeneous enhancement on dynamic imaging.
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Affiliation(s)
- Musab Almushayqih
- Department of Diagnostic and Interventional Radiology, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, M5G 1X8, Canada
- Department of Medical Imaging, University of Toronto, Toronto, Ontario, Canada
| | - Rose Chami
- Division of Pathology, The Hospital for Sick Children, Toronto, Ontario, Canada
- Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, Canada
| | - Aleena Malik
- Department of Diagnostic and Interventional Radiology, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, M5G 1X8, Canada
| | - Govind B Chavhan
- Department of Diagnostic and Interventional Radiology, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, M5G 1X8, Canada.
- Department of Medical Imaging, University of Toronto, Toronto, Ontario, Canada.
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Selby LV, Clark E, Chen JL, Tinoco G, Beane JD, Pollock RE, Liebner D, Grignol VP. Use of neoadjuvant paclitaxel in breast angiosarcoma-Impact on surgical resection and response rates. J Surg Oncol 2024; 130:579-585. [PMID: 39016208 DOI: 10.1002/jso.27772] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/03/2024] [Accepted: 06/11/2024] [Indexed: 07/18/2024]
Abstract
OBJECTIVE Breast angiosarcoma is a tumor that can arise as a primary breast tumor or in association with prior radiation therapy. Angiosarcomas are uniquely sensitive to paclitaxel. This study evaluated the impact neoadjuvant paclitaxel (NAC) therapy has on surgical outcomes, tumor recurrence, and survival in breast angiosarcomas. METHODS Patients with angiosarcoma of the breast, either primary or radiation-associated, were identified from a prospective institutional database. Patients receiving NAC were compared to those treated with upfront surgery. Clinical and pathological variables were compared using Student's t-test or Fisher's exact test, differences in survival were calculated using Kaplan-Meier methods. RESULTS Twenty-four patients with angiosarcoma of the breast were identified, 10 with primary angiosarcoma and 14 with radiation-associated angiosarcoma. Twelve patients received NAC, 6 of each with primary angiosarcoma or radiation-associated angiosarcoma. Of these 12 patients, 11 had a margin negative resection (91%) of which, nine had a complete pathological response on surgical pathology. Of the 12 surgery-first patients, four (n = 4/12, 33%) had positive surgical margins, two of the four underwent reoperation. With a median follow-up of 16 months, four NAC patients had recurrence (33%) compared to six patients in the surgery-first group (58%) (p = 0.41). While not statistically significant, NAC patients had a 33% less risk of recurring compared to surgery-first patients ([hazard ratio =0.67 (95% confidence interval 0.16-2.72; p = 0.6]). CONCLUSION NAC for breast angiosarcoma may be associated with high rates of complete pathological response and margin-negative resection. However, this did not impact overall survival. Future prospective control studies and longer follow-up periods are warranted to understand the impact on recurrence and survival.
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Affiliation(s)
- Luke V Selby
- Department of Surgery, Division of Surgical Oncology, The Ohio State University Wexner Medical Center, The Ohio State University, Columbus, Ohio, USA
- Department of Surgery, Division of Colorectal and Oncologic Surgery, University of Kansas School of Medicine, Kansas City, Kansas, USA
| | - Emma Clark
- The Ohio State University School of Medicine, Ohio State University, Columbus, Ohio, USA
- Department of Surgery, Wright State University, Dayton, Ohio, USA
| | - James L Chen
- Department of Medicine, Division of Medical Oncology, The Ohio State University Wexner Medical Center, Columbus, Ohio, USA
| | - Gabriel Tinoco
- Department of Medicine, Division of Medical Oncology, The Ohio State University Wexner Medical Center, Columbus, Ohio, USA
| | - Joal D Beane
- Department of Surgery, Division of Surgical Oncology, The Ohio State University Wexner Medical Center, The Ohio State University, Columbus, Ohio, USA
| | - Raphael E Pollock
- Department of Surgery, Division of Surgical Oncology, The Ohio State University Wexner Medical Center, The Ohio State University, Columbus, Ohio, USA
| | - David Liebner
- Department of Medicine, Division of Medical Oncology, The Ohio State University Wexner Medical Center, Columbus, Ohio, USA
| | - Valerie P Grignol
- Department of Surgery, Division of Surgical Oncology, The Ohio State University Wexner Medical Center, The Ohio State University, Columbus, Ohio, USA
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Balbino M, Masino F, Erriquez D, Carpagnano FA, Montatore M, Fascia G, Sciacqua A, Guglielmi G. A rare case report of breast sarcoma and synchronous thymoma in a 60-year-old woman. Radiol Case Rep 2024; 19:3216-3221. [PMID: 38800081 PMCID: PMC11126876 DOI: 10.1016/j.radcr.2024.04.077] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/13/2024] [Revised: 04/22/2024] [Accepted: 04/24/2024] [Indexed: 05/29/2024] Open
Abstract
This case report aims to describe the clinical presentation, imaging findings, histopathological features and therapeutic approach of a patient diagnosed with coexisting breast sarcoma and thymoma. A 64-year-old woman presented with a palpable lump in her left breast, and subsequent imaging studies (ultrasound, mammography, and MRI) revealed breast sarcoma, a rare and aggressive subtype of breast cancer. At the same time, the MRI revealed the presence of a thymoma. A multidisciplinary approach involving surgeon, breast specialist and oncologist is essential for optimal management and favorable outcomes in patients with this rare diagnosis.
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Affiliation(s)
- Marina Balbino
- Department of Clinical and Experimental Medicine, Foggia University School of Medicine, Viale L. Pinto 1, 71121, Foggia (FG), Italy
| | - Federica Masino
- Department of Clinical and Experimental Medicine, Foggia University School of Medicine, Viale L. Pinto 1, 71121, Foggia (FG), Italy
| | - Daniela Erriquez
- Breast Unit, “Dimiccoli” Hospital, Viale Ippocrate 15, 70051, Barletta (BT), Italy
| | | | - Manuela Montatore
- Department of Clinical and Experimental Medicine, Foggia University School of Medicine, Viale L. Pinto 1, 71121, Foggia (FG), Italy
| | - Giacomo Fascia
- Department of Clinical and Experimental Medicine, Foggia University School of Medicine, Viale L. Pinto 1, 71121, Foggia (FG), Italy
| | - Alessio Sciacqua
- Department of Clinical and Experimental Medicine, Foggia University School of Medicine, Viale L. Pinto 1, 71121, Foggia (FG), Italy
| | - Giuseppe Guglielmi
- Department of Clinical and Experimental Medicine, Foggia University School of Medicine, Viale L. Pinto 1, 71121, Foggia (FG), Italy
- Radiology Unit, “Dimiccoli” Hospital, Viale Ippocrate 15, 70051, Barletta (BT), Italy
- Radiology Unit, “IRCCS Casa Sollievo della Sofferenza” Hospital, Viale Cappuccini 1, 71013 San Giovanni Rotondo (FG), Italy
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5
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Palassini E, Baldi GG, Sulfaro S, Barisella M, Bianchi G, Campanacci D, Fiore M, Gambarotti M, Gennaro M, Morosi C, Navarria F, Palmerini E, Sangalli C, Sbaraglia M, Trama A, Asaftei S, Badalamenti G, Bertulli R, Bertuzzi AF, Biagini R, Bonadonna A, Brunello A, Callegaro D, Cananzi F, Cianchetti M, Collini P, Comandini D, Curcio A, D'Ambrosio L, De Pas T, Dei Tos AP, Ferraresi V, Ferrari A, Franchi A, Frezza AM, Fumagalli E, Ghilli M, Greto D, Grignani G, Guida M, Ibrahim T, Krengli M, Luksch R, Marrari A, Mastore M, Merlini A, Milano GM, Navarria P, Pantaleo MA, Parafioriti A, Pellegrini I, Pennacchioli E, Rastrelli M, Setola E, Tafuto S, Turano S, Valeri S, Vincenzi B, Vitolo V, Ivanescu A, Paloschi F, Casali PG, Gronchi A, Stacchiotti S. Clinical recommendations for treatment of localized angiosarcoma: A consensus paper by the Italian Sarcoma Group. Cancer Treat Rev 2024; 126:102722. [PMID: 38604052 DOI: 10.1016/j.ctrv.2024.102722] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/22/2024] [Revised: 03/17/2024] [Accepted: 03/26/2024] [Indexed: 04/13/2024]
Abstract
Angiosarcoma (AS) represents a rare and aggressive vascular sarcoma, posing distinct challenges in clinical management compared to other sarcomas. While the current European Society of Medical Oncology (ESMO) clinical practice guidelines for sarcoma treatment are applicable to AS, its unique aggressiveness and diverse tumor presentations necessitate dedicated and detailed clinical recommendations, which are currently lacking. Notably, considerations regarding surgical extent, radiation therapy (RT), and neoadjuvant/adjuvant chemotherapy vary significantly in localized disease, depending on each different site of onset. Indeed, AS are one of the sarcoma types most sensitive to cytotoxic chemotherapy. Despite this, uncertainties persist regarding optimal management across different clinical presentations, highlighting the need for further investigation through clinical trials. The Italian Sarcoma Group (ISG) organized a consensus meeting on April 1st, 2023, in Castel San Pietro, Italy, bringing together Italian sarcoma experts from several disciplines and patient representatives from "Sofia nel Cuore Onlus" and the ISG patient advocacy working group. The objective was to develop specific clinical recommendations for managing localized AS within the existing framework of sarcoma clinical practice guidelines, accounting for potential practice variations among ISG institutions. The aim was to try to standardize and harmonize clinical practices, or at least highlight the open questions in the local management of the disease, to define the best evidence-based practice for the optimal approach of localized AS and generate the recommendations presented herein.
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Affiliation(s)
- Elena Palassini
- Medical Oncology Unit 2, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy.
| | | | | | - Marta Barisella
- Department of Pathology, ASST Fatebenefratelli Sacco, Milano, Italy
| | - Giuseppe Bianchi
- Department of Surgery, Istituto Ortopedico Rizzoli, Bologna, Italy
| | - Domenico Campanacci
- Department of Surgery, Azienda Ospedaliera Universitaria Careggi, Firenze, Italy
| | - Marco Fiore
- Department of Surgery, Sarcoma Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy
| | - Marco Gambarotti
- Department of Pathology, Istituto Ortopedico Rizzoli, Bologna, Italy
| | - Massimiliano Gennaro
- Department of Surgery, Breast Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy
| | - Carlo Morosi
- Department of Radiology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy
| | - Federico Navarria
- Department of Radiation Oncology, IRCCS Centro di Riferimento Oncologico di Aviano, Aviano, Pordenone, Italy
| | - Emanuela Palmerini
- Department of Medical Oncology, Istituto Ortopedico Rizzoli, Bologna, Italy
| | - Claudia Sangalli
- Department of Radiation Therapy, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy
| | - Marta Sbaraglia
- Department of Pathology, Università di Padova, Padova, Italy
| | - Annalisa Trama
- Department of Edidemiology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy
| | - Sebastian Asaftei
- Department of Pediatric Oncology, Ospedale Infantile Regina Margherita , Torino
| | - Giuseppe Badalamenti
- Department of Medical Oncology, Azienda Universitaria Policlinico Giaccone, Palermo, Italy
| | - Rossella Bertulli
- Medical Oncology Unit 2, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy
| | - Alexia Francesca Bertuzzi
- Department of Medical Oncology, Humanitas Cancer Center, IRCCS Humanitas Research Hospital, Rozzano, Milano, Italy
| | - Roberto Biagini
- Department of Oncological Orthopedics, IRCCS Istituto Nazionale Tumori Regina Elena - Istituti Fisioterapici Ospitalieri, Roma, Italy
| | - Angela Bonadonna
- Department of Medical Oncology, IRCCS Centro di Riferimento Oncologico di Aviano, Aviano, Pordenone, Italy
| | - Antonella Brunello
- Department of Medical Oncology, Istituto Oncologico Veneto IOV IRCCS, Padova, Italy
| | - Dario Callegaro
- Department of Surgery, Sarcoma Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy
| | - Ferdinando Cananzi
- Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, Milano, Italy; Sarcoma, Melanoma and Rare Tumors Surgery Unit, Humanitas Cancer Center, Department of Surgery, IRCCS Humanitas Research Hospital, Rozzano, Milano, Italy
| | | | - Paola Collini
- Department of Pathology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy
| | - Danila Comandini
- Department of Medical Oncology, IRCCS Ospedale Policlinico San Martino, Genova, Italy
| | - Annalisa Curcio
- Department of Surgery, Ospedale Morgagni e Pierantoni, Forlì, Italy
| | - Lorenzo D'Ambrosio
- Department of Medical Oncology, Ospedale S. Luigi, Orbassano, Torino, Italy
| | - Tommaso De Pas
- Department of Medical Oncology, Humanitas Gavazzeni, Bergamo, Italy
| | | | - Virginia Ferraresi
- Sarcomas and Rare Tumors Departmental Unit, IRCCS Istituto Nazionale Tumori Regina Elena - Istituti Fisioterapici Ospitalieri, Roma, Italy
| | - Andrea Ferrari
- Department of Pediatric Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy
| | - Alessandro Franchi
- Department of Pathology, Azienda Ospedaliera Universitaria Pisana, Pisa, Italy
| | - Anna Maria Frezza
- Medical Oncology Unit 2, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy
| | - Elena Fumagalli
- Medical Oncology Unit 2, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy
| | - Matteo Ghilli
- Breast Centre, Department of Oncology, Azienda Ospedaliera Universitaria Pisana, Pisa, Italy
| | - Daniela Greto
- Department of Radiation Therapy, Azienda Ospedaliera Universitaria Careggi, Firenze, Italy
| | - Giovanni Grignani
- Department of Medical Oncology, Azienda Ospedaliera Univerisitaria Città della Salute e della Scienza, Torino, Italy
| | - Michele Guida
- Department of Medical Oncology, IRCCS Istituto Tumori di Bari Giovanni Paolo II, Bari, Italy
| | - Toni Ibrahim
- Department of Medical Oncology, Istituto Ortopedico Rizzoli, Bologna, Italy
| | - Marco Krengli
- Department of Radiation Therapy, Istituto Oncologico Veneto IOV IRCCS, Padova, Italy
| | - Roberto Luksch
- Department of Pediatric Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy
| | - Andrea Marrari
- Department of Medical Oncology, Istituto Ortopedico Rizzoli, Bologna, Italy
| | | | - Alessandra Merlini
- Department of Medical Oncology, Ospedale S. Luigi, Orbassano, Torino, Italy
| | | | - Piera Navarria
- Department of Radiation Therapy, IRCCS Humanitas Research Hospital, Rozzano, Milano, Italy
| | - Maria Abbondanza Pantaleo
- Department of Medical Oncology, IRCCS Azienda Ospedaliero-Universitaria di Bologna', University of Bologna, Bologna, Italy
| | | | - Ilaria Pellegrini
- Medical Oncology Unit 2, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy
| | | | - Marco Rastrelli
- Department of Surgical Oncology, Istituto Oncologico Veneto IOV IRCCS, Padova, Italy; Department of Surgery, Oncology and Gastroenterology (DISCOG), Università di Padova, Padova, Italy
| | - Elisabetta Setola
- Department of Medical Oncology, Istituto Europeo Oncologia, Milano, Italy
| | - Salvatore Tafuto
- Department of Medical Oncology, Fondazione IRCCS Istituto Nazionale Tumori G. Pascale, Napoli, Italy
| | - Salvatore Turano
- Department of Medical Oncology, Azienda Ospedaliera S.S. Annunziata, Cosenza, Italy
| | - Sergio Valeri
- Department of Surgery, Università Campus Bio-Medico, Roma, Italy
| | - Bruno Vincenzi
- Department of Medical Oncology, Università Campus Bio-Medico, Roma, Italy
| | - Viviana Vitolo
- Department of Radiation Therapy, Centro Nazionale di Adroterapia Oncologica, Fondazione CNAO, Pavia, Italy
| | | | | | - Paolo Giovanni Casali
- Medical Oncology Unit 2, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy
| | - Alessandro Gronchi
- Department of Surgery, Sarcoma Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy
| | - Silvia Stacchiotti
- Medical Oncology Unit 2, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy
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Sebastian J, T. V M, V L, Sreekumar P. Primary Angiosarcoma of Breast: a Case Series of Rare Disease. Indian J Surg Oncol 2024; 15:113-116. [PMID: 38511047 PMCID: PMC10948628 DOI: 10.1007/s13193-023-01814-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/06/2023] [Accepted: 08/24/2023] [Indexed: 03/22/2024] Open
Abstract
Primary angiosarcoma of breast (PAB) is a very rare disease and it accounts for only 0.05% of all malignant breast tumors. We present the clinical radiological and pathological features, treatment given, and outcome of this rare disease in our institute in a period of 5 years, aiming at contributing to the knowledge of prognostic factors of this rare disease. We report a case of PAB treated at our hospital along with 2 more cases of PAB we found on retrospective analysis. The basic criterion for inclusion in the study was the presence of histopathologically confirmed primary angiosarcoma of the breast: graded into low (G1, G2) and high (G3) grades. Surgeons must be aware about its high systemic metastatic potential, atypical presentation, and diffuse nature of infiltration so that it will be diagnosed early, and undergo a margin negative excision with reconstruction after a proper metastatic evaluation.
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Affiliation(s)
- Jino Sebastian
- Department of Surgical Oncology, Kottayam Medical College, 3Rd Floor Casualty Building, Medical College, Kottayam, Kerala State India
| | - Murali T. V
- Department of Surgical Oncology, Kottayam Medical College, 3Rd Floor Casualty Building, Medical College, Kottayam, Kerala State India
| | - Letha V
- Department of Pathology, Kottayam Medical College, Kottayam, Kerala State India
| | - Parvathy Sreekumar
- Department of Surgical Oncology, Kottayam Medical College, 3Rd Floor Casualty Building, Medical College, Kottayam, Kerala State India
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7
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An R, Men XJ, Ni XH, Wang WT, Wang CL. Angiosarcoma of the breast: A review. Heliyon 2024; 10:e24413. [PMID: 38318005 PMCID: PMC10839862 DOI: 10.1016/j.heliyon.2024.e24413] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/08/2023] [Revised: 12/20/2023] [Accepted: 01/08/2024] [Indexed: 02/07/2024] Open
Abstract
Breast angiosarcoma is a rare and highly aggressive malignancy with a poor prognosis. It can occur spontaneously or be associated with factors such as radiation therapy or chronic lymphedema. The etiology and pathogenesis of this disease are still unclear, the clinical symptoms and imaging findings lack specificity, and the pathological morphology is diverse, which is easy to be confused with other diseases. There is no clear guideline for surgical treatment. Although the optimal surgical approach remains unclear, the ultimate goal is surgical excision with optimal margins, which remains the primary method of treatment. In clinical practice, the choice of the surgical approach should be made by considering the tumor size, pathological type, and patient preferences. In clinical practice, the selection of surgical methods should be carried out with comprehensive consideration of tumor size, pathological types and patients' wishes. There is no clear consensus on whether radiotherapy and chemotherapy should be carried out after surgery, and its optimal program and efficacy are uncertain. This article reviews the etiology, clinical manifestations, pathological features, imaging findings, treatment, prognosis and other aspects of breast angiosarcoma, so as to strengthen clinicians' overall understanding of this disease and avoid missed diagnosis and misdiagnosis.
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Affiliation(s)
- Ran An
- School of Clinical Medicine, Shandong Second Medical University, Weifang 261041, Shandong Province, China
- Department of Thyroid and Breast Surgery, Weifang People's Hospital, Weifang 261041, Shandong Province, China
| | - Xiao-Juan Men
- Department of Thyroid and Breast Surgery, Weifang People's Hospital, Weifang 261041, Shandong Province, China
| | - Xi-Hao Ni
- School of Clinical Medicine, Shandong Second Medical University, Weifang 261041, Shandong Province, China
| | - Wei-Tao Wang
- School of Clinical Medicine, Shandong Second Medical University, Weifang 261041, Shandong Province, China
| | - Chang-Liang Wang
- Department of Thyroid and Breast Surgery, Weifang People's Hospital, Weifang 261041, Shandong Province, China
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8
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Bishr MK, Banks J, Abdelaziz MS, Badawi M, Crane PW, Donigiewicz UJ, Elkorety M, Girgis M, Humphreys A, Isherwood J, Kahan J, Keelan S, Lindqvist EK, Nixon I, Sackey H, Sars C, Soliman H, Touqan N, Remoundos DD, Ahmed M. Multidisciplinary Management of Phyllodes Tumours and Breast Sarcoma: A Cross-sectional Survey of Clinical Practice across the UK and Ireland. Clin Oncol (R Coll Radiol) 2024; 36:e31-e39. [PMID: 38294995 DOI: 10.1016/j.clon.2023.10.050] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/11/2023] [Revised: 09/26/2023] [Accepted: 10/17/2023] [Indexed: 02/02/2024]
Abstract
AIMS Phyllodes tumours and breast sarcomas are uncommon tumours and their rarity poses significant challenges in diagnosis and management. This cross-sectional study was conducted to evaluate the multidisciplinary clinical practice for these tumours across the UK and Ireland, with the aim of identifying gaps in knowledge and providing direction for establishing national guidelines. MATERIALS AND METHODS An international survey was adapted and circulated to breast and/or sarcoma surgeons and oncologists in the UK and Ireland through national organisations. Multidisciplinary team (MDT) responses were analysed anonymously. RESULTS Twenty-eight MDTs participated in this study, predominately from high-volume units (85.5%). Although only 43% of the surveyed units were part of a trust that holds a sarcoma MDT, 68% of units managed malignant phyllodes and angiosarcoma, whereas 64.5% managed soft-tissue sarcoma of the breast. Across all subtypes, axillary surgery was recommended by 14-21% of the MDTs and the most recommended resection margins for breast surgery were 'no tumour on ink' in benign phyllodes (39%) and 10 mm in the remaining subtypes (25-29%). Immediate breast reconstruction was supported by 11-18% of MDTs for breast sarcoma subtypes, whereas 36% and 32% advocated this approach in benign and borderline phyllodes tumours, respectively. Adjuvant radiotherapy and chemotherapy were recommended by up to 29% and 11% of the MDTs, respectively. CONCLUSION The results of this study demonstrate a wide variation in clinical practice across the surveyed MDTs. As only 28 MDTs participated in our study, with under-representation from low-volume units, our results might be an underestimation of the variability in practice across the UK and Ireland. This multi-institutional study sheds light on controversial aspects in the management of phyllodes tumours and breast sarcoma, identifies the need for national guidelines to inform best practice, and calls for the centralisation of the management of breast sarcoma within specialist centres.
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Affiliation(s)
- M K Bishr
- The Royal Marsden NHS Foundation Trust, London, UK.
| | - J Banks
- The Royal Devon University Healthcare NHS Foundation Trust, Exeter, UK
| | - M S Abdelaziz
- National Cancer Institute, Cairo University, Cairo, Egypt; University Hospitals Plymouth NHS Trust, Plymouth, UK
| | - M Badawi
- East Sussex Healthcare Trust, Conquest Hospital, St Leonards-on-sea, UK
| | - P W Crane
- Queen Elizabeth II Hospital, Welwyn Garden City, UK
| | | | - M Elkorety
- West Hertfordshire Teaching Hospitals NHS Trust, Watford, UK
| | - M Girgis
- West Suffolk NHS Hospital, Bury St Edmunds, UK
| | - A Humphreys
- Northumbria Healthcare NHS Foundation Trust, Newcastle Upon Tyne, UK
| | - J Isherwood
- Milton Keynes University Hospital NHS Foundation Trust, Milton Keynes, UK
| | - J Kahan
- Velindre Cancer Centre, Cardiff, UK
| | - S Keelan
- Royal College of Surgeons in Ireland (RCSI) University of Medicine and Health Sciences, Dublin, Ireland
| | - E K Lindqvist
- Department of Clinical Science and Education, Stockholm South General Hospital, Karolinska Institutet, Stockholm, Sweden; Department of Surgery, Stockholm South General Hospital, Stockholm, Sweden
| | - I Nixon
- Management Science, Business School, Strathclyde University, Glasgow, UK; The Beatson West of Scotland Cancer Centre, Glasgow, UK
| | - H Sackey
- Division of Cancer, Department of Breast, Endocrine Tumors and Sarcoma, Karolinska University Hospital Stockholm, Stockholm, Sweden; Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden
| | - C Sars
- Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden
| | - H Soliman
- Kings' College Hospital, Orpington, UK
| | - N Touqan
- Manchester University NHS Foundation Trust, North Manchester General Hospital, Manchester, UK
| | - D D Remoundos
- Oxford University Hospitals NHS Foundation Trust, Oxford, UK
| | - M Ahmed
- University College London Hospitals NHS Foundation Trust, London, UK
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9
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Matsuda R, Saimura M, Anan K, Katsuyama K, Tahara Y, Kurata K, Horioka K, Nakamoto M, Koga K, Mitsuyama S, Sato E, Wakamatsu S, Tamiya S. Multiple angiosarcomas of both breasts: a case report. Surg Case Rep 2023; 9:205. [PMID: 38015377 PMCID: PMC10684844 DOI: 10.1186/s40792-023-01782-w] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/21/2023] [Accepted: 11/13/2023] [Indexed: 11/29/2023] Open
Abstract
BACKGROUND Primary angiosarcomas of the breast are rare and highly aggressive. We herein report a rare case of multiple angiosarcomas detected concurrently in both breasts. CASE PRESENTATION A 49-year-old woman visited a doctor after noticing a lump in her right breast. At that time, mammography and ultrasonography revealed no abnormal findings in either breast. She was referred to our hospital 5 months later, because screening mammography had revealed a focal asymmetric density in her right breast. Ultrasonography showed ill-defined hyper- and hypo-echoic lesions in both breasts. Magnetic resonance imaging disclosed five heterogeneously enhanced masses (5.8 cm in maximum diameter) in the right breast and six enhanced masses (approximately 1-3 cm in diameter) in the left breast. Histological examination of core needle biopsies revealed proliferation of irregularly shaped vascular channels lined by atypical endothelial cells throughout the adipose tissue and lobules of the breasts, leading to a diagnosis of well-differentiated angiosarcoma. The lesions were assumed to be primary angiosarcomas, because she had neither a history of breast surgery nor of radiation therapy. She underwent bilateral mastectomies and postoperative chest wall irradiation. Computed tomography 11 weeks after the surgery revealed multiple, small, subcutaneous nodules in the chest wall that were suspected of being angiosarcoma metastases. We started chemotherapy (weekly paclitaxel 80 mg/m2), which achieved shrinkage of these nodules within 2 months. CONCLUSIONS Early diagnosis, immediate initiation of local and systemic therapies, and intensive follow-up are important in improving the prognosis of angiosarcomas.
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Affiliation(s)
- Ryota Matsuda
- Department of Surgery, Kitakyushu Municipal Medical Center, 2-1-1 Bashaku, Kokurakita-Ward, Kitakyushu, Fukuoka, 802-0077, Japan.
| | - Michiyo Saimura
- Department of Surgery, Kitakyushu Municipal Medical Center, 2-1-1 Bashaku, Kokurakita-Ward, Kitakyushu, Fukuoka, 802-0077, Japan
| | - Keisei Anan
- Department of Surgery, Kitakyushu Municipal Medical Center, 2-1-1 Bashaku, Kokurakita-Ward, Kitakyushu, Fukuoka, 802-0077, Japan
| | - Kento Katsuyama
- Department of Surgery, Kitakyushu Municipal Medical Center, 2-1-1 Bashaku, Kokurakita-Ward, Kitakyushu, Fukuoka, 802-0077, Japan
| | - Yuki Tahara
- Department of Surgery, Kitakyushu Municipal Medical Center, 2-1-1 Bashaku, Kokurakita-Ward, Kitakyushu, Fukuoka, 802-0077, Japan
| | - Kanako Kurata
- Department of Surgery, Kitakyushu Municipal Medical Center, 2-1-1 Bashaku, Kokurakita-Ward, Kitakyushu, Fukuoka, 802-0077, Japan
| | - Kohei Horioka
- Department of Surgery, Kitakyushu Municipal Medical Center, 2-1-1 Bashaku, Kokurakita-Ward, Kitakyushu, Fukuoka, 802-0077, Japan
| | - Mitsuhiro Nakamoto
- Department of Surgery, Kitakyushu Municipal Medical Center, 2-1-1 Bashaku, Kokurakita-Ward, Kitakyushu, Fukuoka, 802-0077, Japan
| | - Kenichiro Koga
- Department of Surgery, Kitakyushu Municipal Medical Center, 2-1-1 Bashaku, Kokurakita-Ward, Kitakyushu, Fukuoka, 802-0077, Japan
| | - Shoshu Mitsuyama
- Department of Surgery, Kitakyushu Municipal Medical Center, 2-1-1 Bashaku, Kokurakita-Ward, Kitakyushu, Fukuoka, 802-0077, Japan
| | - Eiichi Sato
- Department of Medical Oncology, Kitakyushu Municipal Medical Center, 2-1-1 Bashaku, Kokurakita-Ward, Kitakyushu, Fukuoka, 802-0077, Japan
| | - Shinichi Wakamatsu
- Department of Medical Oncology, Kitakyushu Municipal Medical Center, 2-1-1 Bashaku, Kokurakita-Ward, Kitakyushu, Fukuoka, 802-0077, Japan
- Wakamatsu Oncology Clinic, 2nd floor, Kokura KMM Building, 2-14-1 Asano, KokuraKita-Ward, Kitakyushu, Fukuoka, 802-0001, Japan
| | - Sadafumi Tamiya
- Department of Pathology, Kitakyushu Municipal Medical Center, 2-1-1 Bashaku, Kokurakita-Ward, Kitakyushu, Fukuoka, 802-0077, Japan
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10
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Esperança-Martins M, Melo-Alvim C, Dâmaso S, Lopes-Brás R, Peniche T, Nogueira-Costa G, Abreu C, Luna Pais H, de Sousa RT, Torres S, Gallego-Paez LM, Martins M, Ribeiro L, Costa L. Breast Sarcomas, Phyllodes Tumors, and Desmoid Tumors: Turning the Magnifying Glass on Rare and Aggressive Entities. Cancers (Basel) 2023; 15:3933. [PMID: 37568749 PMCID: PMC10416994 DOI: 10.3390/cancers15153933] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/30/2023] [Revised: 07/27/2023] [Accepted: 07/28/2023] [Indexed: 08/13/2023] Open
Abstract
Breast sarcomas (BSs), phyllodes tumors (PTs), and desmoid tumors (DTs) are rare entities that arise from connective tissue. BSs can be classified as either primary or secondary, whether they develop de novo or after radiation exposure or lymphedema. PIK3CA seems to play an important common role in different BS. Malignant PTs show similar behavior to BSs, while DTs are locally aggressive but rarely metastasize. BSs usually present as unilateral, painless, rapidly growing masses with rare nodal involvement. The diagnosis should be based on magnetic resonance imaging and a core needle biopsy. Staging should comprise a chest computed tomography (CT) scan (except for benign PT and DT), while abdominal and pelvic CT scans and bone scans should be added in certain subtypes. The mainstay of treatment for localized BS is surgery, with margin goals that vary according to subtype. Radiotherapy and chemotherapy can be used as neoadjuvant or adjuvant approaches, but their use in these settings is not standard. Advanced BS should be treated with systemic therapy, consistent with recommendations for advanced soft tissue sarcomas of other topographies. Given the rarity and heterogeneity of these entities, multidisciplinary and multi-institutional collaboration and treatment at reference centers are critical.
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Affiliation(s)
- Miguel Esperança-Martins
- Medical Oncology Department, Centro Hospitalar Universitário Lisboa Norte, 1649-028 Lisboa, Portugal; (C.M.-A.); (S.D.); (R.L.-B.); (G.N.-C.); (C.A.); (H.L.P.); (R.T.d.S.); (S.T.); (L.R.)
- Luis Costa Lab, Instituto de Medicina Molecular João Lobo Antunes, Faculdade de Medicina da Universidade de Lisboa, 1649-028 Lisboa, Portugal; (T.P.); (L.M.G.-P.); (M.M.)
| | - Cecília Melo-Alvim
- Medical Oncology Department, Centro Hospitalar Universitário Lisboa Norte, 1649-028 Lisboa, Portugal; (C.M.-A.); (S.D.); (R.L.-B.); (G.N.-C.); (C.A.); (H.L.P.); (R.T.d.S.); (S.T.); (L.R.)
- Faculdade de Medicina da Universidade de Lisboa, 1649-028 Lisboa, Portugal
| | - Sara Dâmaso
- Medical Oncology Department, Centro Hospitalar Universitário Lisboa Norte, 1649-028 Lisboa, Portugal; (C.M.-A.); (S.D.); (R.L.-B.); (G.N.-C.); (C.A.); (H.L.P.); (R.T.d.S.); (S.T.); (L.R.)
| | - Raquel Lopes-Brás
- Medical Oncology Department, Centro Hospitalar Universitário Lisboa Norte, 1649-028 Lisboa, Portugal; (C.M.-A.); (S.D.); (R.L.-B.); (G.N.-C.); (C.A.); (H.L.P.); (R.T.d.S.); (S.T.); (L.R.)
| | - Tânia Peniche
- Luis Costa Lab, Instituto de Medicina Molecular João Lobo Antunes, Faculdade de Medicina da Universidade de Lisboa, 1649-028 Lisboa, Portugal; (T.P.); (L.M.G.-P.); (M.M.)
| | - Gonçalo Nogueira-Costa
- Medical Oncology Department, Centro Hospitalar Universitário Lisboa Norte, 1649-028 Lisboa, Portugal; (C.M.-A.); (S.D.); (R.L.-B.); (G.N.-C.); (C.A.); (H.L.P.); (R.T.d.S.); (S.T.); (L.R.)
- Faculdade de Medicina da Universidade de Lisboa, 1649-028 Lisboa, Portugal
| | - Catarina Abreu
- Medical Oncology Department, Centro Hospitalar Universitário Lisboa Norte, 1649-028 Lisboa, Portugal; (C.M.-A.); (S.D.); (R.L.-B.); (G.N.-C.); (C.A.); (H.L.P.); (R.T.d.S.); (S.T.); (L.R.)
- Faculdade de Medicina da Universidade de Lisboa, 1649-028 Lisboa, Portugal
| | - Helena Luna Pais
- Medical Oncology Department, Centro Hospitalar Universitário Lisboa Norte, 1649-028 Lisboa, Portugal; (C.M.-A.); (S.D.); (R.L.-B.); (G.N.-C.); (C.A.); (H.L.P.); (R.T.d.S.); (S.T.); (L.R.)
- Faculdade de Medicina da Universidade de Lisboa, 1649-028 Lisboa, Portugal
| | - Rita Teixeira de Sousa
- Medical Oncology Department, Centro Hospitalar Universitário Lisboa Norte, 1649-028 Lisboa, Portugal; (C.M.-A.); (S.D.); (R.L.-B.); (G.N.-C.); (C.A.); (H.L.P.); (R.T.d.S.); (S.T.); (L.R.)
- Faculdade de Medicina da Universidade de Lisboa, 1649-028 Lisboa, Portugal
| | - Sofia Torres
- Medical Oncology Department, Centro Hospitalar Universitário Lisboa Norte, 1649-028 Lisboa, Portugal; (C.M.-A.); (S.D.); (R.L.-B.); (G.N.-C.); (C.A.); (H.L.P.); (R.T.d.S.); (S.T.); (L.R.)
| | - Lina Marcela Gallego-Paez
- Luis Costa Lab, Instituto de Medicina Molecular João Lobo Antunes, Faculdade de Medicina da Universidade de Lisboa, 1649-028 Lisboa, Portugal; (T.P.); (L.M.G.-P.); (M.M.)
| | - Marta Martins
- Luis Costa Lab, Instituto de Medicina Molecular João Lobo Antunes, Faculdade de Medicina da Universidade de Lisboa, 1649-028 Lisboa, Portugal; (T.P.); (L.M.G.-P.); (M.M.)
| | - Leonor Ribeiro
- Medical Oncology Department, Centro Hospitalar Universitário Lisboa Norte, 1649-028 Lisboa, Portugal; (C.M.-A.); (S.D.); (R.L.-B.); (G.N.-C.); (C.A.); (H.L.P.); (R.T.d.S.); (S.T.); (L.R.)
- Faculdade de Medicina da Universidade de Lisboa, 1649-028 Lisboa, Portugal
| | - Luís Costa
- Medical Oncology Department, Centro Hospitalar Universitário Lisboa Norte, 1649-028 Lisboa, Portugal; (C.M.-A.); (S.D.); (R.L.-B.); (G.N.-C.); (C.A.); (H.L.P.); (R.T.d.S.); (S.T.); (L.R.)
- Luis Costa Lab, Instituto de Medicina Molecular João Lobo Antunes, Faculdade de Medicina da Universidade de Lisboa, 1649-028 Lisboa, Portugal; (T.P.); (L.M.G.-P.); (M.M.)
- Faculdade de Medicina da Universidade de Lisboa, 1649-028 Lisboa, Portugal
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11
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Teng L, Yan S, Du J, Yang R, Xu P, Tao W. Clinicopathological analysis and prognostic treatment study of angiosarcoma of the breast: a SEER population-based analysis. World J Surg Oncol 2023; 21:144. [PMID: 37158932 PMCID: PMC10169469 DOI: 10.1186/s12957-023-03030-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/06/2023] [Accepted: 05/03/2023] [Indexed: 05/10/2023] Open
Abstract
INTRODUCTION Breast angiosarcoma is a rare malignancy of endovascular origin, accounting for less than 1% of all mammary cancers. Our aim was to explore clinicopathological features and the factors associated with prognosis. METHODS We extracted information from the Surveillance, Epidemiology, and End Results Program (SEER) for all patients with breast angiosarcoma between 2004 and 2015. Chi-square analysis was used to compare the clinicopathological features in all patients. Overall survival (OS) was assessed using the Kaplan and Meier method. Univariate and multivariate analyses were performed to evaluate the factors associated with prognosis. RESULTS A total of 247 patients were included in the analyses. The median OS of patients with primary breast angiosarcoma (PBSA) and secondary breast angiosarcoma (SBAB) was 38 months and 42 months, respectively. The 1-, 3- and 5-year OS with PBSA was 80%, 39%, and 25%, respectively, and the 1-, 3- and 5-year OS with SBAB was 80%, 42%, and 34%, respectively. Multivariate analysis revealed that tumor size (p = 0.001), tumor grade (p < 0.001), tumor extension (p = 0.015), and tumor spread (p < 0.001) were statistically significant factors for OS. Partial mastectomy with radiation (HR = 0.160, 95% CI, 0.036-0.719, p = 0.016), partial mastectomy with chemotherapy (HR = 0.105, 95% CI, 0.011-1.015, p = 0.052), and partial mastectomy (HR = 0.125, 95% CI, 0.028-0.583, p = 0.007) were related to significantly better OS outcomes in primary angiosarcoma. CONCLUSION Primary breast angiosarcoma has a better clinical phenotype than secondary breast angiosarcoma. Although overall survival was not statistically significant, primary breast angiosarcoma was better than secondary breast angiosarcoma with systemic therapy. Depending on the outcome of survival, partial mastectomy is effective in treating primary breast angiosarcoma.
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Affiliation(s)
- Lizhi Teng
- Department of Breast Surgery, The First Affiliated Hospital of Harbin Medical University, Harbin, 150001, China
- Key Laboratory of Acoustic, Optical and Electromagnetic Diagnosis and Treatment of Cardiovascular Diseases, Heilongjiang, China
- The Cell Transplantation Key Laboratory of National Health Commission, Harbin, Heilongjiang, 150001, China
| | - Shuai Yan
- Department of Breast Surgery, The First Affiliated Hospital of Harbin Medical University, Harbin, 150001, China
- Key Laboratory of Acoustic, Optical and Electromagnetic Diagnosis and Treatment of Cardiovascular Diseases, Heilongjiang, China
- The Cell Transplantation Key Laboratory of National Health Commission, Harbin, Heilongjiang, 150001, China
- Key Laboratory of Hepatosplenic Surgery, Ministry of Education, Harbin, Heilongjiang, 150001, China
| | - Juntong Du
- Department of Breast Surgery, The First Affiliated Hospital of Harbin Medical University, Harbin, 150001, China
- Key Laboratory of Acoustic, Optical and Electromagnetic Diagnosis and Treatment of Cardiovascular Diseases, Heilongjiang, China
- The Cell Transplantation Key Laboratory of National Health Commission, Harbin, Heilongjiang, 150001, China
| | - Ru Yang
- Department of Breast Surgery, The First Affiliated Hospital of Harbin Medical University, Harbin, 150001, China
| | - Peng Xu
- Department of Breast Surgery, The First Affiliated Hospital of Harbin Medical University, Harbin, 150001, China
| | - Weiyang Tao
- Department of Breast Surgery, The First Affiliated Hospital of Harbin Medical University, Harbin, 150001, China.
- Key Laboratory of Acoustic, Optical and Electromagnetic Diagnosis and Treatment of Cardiovascular Diseases, Heilongjiang, China.
- The Cell Transplantation Key Laboratory of National Health Commission, Harbin, Heilongjiang, 150001, China.
- Key Laboratory of Hepatosplenic Surgery, Ministry of Education, Harbin, Heilongjiang, 150001, China.
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12
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Conti M, Morciano F, Rossati C, Gori E, Belli P, Fornasa F, Romanucci G, Rella R. Angiosarcoma of the Breast: Overview of Current Data and Multimodal Imaging Findings. J Imaging 2023; 9:jimaging9050094. [PMID: 37233313 DOI: 10.3390/jimaging9050094] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/31/2023] [Revised: 04/25/2023] [Accepted: 04/26/2023] [Indexed: 05/27/2023] Open
Abstract
Angiosarcoma of the breast is a rare breast cancer, which can arise de novo (primary breast angiosarcoma, PBA) or as a secondary malignancy (secondary breast angiosarcoma, SBA) as a result of a biological insult. In the latter case, it is usually diagnosed in patients with a previous history of radiation therapy following a conserving treatment for breast cancer. Over the years, the advances in early diagnosis and treatment of breast cancer, with increasing use of breast-conserving surgery and radiation therapy (instead of radical mastectomy), brought about an increased incidence of the secondary type. PBA and SBA have different clinical presentations and often represent a diagnostic challenge due to the nonspecific imaging findings. The purpose of this paper is to review and describe the radiological features of breast angiosarcoma, both in conventional and advanced imaging to guide radiologists in the diagnosis and management of this rare tumor.
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Affiliation(s)
- Marco Conti
- UOC di Radiologia Toracica e Cardiovascolare, Dipartimento di Diagnostica per Immagini, Radioterapia Oncologica ed Ematologia, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Largo A. Gemelli 8, 00168 Rome, Italy
| | - Francesca Morciano
- Facoltà di Medicina e Chirurgia, Università Cattolica Sacro Cuore, Largo F. Vito 1, 00168 Rome, Italy
| | - Claudia Rossati
- UOSD Breast Unit ULSS9, Ospedale di Marzana, Piazzale Lambranzi, 1, 37142 Verona, Italy
| | - Elisabetta Gori
- Facoltà di Medicina e Chirurgia, Università Cattolica Sacro Cuore, Largo F. Vito 1, 00168 Rome, Italy
| | - Paolo Belli
- UOC di Radiologia Toracica e Cardiovascolare, Dipartimento di Diagnostica per Immagini, Radioterapia Oncologica ed Ematologia, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Largo A. Gemelli 8, 00168 Rome, Italy
- Facoltà di Medicina e Chirurgia, Università Cattolica Sacro Cuore, Largo F. Vito 1, 00168 Rome, Italy
| | - Francesca Fornasa
- UOSD Breast Unit ULSS9, Ospedale di Marzana, Piazzale Lambranzi, 1, 37142 Verona, Italy
| | - Giovanna Romanucci
- UOSD Breast Unit ULSS9, Ospedale di Marzana, Piazzale Lambranzi, 1, 37142 Verona, Italy
| | - Rossella Rella
- UOC Diagnostica per Immagini, Ospedale G.B. Grassi, Via Gian Carlo Passeroni, 28, 00122 Rome, Italy
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13
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Ooe Y, Terakawa H, Kawashima H, Ikeda H, Inaki N. Bilateral primary angiosarcoma of the breast: a case report. J Med Case Rep 2023; 17:60. [PMID: 36803941 PMCID: PMC9942292 DOI: 10.1186/s13256-023-03791-7] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/21/2022] [Accepted: 01/24/2023] [Indexed: 02/22/2023] Open
Abstract
BACKGROUND Primary angiosarcoma of the breast is very rare, accounting for 0.05% of all malignant breast tumors. It has very high malignant potential and poor prognosis, though due to the rarity of the disease, there is no established treatment. We report this case along with a literature review. CASE PRESENTATION We report the case of a 30-year-old Asian woman who was diagnosed with bilateral primary angiosarcoma of the breast while breastfeeding. After surgery, she underwent radiation therapy, chemotherapy, and hepatic arterial infusion chemotherapy for local recurrence of liver metastases, but these were ineffective, and she required several arterial embolization procedures for intratumoral bleeding and rupture of liver metastases. CONCLUSIONS Angiosarcoma has a poor prognosis due to a high rate of local recurrence and distant metastasis. Although there is no established evidence for radiotherapy or chemotherapy, multimodality treatment may be necessary because of the high malignancy and rapid progression of the disease.
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Affiliation(s)
- Yuka Ooe
- Department of Breast Surgery, Kanazawa University Hospital, 13-1 Takaramachi, Kanazawa, Ishikawa, Japan. .,Breast Center, Kanazawa University Hospital, 13-1 Takaramachi, Kanazawa, Ishikawa, Japan.
| | - Hirofumi Terakawa
- grid.412002.50000 0004 0615 9100Department of Breast Surgery, Kanazawa University Hospital, 13-1 Takaramachi, Kanazawa, Ishikawa Japan ,grid.412002.50000 0004 0615 9100Breast Center, Kanazawa University Hospital, 13-1 Takaramachi, Kanazawa, Ishikawa Japan
| | - Hiroko Kawashima
- grid.412002.50000 0004 0615 9100Breast Center, Kanazawa University Hospital, 13-1 Takaramachi, Kanazawa, Ishikawa Japan
| | - Hiroko Ikeda
- grid.412002.50000 0004 0615 9100Department of Diagnostic Pathology, Kanazawa University Hospital, 13-1 Takaramachi, Kanazawa, Ishikawa Japan
| | - Noriyuki Inaki
- grid.412002.50000 0004 0615 9100Department of Gastrointestinal Surgery/Breast Surgery, Kanazawa University Hospital, 13-1 Takaramachi, Kanazawa, Ishikawa Japan
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14
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Ferre R, Kuzmiak CM. A rare presentation of pregnancy associated primary angiosarcoma of the breasts. Radiol Case Rep 2022; 17:2708-2713. [PMID: 35669222 PMCID: PMC9162934 DOI: 10.1016/j.radcr.2022.02.061] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/15/2022] [Revised: 02/16/2022] [Accepted: 02/19/2022] [Indexed: 11/27/2022] Open
Abstract
Primary angiosarcoma of the breast is a rare malignancy that is important for radiologists to recognize in young patients because its imaging appearance may mimic benign lesions resulting in advanced stages of disease and overall decreased patient survival. We present a unique case of a bilateral primary angiosarcoma in a pregnant patient in her twenties. She presented with a self-detected, rapidly enlarging, non-tender right breast mass while in her third trimester.
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Affiliation(s)
- Romuald Ferre
- Guelph Medical Imaging, 54 Cardigan Street, Guelph, Ontario, Canada
| | - Cherie M Kuzmiak
- Division of Breast Imaging, Department of Radiology, Breast Imaging Fellowship Program, UNC School of Medicine, Chapel Hill, NC
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15
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Ramelli E, Weingertner N, Welsch A, Reix N, Antoni D, Amé S, Molière S, Mathelin C. Innovative approach to lymphadenectomy in breast sarcoma. Bull Cancer 2022; 109:1017-1028. [DOI: 10.1016/j.bulcan.2022.04.012] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/21/2021] [Revised: 04/13/2022] [Accepted: 04/19/2022] [Indexed: 11/27/2022]
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Kim YJ, Ryu JM, Lee SK, Chae BJ, Kim SW, Nam SJ, Yu JH, Lee JE. Primary Angiosarcoma of the Breast: A Single-Center Retrospective Study in Korea. Curr Oncol 2022; 29:3272-3281. [PMID: 35621657 PMCID: PMC9139487 DOI: 10.3390/curroncol29050267] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/20/2022] [Revised: 04/25/2022] [Accepted: 04/29/2022] [Indexed: 11/16/2022] Open
Abstract
Due to the rarity of primary angiosarcoma of the breast, optimal management is based on expert opinion. The aim of this study was to review all primary angiosarcomas of the breast obtained from a single center in terms of clinicopathologic characteristics, treatment, and survival outcomes. From 1997 to 2020, 15 patients with primary angiosarcoma of the breast underwent either mastectomy or wide excision. We analyzed the clinicopathologic data to assess disease-free survival and overall survival. Fifteen women with primary angiosarcoma of the breast were identified. The mean age at diagnosis was 33 years (range: 14–63 years). The overall mean tumor size was 7.7 cm (range 3.5–20 cm). Upon histological grading, there were three cases of low grade, five intermediate grade, six high grade, and one unidentified grade. The five-year disease-free survival rate was 24.4%, and the five-year survival rate was 37.2%. The survival rate of the low-grade patient group was statistically higher than that of the intermediate- or high-grade patient groups (p = 0.024). Primary angiosarcoma of the breast is a rare aggressive tumor characterized by high grade and poor outcome. Histologic grade appears to be a reliable predictor of survival. There are no standard treatment guidelines; thus, optimal R0 surgical resection remains the best approach. The roles of neoadjuvant, adjuvant chemotherapy, and radiotherapy remain unclear.
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Affiliation(s)
| | | | | | | | | | | | - Jong-Han Yu
- Correspondence: (J.-H.Y.); (J.-E.L.); Tel.: +82-2-3410-0260 (J.-H.Y.); +82-2-3410-3479 (J.-E.L.); Fax: +82-2-3410-6982 (J.-H.Y.); +82-2-3410-6982 (J.-E.L.)
| | - Jeong-Eon Lee
- Correspondence: (J.-H.Y.); (J.-E.L.); Tel.: +82-2-3410-0260 (J.-H.Y.); +82-2-3410-3479 (J.-E.L.); Fax: +82-2-3410-6982 (J.-H.Y.); +82-2-3410-6982 (J.-E.L.)
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17
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Herb J, Maduekwe UN, Goel N, Rosenberger LH, Spanheimer PM. Does Angiosarcoma of the Breast Need Nodal Staging? J Am Coll Surg 2022; 234:774-782. [PMID: 35426390 PMCID: PMC9309687 DOI: 10.1097/xcs.0000000000000131] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
BACKGROUND Breast angiosarcoma is a rare malignancy classically associated with hematogenous metastases. We sought to determine the prevalence of pathologic nodal involvement in patients with nonmetastatic, resected breast angiosarcoma and its association with overall survival. STUDY DESIGN The National Cancer Database was used to identify patients with nonmetastatic angiosarcoma of the breast who underwent surgical resection from 2004 to 2017. The prevalence of regional lymph node operation and nodal positivity was calculated. The Kaplan-Meier method was used to evaluate overall survival among node-positive and node-negative patients. Cox proportional hazard modeling was used to evaluate the adjusted association of nodal positivity with overall survival. RESULTS We included 991 patients with angiosarcoma. The median age was 69 years (interquartile range 57 to 78), and the cohort was 99% female. A total of 298 patients (30%) had pathologic regional nodal evaluation. Of those, 15 (5.0%) had positive regional lymph nodes. Node-positive patients had significantly worse survival than patients with negative regional lymph nodes. After adjusting for patient, tumor, and treatment factors, a positive regional lymph node was associated with worse overall survival compared with patients with no nodal evaluation (hazard ratio 3.20; 95% CI 1.75 to 5.86; p < 0.001). CONCLUSIONS Patients with nonmetastatic angiosarcoma of the breast have a 5% regional lymph node positivity rate, which is at a common threshold to consider evaluation, and identifies patients with poor survival. A prospective study to determine performance characteristics of sentinel lymph node biopsy is warranted.
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Affiliation(s)
- Joshua Herb
- Department of Surgery, University of North Carolina, Chapel Hill, NC
| | | | - Neha Goel
- Department of Surgery, University of Miami Sylvester Comprehensive Cancer Center, Miami, FL
| | | | - Philip M. Spanheimer
- Department of Surgery, University of North Carolina, Chapel Hill, NC
- UNC Lineberger Comprehensive Cancer Center, Chapel Hill, NC
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Kokkali S, Moreno JD, Klijanienko J, Theocharis S. Clinical and Molecular Insights of Radiation-Induced Breast Sarcomas: Is There Hope on the Horizon for Effective Treatment of This Aggressive Disease? Int J Mol Sci 2022; 23:ijms23084125. [PMID: 35456944 PMCID: PMC9029574 DOI: 10.3390/ijms23084125] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/03/2022] [Revised: 03/31/2022] [Accepted: 04/01/2022] [Indexed: 11/16/2022] Open
Abstract
Radiation-induced breast sarcomas (RIBS) are rare entities representing <1% of all primary breast malignancies, limiting most reports to small retrospective case series. They constitute a heterogeneous group of neoplasms, with high-grade angiosarcoma being the most common subtype. Other sarcoma histotypes, such as undifferentiated pleomorphic sarcoma and leiomyosarcoma, can also be identified. Radiation-induced breast angiosarcoma (RIBA) has an incidence of approximately 0.1% after breast-conserving therapy and arises mainly from the dermis of the irradiated breast. MYC gene amplification is highly indicative of secondary breast angiosarcomas. Their clinical presentation often mimics benign port-radiation lesions, leading to a delay in diagnosis and a lost window of opportunity for cure. Surgery with negative margins is the mainstay of treatment of localized RIBS. In the case of angiosarcoma, technical difficulties, including multifocality, infiltrative margins, and difficulty in assessing tumor margins, render surgical treatment quite challenging. A limited number of studies showed that adjuvant radiation therapy reduces local recurrences; therefore, it is proposed by many groups for large, high-grade tumors. Chemotherapy has been evaluated retrospectively in a small subset of patients, with some evidence supporting its use in angiosarcoma patients. Approximately half of patients with RIBA will show local recurrence. In the advanced setting, different therapeutic options are discussed in the review, including chemotherapy, antiangiogenic therapy, and immunotherapy, whereas the need for further research on molecular therapeutic targets is pointed out.
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Affiliation(s)
- Stefania Kokkali
- First Department of Pathology, Medical School, National and Kapodistrian University of Athens, 75 Mikras Asias Street, 11527 Athens, Greece;
- Oncology Unit, 2nd Department of Medicine, Medical School, National and Kapodistrian University of Athens, Hippocratio General Hospital of Athens, V. Sofias 114, 11527 Athens, Greece
- Correspondence: ; Tel.: +30-6932326547
| | - Jose Duran Moreno
- Hellenic Group of Sarcoma and Rare Cancers, G. Theologou 5, 11471 Athens, Greece;
| | - Jerzy Klijanienko
- Department of Pathology, Institut Curie, 26 Rue d’Ulm, CEDEX 05, 75248 Paris, France;
| | - Stamatios Theocharis
- First Department of Pathology, Medical School, National and Kapodistrian University of Athens, 75 Mikras Asias Street, 11527 Athens, Greece;
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19
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He Y, Qian L, Chen L, Liu Y, Wen Y, Cao P. Primary breast angiosarcoma: A case report. Front Surg 2022; 9:966792. [PMID: 36873809 PMCID: PMC9982166 DOI: 10.3389/fsurg.2022.966792] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/11/2022] [Accepted: 12/19/2022] [Indexed: 02/19/2023] Open
Abstract
Background Primary breast angiosarcoma (PBA) is a rare sarcoma, accounting for only 0.04% of all breast malignancies, with a difficult diagnosis and a poor prognosis. Mastectomy is the standard treatment, and the role of adjuvant treatment (chemotherapy and/or radiotherapy following surgery) remains uncertain with very few studies. Case Presentation We report the case of a 17-year-old female patient who presented with a right breast lump that had rapidly increased in size and was hemorrhaging. She was diagnosed with breast angiosarcoma by needle biopsy and pathological evaluation. However, the mass showed a quick tendency to bleed during biopsies. After that, we performed angiography and tumor vascular embolization. The patient underwent a mastectomy followed by adjuvant chemotherapy. Conclusion Tumor vascular embolization reduced the surgical risk of PBA with hemorrhage complications. Postoperative therapeutic roles still need further exploration and verification.
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Affiliation(s)
- Yu He
- Department of Oncology, Third Xiangya Hospital, Central South University, Changsha, China
| | - Liyuan Qian
- Department of Breast and Thyroid Surgery, Third Xiangya Hospital, Central South University, Changsha, China
| | - Lang Chen
- Department of Hepatobiliary and Pancreatic Surgery, Third Xiangya Hospital, Central South University, Changsha, China
| | - Yang Liu
- Department of Pathology, Third Xiangya Hospital, Central South University, Changsha, China
| | - Yanguang Wen
- Department of Breast and Thyroid Surgery, Third Xiangya Hospital, Central South University, Changsha, China
| | - Peiguo Cao
- Department of Oncology, Third Xiangya Hospital, Central South University, Changsha, China
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20
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Javed N, Stowman AM. Educational Case: Radiation-Induced Angiosarcoma of the Breast. Acad Pathol 2021; 8:23742895211060529. [PMID: 34926795 PMCID: PMC8679045 DOI: 10.1177/23742895211060529] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/02/2021] [Revised: 09/14/2021] [Accepted: 10/17/2021] [Indexed: 11/26/2022] Open
Abstract
The following fictional case is intended as a learning tool within the Pathology
Competencies for Medical Education (PCME), a set of national standards for teaching
pathology. These are divided into three basic competencies: Disease Mechanisms and
Processes, Organ System Pathology, and Diagnostic Medicine and Therapeutic Pathology.
For additional information, and a full list of learning objectives for all three
competencies, seehttp://journals.sagepub.com/doi/10.1177/2374289517715040.1
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Affiliation(s)
- Noman Javed
- Department of Pathology and Laboratory Medicine, University of Vermont Medical Center, Burlington, VT, USA
| | - Anne M Stowman
- Department of Pathology and Laboratory Medicine, University of Vermont Medical Center, Burlington, VT, USA
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21
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Corines MJ, Krystel-Whittemore M, Murray M, Mango V. Uncommon Tumors and Uncommon Presentations of Cancer in the Breast. CURRENT BREAST CANCER REPORTS 2021; 13:331-346. [PMID: 36589040 PMCID: PMC9798716 DOI: 10.1007/s12609-021-00435-x] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/05/2023]
Abstract
Purpose of review The purpose is to present a case series of rare diagnoses and unusual presentations of breast lesions with radiologic-pathologic correlation from a major cancer center, and to review the recent literature on each entity with a focus on radiology-pathology concordance. We present our findings and experience from cases of metastatic small cell lung carcinoma to the breast, IgG-4 related breast disease, breast implant associated anaplastic large cell lymphoma, granular cell tumor, pleomorphic sarcoma, adenomyoepithelioma, post-radiation angiosarcoma, and breast carcinoma after risk-reducing total mastectomy. Recent findings It is essential for physicians to have knowledge of rare breast diagnoses and unusual breast disease presentations to formulate a complete differential diagnosis, recognize radiological-pathological concordance of these entities and provide appropriate patient care. Summary Current literature on these rare described entities exists mainly as case reports, case series and small-scale studies. By sharing our findings, we hope to educate trainees in radiology, pathology and other fields across the continuum of care in radiologic-pathologic correlation, while also augmenting the existing literature on these rare entities.
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Affiliation(s)
- Marina J. Corines
- Memorial Sloan Kettering Cancer Center, Department of Radiology, 300 E 66th Street, New York, NY 10065,Corresponding author: Marina J. Corines, MD, 1275 York Ave, Department of Radiology, New York, New York, 10065, Telephone: (914) 319-6970,
| | | | - Melissa Murray
- Memorial Sloan Kettering Cancer Center, Department of Pathology, 1275 York Avenue, New York, NY 10065
| | - Victoria Mango
- Memorial Sloan Kettering Cancer Center, Department of Radiology, 300 E 66th Street, New York, NY 10065
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22
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Schott I, Liffers ST, Farzaliyev F, Falkenhorst J, Steinau HU, Treckmann JW, Podleska LE, Pöttgen C, Schildhaus HU, Ahrens M, Dirksen U, Murat FZ, Siveke JT, Bauer S, Hamacher R. Localized Angiosarcoma, Not One Disease: A Retrospective Single-Center Study on Prognosis Depending on the Primary Site and Etiology. Sarcoma 2021; 2021:9960085. [PMID: 34545273 PMCID: PMC8449723 DOI: 10.1155/2021/9960085] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/24/2021] [Revised: 06/16/2021] [Accepted: 08/14/2021] [Indexed: 01/21/2023] Open
Abstract
BACKGROUND Angiosarcomas are rare and heterogeneous tumors with poor prognosis. The clinical subtypes are classified depending on the primary site and etiology. METHODS We conducted a retrospective, monocentric study of 136 patients with localized AS between May 1985 and November 2018. Overall survival (OS), local recurrence-free survival (LRFS), and metastasis-free survival (MFS) were estimated using the Kaplan-Meier method. To identify prognostic factors, univariate and multivariate analyses were performed based on Cox regressions. RESULTS The median age was 67 years (19-72.8 years). Primary sites were cutaneous (27.2%), breast (38.2%), and deep soft tissue (34.6%). The majority was primary angiosarcomas (55.9%) followed by postradiation (40.4%) and chronic lymphedema angiosarcomas (2.9%). Prognosis significantly differed depending on the primary site and etiology. Shortest median OS and MFS were observed in deep soft tissue angiosarcomas, whereas cutaneous angiosarcomas, angiosarcomas of the breast, and radiation-associated angiosarcomas displayed worse median LRFS. Univariate analyses showed better OS for tumor size <10 cm (p = 0.009), negative surgical margins (p = 0.021), and negative lymph node status (p = 0.007). LRFS and MFS were longer for tumor size <10 cm (p = 0.012 and p = 0.013). In multivariate analyses, age <70 years was the only independent positive prognostic factor for OS in all subgroups. For LRFS, secondary AS of the breast was a negative prognostic factor (HR: 2.35; p = 0.035). CONCLUSIONS Different behaviors and prognoses depending on the primary site and etiology should be considered for the treatment of this heterogeneous disease. In cutaneous angiosarcomas of the head/neck and postradiation angiosarcomas of the breast, local recurrence seems to have a crucial impact on OS. Therefore, improved local therapies and local tumor staging may have to be implemented. However, in deep soft tissue angiosarcomas, distant recurrence seems to have a major influence on prognosis, which indicates a benefit of additional perioperative chemotherapy.
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Affiliation(s)
- Inna Schott
- Department of Medical Oncology, Sarcoma Center, West German Cancer Center, University Hospital Essen, Essen, Germany
| | - Sven-Thorsten Liffers
- Bridge Institute of Experimental Tumor Therapy, West German Cancer Center, University Hospital Essen, Essen, Germany
- Division of Solid Tumor Translational Oncology, German Cancer Consortium (DKTK,Partner Site Essen) and German Cancer Research Center, DKFZ, Heidelberg, Germany
| | - Farhad Farzaliyev
- Department of Orthopedic Oncology, West German Cancer Center, University Hospital Essen, Essen, Germany
| | - Johanna Falkenhorst
- Department of Medical Oncology, Sarcoma Center, West German Cancer Center, University Hospital Essen, Essen, Germany
- German Cancer Consortium (DKTK), Partner Site University Hospital Essen, Essen, Germany
| | - Hans-Ulrich Steinau
- Department of General, Visceral and Transplantion Surgery, Sarcoma Center, West German Cancer Center, University Hospital Essen, Essen, Germany
| | - Jürgen-Walter Treckmann
- Department of General, Visceral and Transplantion Surgery, Sarcoma Center, West German Cancer Center, University Hospital Essen, Essen, Germany
| | - Lars Erik Podleska
- Department of Orthopedic Oncology, West German Cancer Center, University Hospital Essen, Essen, Germany
| | - Christoph Pöttgen
- Department of Radiotherapy, West German Cancer Center, University Hospital Essen, Essen, Germany
| | - Hans-Ulrich Schildhaus
- Institute of Pathology, West German Cancer Center, University Hospital Essen, Essen, Germany
| | - Marit Ahrens
- Medical Clinic II, University Hospital Frankfurt, Frankfurt am Main, Germany
| | - Uta Dirksen
- German Cancer Consortium (DKTK), Partner Site University Hospital Essen, Essen, Germany
- Pediatrics III Pediatric Hematology, Oncology, Immunology, Cardiology, Pulmonology, West German Cancer Center, University Hospital Essen, Essen, Germany
| | - Fatma-Zehra Murat
- Department of Medical Oncology, Sarcoma Center, West German Cancer Center, University Hospital Essen, Essen, Germany
| | - Jens T. Siveke
- Department of Medical Oncology, Sarcoma Center, West German Cancer Center, University Hospital Essen, Essen, Germany
- Bridge Institute of Experimental Tumor Therapy, West German Cancer Center, University Hospital Essen, Essen, Germany
- Division of Solid Tumor Translational Oncology, German Cancer Consortium (DKTK,Partner Site Essen) and German Cancer Research Center, DKFZ, Heidelberg, Germany
| | - Sebastian Bauer
- Department of Medical Oncology, Sarcoma Center, West German Cancer Center, University Hospital Essen, Essen, Germany
- German Cancer Consortium (DKTK), Partner Site University Hospital Essen, Essen, Germany
| | - Rainer Hamacher
- Department of Medical Oncology, Sarcoma Center, West German Cancer Center, University Hospital Essen, Essen, Germany
- German Cancer Consortium (DKTK), Partner Site University Hospital Essen, Essen, Germany
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23
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Kim J, Jung HK, Kim W. Angiosarcoma of the Breast and Spleen: A Case Report with Imaging and Pathologic Findings. TAEHAN YONGSANG UIHAKHOE CHI 2021; 82:1310-1314. [PMID: 36238393 PMCID: PMC9432375 DOI: 10.3348/jksr.2020.0179] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 10/17/2020] [Revised: 01/13/2021] [Accepted: 02/01/2021] [Indexed: 12/04/2022]
Abstract
Angiosarcoma of the breast is a rare malignant sarcoma of endovascular origin that presents with various radiological findings. We encountered a case of angiosarcoma of the breast presenting as an irregular indistinct hyperechoic mass on ultrasonography, with a large single angiosarcoma of the spleen in a previously healthy 36-year-old female. Herein, we report the imaging and pathologic findings and review the literature.
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24
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Kronenfeld JP, Crystal JS, Ryon EL, Yadegarynia S, Chitters C, Yechieli R, D’Amato G, Rosenberg AE, Kesmodel SB, Trent JC, Goel N. Clinical Outcomes for Primary and Radiation-Associated Angiosarcoma of the Breast with Multimodal Treatment: Long-Term Survival Is Achievable. Cancers (Basel) 2021; 13:3814. [PMID: 34359716 PMCID: PMC8345179 DOI: 10.3390/cancers13153814] [Citation(s) in RCA: 11] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/30/2021] [Revised: 07/24/2021] [Accepted: 07/27/2021] [Indexed: 12/22/2022] Open
Abstract
BACKGROUND The optimal management of primary angiosarcoma (PAS) and radiation-associated angiosarcoma (RAAS) of the breast remains undefined. Available data show persistently poor survival outcomes following treatment with surgery or chemotherapy alone. The objective of this study was to evaluate long-term outcomes in patients treated with multimodality therapy. METHODS Patients diagnosed with stage I-III PAS or RAAS of the breast were identified from our local tumor registry (2010-2020). Patient demographics, tumor characteristics, and treatment were collected. Primary outcomes were local recurrence (LR), distant recurrence (DR), and median overall survival (OS). A secondary outcome was pathologic complete response (pCR) following neoadjuvant chemotherapy (NAC). Mann-Whitney U, chi-squared, or Fisher exact tests were used to analyze data. Kaplan-Meier curves compared OS for PAS and RAAS. RESULTS Twenty-two patients met inclusion criteria, including 11 (50%) with RAAS and 11 (50%) with PAS. Compared to PAS patients, RAAS patients were older and had more comorbidities. For RAAS patients, median time from radiation to diagnosis was 6 years (IQR: 5-11). RAAS patients were more likely to have a pCR to NAC (40% vs. 20%, p = 0.72). RAAS patients had a higher LR rate (43% vs. 38%, p = 0.83), and PAS patients were more likely to develop a DR (38% vs. 0%, p = 0.07). Median OS was 81 months in PAS patients and 90 months in RAAS patients (p = 1.00). DISCUSSION Long-term survival can be achieved in patients with PAS and RAAS who undergo multimodality treatment. NAC can result in pCR. The long-term clinical implications of pCR warrant further investigation.
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Affiliation(s)
- Joshua P. Kronenfeld
- Sylvester Comprehensive Cancer Center, Division of Surgical Oncology, Department of Surgery, University of Miami Miller School of Medicine, Miami, FL 33132, USA; (J.P.K.); (J.S.C.); (E.L.R.); (S.Y.); (C.C.); (S.B.K.)
| | - Jessica S. Crystal
- Sylvester Comprehensive Cancer Center, Division of Surgical Oncology, Department of Surgery, University of Miami Miller School of Medicine, Miami, FL 33132, USA; (J.P.K.); (J.S.C.); (E.L.R.); (S.Y.); (C.C.); (S.B.K.)
| | - Emily L. Ryon
- Sylvester Comprehensive Cancer Center, Division of Surgical Oncology, Department of Surgery, University of Miami Miller School of Medicine, Miami, FL 33132, USA; (J.P.K.); (J.S.C.); (E.L.R.); (S.Y.); (C.C.); (S.B.K.)
| | - Sina Yadegarynia
- Sylvester Comprehensive Cancer Center, Division of Surgical Oncology, Department of Surgery, University of Miami Miller School of Medicine, Miami, FL 33132, USA; (J.P.K.); (J.S.C.); (E.L.R.); (S.Y.); (C.C.); (S.B.K.)
| | - Celeste Chitters
- Sylvester Comprehensive Cancer Center, Division of Surgical Oncology, Department of Surgery, University of Miami Miller School of Medicine, Miami, FL 33132, USA; (J.P.K.); (J.S.C.); (E.L.R.); (S.Y.); (C.C.); (S.B.K.)
| | - Raphael Yechieli
- Sylvester Comprehensive Cancer Center, Department of Radiation Oncology, University of Miami Miller School of Medicine, Miami, FL 33132, USA;
| | - Gina D’Amato
- Sylvester Comprehensive Cancer Center, Division of Medical Oncology, Department of Medicine, University of Miami Miller School of Medicine, Miami, FL 33132, USA; (G.D.); (J.C.T.)
| | - Andrew E. Rosenberg
- Sylvester Comprehensive Cancer Center, Division of Anatomic Pathology Services, Department of Pathology & Laboratory Medicine, University of Miami Miller School of Medicine, Miami, FL 33132, USA;
| | - Susan B. Kesmodel
- Sylvester Comprehensive Cancer Center, Division of Surgical Oncology, Department of Surgery, University of Miami Miller School of Medicine, Miami, FL 33132, USA; (J.P.K.); (J.S.C.); (E.L.R.); (S.Y.); (C.C.); (S.B.K.)
| | - Jonathan C. Trent
- Sylvester Comprehensive Cancer Center, Division of Medical Oncology, Department of Medicine, University of Miami Miller School of Medicine, Miami, FL 33132, USA; (G.D.); (J.C.T.)
| | - Neha Goel
- Sylvester Comprehensive Cancer Center, Division of Surgical Oncology, Department of Surgery, University of Miami Miller School of Medicine, Miami, FL 33132, USA; (J.P.K.); (J.S.C.); (E.L.R.); (S.Y.); (C.C.); (S.B.K.)
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Tripathy S, Rastogi S, Prakash S, Arun Raj ST, Sagar S, Shamim SA. Solitary Subcutaneous Tissue Metastasis as Recurrence in a Case of Primary Angiosarcoma of Breast: Findings on 18F-Fluorodeoxyglucose Positron Emission Tomography-Computed Tomography. Indian J Nucl Med 2021; 36:210-211. [PMID: 34385799 PMCID: PMC8320824 DOI: 10.4103/ijnm.ijnm_84_20] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/27/2020] [Revised: 05/01/2020] [Accepted: 05/19/2020] [Indexed: 11/04/2022] Open
Abstract
Primary angiosarcomas of the breast are rare tumors, with a fatal outcome. We present a rare case of an operated primary angiosarcoma of the right breast in a 20-year-old female who showed disease recurrence in the right posterior arm subcutaneous tissue on 18F-fluorodeoxyglucose positron emission tomography-computed tomography after 1 year of surgery without any other visceral metastasis.
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Affiliation(s)
- Sarthak Tripathy
- Department of Nuclear Medicine and PET-CT, All India Institute of Medical Sciences, New Delhi, India
| | - Sameer Rastogi
- Department of Medical Oncology, All India Institute of Medical Sciences, New Delhi, India
| | - Sneha Prakash
- Department of Nuclear Medicine and PET-CT, All India Institute of Medical Sciences, New Delhi, India
| | | | - Sambit Sagar
- Department of Nuclear Medicine and PET-CT, All India Institute of Medical Sciences, New Delhi, India
| | - Shamim Ahmed Shamim
- Department of Nuclear Medicine and PET-CT, All India Institute of Medical Sciences, New Delhi, India
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26
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Mumin NA, Rahmat K, Hamid MTR, Ng WL, Chan WY, Cheah XY, See MH, Yip CH. Primary Breast Angiosarcoma: Utilisation of Pre-surgical Magnetic Resonance Imaging (MRI) for Accurate Tumour Characterization and Planning - A Case Report and Literature Review. Curr Med Imaging 2021; 17:552-558. [PMID: 33030134 DOI: 10.2174/1573405616666201007161119] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/17/2020] [Revised: 08/05/2020] [Accepted: 08/12/2020] [Indexed: 02/08/2023]
Abstract
BACKGROUND Primary breast angiosarcoma is a rare malignancy with non-specific clinical and radiological findings. CASE REPORT A 30-year-old lady presented with left breast pain and lumpiness for over one year. She has had several breast ultrasounds (US) and was treated for acute mastitis and abscess. Subsequently, in view of the rapid growth of the lump and worsening pain, she was re-investigated with US, elastography, digital breast tomosynthesis (DBT) and MRI. MRI raised the suspicion of angiosarcoma. The diagnosis was confirmed after biopsy and she underwent mastectomy. DISCUSSION Literature review on imaging findings of breast angiosarcoma, especially on MRI, is discussed. MRI features showed heterogeneous low signal intensity on T1 and high signal intensity on T2. Dynamic contrast enhancement (DCE) features included either early enhancement with or without washout in the delayed phase, and some reported central areas of non-enhancement. CONCLUSION This case report emphasises on the importance of MRI in clinching the diagnosis of breast angiosarcoma, and hence, should be offered sooner to prevent diagnostic delay.
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Affiliation(s)
- Nazimah Ab Mumin
- Department of Biomedical Imaging, University of Malaya Research Imaging Centre, Kuala Lumpur, Malaysia
| | - Kartini Rahmat
- Department of Biomedical Imaging, University of Malaya Research Imaging Centre, Kuala Lumpur, Malaysia
| | - Marlina Tanty Ramli Hamid
- Department of Biomedical Imaging, University of Malaya Research Imaging Centre, Kuala Lumpur, Malaysia
| | - Wei Lin Ng
- Department of Biomedical Imaging, University of Malaya Research Imaging Centre, Kuala Lumpur, Malaysia
| | - Wai Yee Chan
- Department of Biomedical Imaging, University of Malaya Research Imaging Centre, Kuala Lumpur, Malaysia
| | - Xin Ying Cheah
- Department of Radiology, Hospital Sultan Ismail, Johor, Malaysia
| | - Mee Hoong See
- Department of Surgery, University of Malaya Medical Centre, Kuala Lumpur, Malaysia
| | - Cheng Har Yip
- Department of Surgery, Subang Jaya Medical Centre, Kuala Lumpur, Malaysia
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Qin X, Wu Y, Yu L, Lv Q, Xie M. Metastasis of primary breast angiosarcoma to axillary and supraclavicular lymph nodes: a rare case diagnosed using imaging data. J Int Med Res 2021; 49:3000605211002337. [PMID: 33784853 PMCID: PMC8020107 DOI: 10.1177/03000605211002337] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022] Open
Abstract
Primary breast angiosarcoma (PBA) is a rare malignant tumor. PBA usually undergoes hematogenous metastasis; lymph node metastasis is very rare in such patients, and metastasis of PBA to the supraclavicular lymph nodes has not previously been reported. Here, we describe a rare case of PBA manifested by a diffuse enlargement of the left breast, with metastasis to the left axillary and bilateral supraclavicular lymph nodes. Contrast-enhanced ultrasound and positron emission tomography findings indicated a malignant lesion, whereas magnetic resonance imaging suggested a benign lesion. Core needle biopsy identified the lesion as a lymphangioma, and the histological characteristics suggested a high-grade angiosarcoma. Multimodal imaging and perfusion patterns obtained using various contrast agents can thus help to diagnose PBA.
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Affiliation(s)
- Xiaojuan Qin
- Department of Ultrasound, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Yu Wu
- Department of Ultrasound, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Lan Yu
- Department of Pathology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Qing Lv
- Department of Ultrasound, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Mingxing Xie
- Department of Ultrasound, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
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Kouhashi R, Kashiwagi S, Asano Y, Morisaki T, Ishihara S, Goto W, Tanaka S, Kuwae Y, Ohsawa M, Hirakawa K, Ohira M. Breast Angiosarcoma with a Preoperative Diagnosis of Late Recurrence of Breast Cancer: A Case Report. Case Rep Oncol 2021; 14:604-609. [PMID: 33976641 PMCID: PMC8077598 DOI: 10.1159/000513906] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/10/2020] [Accepted: 12/15/2020] [Indexed: 11/19/2022] Open
Abstract
Angiosarcoma is a malignant mesenchymal tumor characterized by the presence of vascular endothelial cells. Although rare, angiosarcoma developing in the mammary glands has a poor prognosis. We report a case of breast angiosarcoma with a preoperative diagnosis of late recurrence of breast cancer. A 78-year-old woman noticed a tumor in her right breast and visited our hospital. The patient had undergone breast-conserving surgery and axillary lymph node dissection from the right breast 12 years before the visit. The tumor was diagnosed as T4bN0M0, stage IIIB. Anastrozole was administered as postoperative adjuvant therapy for 5 years; the patient also received 50-Gy whole-breast radiation therapy after surgery. Physical examination during her visit revealed an elevated lesion with blue purpura around the nipple in the right breast. We performed breast ultrasound and detected a well-defined 19.6 × 16.4 × 10.7 mm hypoechoic tumor in the left subareolar area. The patient underwent core needle biopsy (CNB). Based on the CNB specimen findings, she was suspected to experience late local recurrence after surgery. Therefore, she underwent total mastectomy after breast-conserving surgery. A dark-red tumor sized 18 × 12 mm was found in a specimen from the nipple. The pathological diagnosis of the specimen revealed short spindle-shaped tumor cells with strong nuclear pleomorphism and a significant interstitial fibrosis. Immunohistochemistry using D2-40 and CD31 antibodies showed irregular luminal proliferation at the anastomosis, infiltration into the surrounding tissue, and massive necrosis, thereby leading to the diagnosis of breast angiosarcoma. We have reported a case of breast angiosarcoma with a preoperative diagnosis of late recurrence of breast cancer.
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Affiliation(s)
- Rika Kouhashi
- Department of Breast and Endocrine Surgery, Osaka City University Graduate School of Medicine, Osaka, Japan
| | - Shinichiro Kashiwagi
- Department of Breast and Endocrine Surgery, Osaka City University Graduate School of Medicine, Osaka, Japan
| | - Yuka Asano
- Department of Breast and Endocrine Surgery, Osaka City University Graduate School of Medicine, Osaka, Japan
| | - Tamami Morisaki
- Department of Breast and Endocrine Surgery, Osaka City University Graduate School of Medicine, Osaka, Japan
| | - Sae Ishihara
- Department of Breast and Endocrine Surgery, Osaka City University Graduate School of Medicine, Osaka, Japan
| | - Wataru Goto
- Department of Breast and Endocrine Surgery, Osaka City University Graduate School of Medicine, Osaka, Japan
| | - Sayaka Tanaka
- Department of Gastrointestinal Surgery, Osaka City University Graduate School of Medicine, Osaka, Japan
| | - Yuko Kuwae
- Department of Gastrointestinal Surgery, Osaka City University Graduate School of Medicine, Osaka, Japan
| | - Masahiko Ohsawa
- Department of Gastrointestinal Surgery, Osaka City University Graduate School of Medicine, Osaka, Japan
| | - Kosei Hirakawa
- Department of Breast and Endocrine Surgery, Osaka City University Graduate School of Medicine, Osaka, Japan
| | - Masaichi Ohira
- Department of Breast and Endocrine Surgery, Osaka City University Graduate School of Medicine, Osaka, Japan
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Kokkali S, Stravodimou A, Duran-Moreno J, Koufopoulos N, Voutsadakis IA, Digklia A. Chemotherapy and targeted treatments of breast sarcoma by histologic subtype. Expert Rev Anticancer Ther 2021; 21:591-604. [PMID: 33554686 DOI: 10.1080/14737140.2021.1880327] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/22/2022]
Abstract
Introduction: Breast sarcomas (BS) are uncommon and often present both diagnostic and therapeutic challenges. Historically, radical surgery has been the mainstay of treatment for localized breast sarcomas. On the other hand, in advanced disease, since they are a heterogeneous group of neoplasms consisting of several different subtypes including angiosarcoma, phyllodes tumor, and pleomorphic undifferentiated sarcoma, there is a lack of proven specific therapy. As a result, their treatment is based on the soft tissue sarcoma (STS) paradigm, whereas histotype-tailored approaches apply to specific subtypes like dermatofibrosarcoma protuberans. To date, advanced stages constitute an incurable form of disease and chemotherapy remains the cornerstone of treatment with the aim of palliation of symptoms and increase in survival.Areas covered: In this manuscript, we review the clinicopathologic characteristics of the most common subtypes of BS, as well as the current treatment landscape of BS, with a particular focus on opportunities and challenges provided by new targeted molecules and immunotherapy.Expert opinion: The treatment approach of advanced BS is based on the pathologic subtype. A true breakthrough has still to be obtained, as the development of new agents in BS suffers from the same weaknesses as in other STS.
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Affiliation(s)
- Stefania Kokkali
- First Medical Oncology Clinic, Saint-Savvas Anticancer Hospital, Athens, Greece
| | - Athina Stravodimou
- Department of Oncology, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland
| | - Jose Duran-Moreno
- Fourth Department of Internal Medicine, Hematology Oncology Unit, Attikon University General Hospital, National and Kapodistrian University of Athens, Athens, Greece
| | - Nektarios Koufopoulos
- Second Pathology Department, National and Kapodistrian University of Athens, Attikon University General Hospital, Athens, Greece
| | - Ioannis A Voutsadakis
- Algoma District Cancer Program, Sault Area Hospital, Sault Ste. Marie, ON, Canada.,Division of Clinical Sciences, Northern Ontario School of Medicine, Sudbury, ON, Canada
| | - Antonia Digklia
- Department of Oncology, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland
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Li GZ, Raut CP, Hunt KK, Feng M, Chugh R. Breast Sarcomas, Phyllodes Tumors, and Desmoid Tumors: Epidemiology, Diagnosis, Staging, and Histology-Specific Management Considerations. Am Soc Clin Oncol Educ Book 2021; 41:390-404. [PMID: 34010054 DOI: 10.1200/edbk_321341] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
Abstract
Breast sarcomas arise from connective tissues of the breast and account for fewer than 1% of all breast malignancies. They can be subclassified as primary breast sarcomas, which arise de novo and are histologically diverse, and secondary breast sarcomas, which arise as a result of radiation or lymphedema and are most commonly angiosarcomas. Two other connective tissue neoplasms that occur within the breast include phyllodes tumors and desmoid tumors, which exhibit a spectrum of behaviors. Malignant phyllodes tumors are biologically similar to primary breast sarcomas, whereas desmoid tumors are technically benign but often locally aggressive. Patients with breast sarcomas often present with a rapidly growing mass or, in cases of radiation-associated angiosarcoma, violaceous cutaneous lesions. Core needle biopsy is generally required to confirm the diagnosis of sarcomas. Staging workup includes MRI and chest imaging, although these are not required in the case of benign phyllodes or desmoid tumors. In general, localized breast sarcomas should be resected, with the extent of resection tailored to histologic subtype. Radiation and chemotherapy can be used in the neoadjuvant or adjuvant setting, but data are limited, so treatment decisions should be made on an individualized basis. Systemic therapy options for metastatic disease and refractory breast desmoids mimic those used for the same histologies when present in other sites. Given the rarity and heterogeneity of breast sarcoma, as well as limited literature describing these entities, expert multidisciplinary evaluation is crucial for optimal decision making.
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Affiliation(s)
- George Z Li
- Department of Surgery, Brigham and Women's Hospital and Harvard Medical School, Boston, MA
| | - Chandrajit P Raut
- Department of Surgery, Brigham and Women's Hospital and Harvard Medical School, Boston, MA.,Center for Sarcoma and Bone Oncology, Dana-Farber Cancer Institute, Boston, MA
| | - Kelly K Hunt
- Department of Breast Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX
| | - Mary Feng
- Department of Radiation Oncology, University of California San Francisco, San Francisco, CA
| | - Rashmi Chugh
- Division of Hematology/Oncology, Department of Internal Medicine, University of Michigan, Ann Arbor, MI
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31
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Soren DN, Gunasekaran G, Naik D, Prusty GC, Chinnakkulam Kandhasamy S. Primary Mammary Angiosarcoma: Literature Review. Cureus 2020; 12:e8589. [PMID: 32676230 PMCID: PMC7359971 DOI: 10.7759/cureus.8589] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/15/2020] [Accepted: 06/12/2020] [Indexed: 01/13/2023] Open
Abstract
Angiosarcomas of the breast are extremely rare, highly aggressive tumors of vascular origin comprising 0.04% of all malignant neoplasms of the breast. They can be classified into primary mammary angiosarcomas and cutaneous (secondary) angiosarcomas. Primary angiosarcomas, owing to their unusual clinical presentation, are diagnosed late. In addition, the available literature to date lacks sufficient evidence to establish standard treatment guidelines for this group of tumors, thereby resulting in poor prognosis. In medical database, most available papers concern secondary angiosarcomas, with only a few case reports of primary angiosarcomas. The aim of this paper is to review what is known hitherto about the presentation, diagnostic tools, and therapeutic modalities for primary mammary angiosarcomas.
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Affiliation(s)
- Dhirendra Nath Soren
- General Surgery, Veer Surendra Sai Institute of Medical Sciences and Research, Burla, IND
| | - Gopalakrishnan Gunasekaran
- Surgical Gastroenterology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, IND
| | - Debasis Naik
- General Surgery, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, IND
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Smrke A, Hamm J, Karvat A, Simmons C, Srikanthan A. A retrospective review of 145 patients with angiosarcoma: Radiation therapy, extent of resection and chemotherapy are important predictors of survival. Mol Clin Oncol 2020; 13:179-185. [PMID: 32714543 DOI: 10.3892/mco.2020.2055] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/04/2019] [Accepted: 02/24/2020] [Indexed: 12/25/2022] Open
Abstract
Angiosarcoma is a subset of soft-tissue sarcomas with poor 5-year survival rate. Given its rarity, limited large cohort data is available for this disease. Therefore, the present study evaluated data from patients with angiosarcoma treated at a provincial Institution (BC Cancer) to determine potential modifiable predictors of survival. A retrospective review of patients across British Columbia (Canada) was conducted at the Sarcoma Outcome Unit of BC Cancer from January 1, 1969 to September 19, 2017. Cox proportional hazard models were used to calculate hazard ratios (HR) for the overall survival (OS) and progression free survival (PFS) of patients. A total of 145 patients with angiosarcoma were identified, of which 68 were metastatic/unresectable at presentation. Of the 145 patients included, 38 received chemotherapy, with 15 receiving taxane. A single patient received chemotherapy in a neoadjuvant setting. Of the resectable patients, 71 had first line surgery and 38 had curative-intent radiation during their treatment. Of the study cohort, 38 patients received prior radiation for an unrelated cancer and 4 patients had pre-existing chronic lymphedema. Resectable disease (HR, 0.22; P<0.01), first treatment with either surgery (HR, 0.08; P<0.01), radiation (HR, 0.19; P<0.01) or chemotherapy (HR, 0.22; P<0.01) were predictors of improved OS. First line surgery resulted in improved OS (HR, 0.36; P<0.01) and PFS (HR, 0.48; P<0.01). In addition, OS was positively impacted by the extent of surgery [complete (R0) vs. microscopic residual tumor (R1); HR, 0.26; P<0.01; R0 vs. macroscopic residual tumor (R2); HR, 0.08; P<0.01) resection. Extent of surgery and any radiation treatment were determined to be important predictors of OS. The results also revealed that patient outcome was improved following any treatment compared with supportive care alone. In conclusion, multidisciplinary care is critical for the treatment of patients diagnosed with angiosarcoma.
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Affiliation(s)
- Alannah Smrke
- Department of Medical Oncology, BC Cancer, Vancouver Centre, Vancouver, BC V5Z4E6, Canada
| | - Jeremy Hamm
- Department of Cancer Surveillance Outcomes, BC Cancer, Vancouver Centre, Vancouver, BC V5Z4E6, Canada
| | - Anand Karvat
- Department of Radiation Oncology, BC Cancer-Surrey, Surrey, BC V3V1Z2, Canada
| | - Christine Simmons
- Department of Medical Oncology, BC Cancer, Vancouver Centre, Vancouver, BC V5Z4E6, Canada
| | - Amirrtha Srikanthan
- Division of Medical Oncology, Ottawa Hospital Cancer Centre, Ottawa, ON K1H8L6, Canada
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Altmışdörtoğlu Ö, Gökgöz MŞ, Yalçınkaya U, Nalca Andrieu M. A Case Report of Primary Breast Angiosarcoma: Clinical Presentation and Outcome After Adjuvant Radiotherapy. Eur J Breast Health 2020; 16:290-294. [PMID: 33062971 DOI: 10.5152/ejbh.2020.4984] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/09/2019] [Accepted: 12/30/2019] [Indexed: 11/22/2022]
Abstract
Angiosarcomas of the breast are infrequent subtypes of sarcoma that are often diagnosed after radiation therapy for primary breast cancer. Primary angiosarcomas (PAS) are rare tumors that constitute 0.04% of all malignant breast tumors. We report a case of a 40-year-old woman with a lump in the right breast and diagnosed as angiosarcoma by pathological evaluation. She underwent simple mastectomy followed by adjuvant radiation. She is alive and disease-free for 66 months although tumor size was large and one surgical margin was tumor positive. Breast angiosarcoma is often in advanced stage at diagnosis and tends to recur locally. Although surgical methods constitute the primary treatment, we believe that a multidisciplinary treatment strategy should be used in high-risk patients with large primary tumors and tumor positive margins.
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Affiliation(s)
- Özgür Altmışdörtoğlu
- Department of Radiation Oncology, Near East University School of Medicine, Nicosia, Turkish Republic of Northern Cyprus
| | | | - Ulviye Yalçınkaya
- Department of Pathology, Uludağ University School of Medicine, Bursa, Turkey
| | - Meltem Nalca Andrieu
- Department of Radiation Oncology, Near East University School of Medicine, Nicosia, Turkish Republic of Northern Cyprus
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Axillary Lymph Node Dissection in Angiosarcomas of the Breast: An Asian Institutional Perspective. Sarcoma 2020; 2020:4890803. [PMID: 32300278 PMCID: PMC7136812 DOI: 10.1155/2020/4890803] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/17/2020] [Accepted: 02/25/2020] [Indexed: 12/29/2022] Open
Abstract
Angiosarcomas of the breast (ASB) are rare, making up to less than 8% of all angiosarcomas. The surgical management for this disease continues to vary throughout centres worldwide due to the current limited evidence. We aim to examine the necessity of axillary lymph node dissection in this pathology through a retrospective study of axillary metastasis and recurrence patterns in patients treated at our institution. A retrospective review of a prospectively-maintained database was performed. All adult patients with a histologically confirmed diagnosis of ASB seen at the National Cancer Centre Singapore between 2006 and 2019 were identified. Axillary lymph node status, treatment, survival, and recurrence data were collated. Thirteen patients were identified with a confirmed diagnosis of ASB, of which there were 11 primary and 2 secondary angiosarcoma cases. Eight patients had some form of axillary lymph node dissection and 5 did not. No positive nodes were found in any examined axillary nodes despite high median number of nodes harvested (13, range 8–24). 5/13 patients had disease progression, of whom none had locoregional recurrence to the axilla. ASB continues to be rare and recurrent and presents as a challenge to treat. Axillary lymph node involvement is most likely not present in a majority of patients. Prophylactic removal is unwarranted in patients presenting without lymph node involvement due to the lack of axillary metastasis.
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35
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Wu M, Huang Y, Tian W, Yao Y, Deng Y. Recurrence of Primary Breast Angiosarcoma 7 Years after Mastectomy in a 17-Year-Old Woman: A Case Report. Breast Care (Basel) 2019; 14:330-333. [PMID: 31798394 DOI: 10.1159/000493881] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/19/2022] Open
Abstract
Background Primary breast angiosarcoma is a rare neoplasm; breast angiosarcoma patients under 20 years old are extremely rare. The prognosis is often poor due to a high relapse rate after surgical resection. Case Report We report on a case of a 17-year-old woman suffering from a primary breast angiosarcoma, associated with the local recurrence of a right-breast angiosarcoma 86 months after mastectomy. She received extensive local excision and transplantation of an adjacent skin flap. The post-operation diagnosis was angiosarcoma of histological grade III. Conclusions This is a rare case as the patient was below 18 years old and the follow-up was greater than 5 years. Taken together, long-term close follow-up is extremely important, regardless of how long after surgery and the status of the surgical margins.
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Affiliation(s)
- Miaowei Wu
- Department of Surgical Oncology, the Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China
| | - Yajing Huang
- Department of Pathology, the Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China
| | - Wei Tian
- Department of Surgical Oncology, the Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China
| | - Yihan Yao
- Department of Surgical Oncology, the Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China
| | - Yongchuan Deng
- Department of Surgical Oncology, the Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China
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Primary and secondary breast angiosarcoma: single center report and a meta-analysis. Breast Cancer Res Treat 2019; 178:523-533. [PMID: 31522347 PMCID: PMC6817750 DOI: 10.1007/s10549-019-05432-4] [Citation(s) in RCA: 56] [Impact Index Per Article: 9.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/24/2019] [Accepted: 08/30/2019] [Indexed: 12/14/2022]
Abstract
Background Primary and secondary breast angiosarcoma is a rare and aggressive malignancy with limited published literature. Optimal management is mostly based on expert opinion. Our study aims to describe a single institution experience with breast angiosarcoma and evaluate other publications on this topic to further clarify prognostic outcomes and treatment modalities in this disease. Methods Twenty two cases of breast angiosarcoma from Roswell Park Comprehensive Cancer Center were retrospectively analyzed. Additionally, a systemic review and meta-analysis was conducted to study the association between survival outcomes, overall survival (OS), and recurrence-free survival (RFS) in both primary (PAS) and secondary breast angiosarcoma (SAS). Results 9 PAS patients (41%) and 13 SAS patients (59%) were retrospectively analyzed. No significant differences were noted in tumor characteristics and survival outcomes between PAS and SAS. Treatment modality had no significant effects on survival outcomes although adjuvant chemotherapy demonstrated a trend towards improved RFS in high grade tumors. 380 PAS and 595 SAS patients were included in the outcome meta-analysis. Survival outcomes were significantly worse with high grade tumors and tumor size of > 5 cm. Adjuvant radiation therapy demonstrated significantly better RFS, while adjuvant chemotherapy had no effect on survival outcomes. Conclusion Tumor size and grade seem to be reliable predictors of survival in both PAS and SAS. Mastectomy does not seem to be adding any additional benefit to BCS. Adjuvant radiation therapy showed statistically significant RFS benefit, while adjuvant chemotherapy can be beneficial in high grade tumors.
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Singh R, Chufal KS, Pahuja AK, Suresh T, Chowdhary RL, Ahmad I. Primary angiosarcoma of the breast: a radiation oncologist's perspective with a concise review of the literature. BMJ Case Rep 2019; 12:12/7/e227036. [PMID: 31324666 DOI: 10.1136/bcr-2018-227036] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/25/2022] Open
Abstract
A 28-year-old premenopausal woman presented with a painful rapidly growing mass in her right breast and was evaluated with a core needle biopsy, which was suggestive of poorly differentiated carcinoma. Immunohistochemical evaluation revealed primary angiosarcoma of breast. Whole body 18flouro-deoxyglucose positron emission tomography CT showed few metabolically active soft tissue lesions in upper inner quadrant of right breast. The patient underwent breast conservation surgery and in view of positive surgical margins, received adjuvant radiation therapy. Post-treatment completion, the patient has been disease free for 6 months. Primary angiosarcoma of the breast is a rare malignancy which is best managed with a surgery as first approach, with due importance being given to the patient's perspective on their disease and choices for adjuvant treatment. Decisions in addition to adjuvant radiotherapy need to be made in the multidisciplinary clinic, due to paucity of data.
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Affiliation(s)
- Rajpal Singh
- Radiation Oncology, Rajiv Gandhi Cancer Institute and Research Centre, New Delhi, Delhi, India
| | - Kundan Singh Chufal
- Radiation Oncology, Rajiv Gandhi Cancer Institute and Research Centre, New Delhi, Delhi, India
| | - Anjali K Pahuja
- Radiation Oncology, Rajiv Gandhi Cancer Institute and Research Centre, New Delhi, Delhi, India
| | - Tamilarasu Suresh
- Radiation Oncology, Rajiv Gandhi Cancer Institute and Research Centre, New Delhi, Delhi, India
| | - Rahul Lal Chowdhary
- Radiation Oncology, Rajiv Gandhi Cancer Institute and Research Centre, New Delhi, Delhi, India
| | - Irfan Ahmad
- Radiation Oncology, Rajiv Gandhi Cancer Institute and Research Centre, New Delhi, Delhi, India
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Clinical Outcomes and Prognostic Features of Angiosarcoma: Significance of Prior Radiation Therapy. Clin Oncol (R Coll Radiol) 2019; 31:232-241. [PMID: 30718086 DOI: 10.1016/j.clon.2019.01.006] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/09/2018] [Revised: 11/14/2018] [Accepted: 12/04/2018] [Indexed: 02/06/2023]
Abstract
AIMS Angiosarcoma is a rare and aggressive malignancy with a poor prognosis. There is limited literature describing prognostic factors and guidelines for treatment. We aim to describe outcomes in angiosarcoma, including the impact of patient-, tumour- and treatment-related factors on prognosis. MATERIALS AND METHODS Patients with non-metastatic angiosarcoma diagnosed between 2008 and 2017 were retrospectively reviewed. Univariable and multivariable Cox proportional hazards methods were used to evaluate factors associated with locoregional recurrence, distant failure and overall survival. The Kaplan-Meier method and log-rank statistics were used to compare outcomes among patients with and without a history of prior radiation therapy. RESULTS The cohort included 65 patients. The median age at diagnosis was 68 years (35-93). Nineteen patients had a history of receiving prior radiation therapy at the anatomic location of their angiosarcoma. Treatment modalities included surgery (n = 19), surgery + radiation therapy (n = 12), surgery + chemotherapy (n = 8), chemotherapy + radiation therapy (n = 7) and all three modalities (n = 14). The median follow-up was 18 (2-192) months. The 2-year locoregional control, distant control and overall survival were 61.8, 63.6 and 58.9%, respectively. On multivariable analysis, a history of previous radiation therapy was associated with inferior outcomes with respect to locoregional recurrence (hazard ratio 89.67, 95% confidence interval 8.45-951.07, P < 0.001), distant failure (hazard failure 3.74, 95% confidence interval 1.57-8.91, P = 0.003) and overall survival (hazard ratio 3.89, 95% confidence interval 1.56-9.60, P = 0.003). In patients with primary angiosarcoma, the rates of locoregional control, distant control and overall survival were 72.4, 73.4 and 65.1%, respectively, compared with 31.9, 41.1 and 45.1% in patients with radiation therapy-induced angiosarcoma (P = 0.001). CONCLUSION Angiosarcomas that arise as a result of previous radiation therapy have worse outcomes compared with primary angiosarcomas. Although selection bias and compromise of clinical care in radiation therapy-induced angiosarcoma are partially to blame, differences in genomic profiles of the tumours need to be characterised to evaluate the underlying biological differences, as this may guide future treatment management. This study adds to the existing body of literature on angiosarcoma. Results from the current study are presented alongside previously published data to further characterise outcomes and prognostic factors on this rare and aggressive malignancy.
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Im S, Chae BJ, Kim SH, Kang BJ, Song BJ, Lee A. Primary angiosarcoma of the breast: a case report. INTERNATIONAL JOURNAL OF CLINICAL AND EXPERIMENTAL PATHOLOGY 2019; 12:664-668. [PMID: 31933872 PMCID: PMC6945096] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Subscribe] [Scholar Register] [Received: 09/06/2018] [Accepted: 09/27/2018] [Indexed: 06/10/2023]
Abstract
BACKGROUND Primary angiosarcoma of the breast is extremely rare, accounting for less than 0.05% of all primary malignancies of the breast. Here, we report here a case of primary angiosarcoma with full description of radiology and histology, including electron microscopic findings. CASE PRESENTATION A 39-year-old woman complained of a diffuse hard mass in her right breast. She did not have any history of radiation exposure. Ultrasonography revealed a 7 cm sized mass with an irregular anechoic cystic portion replacing the entire right breast. Modified radical mastectomy was performed. The diagnosis of intermediate grade angiosarcoma was made by microscopic examination, immunohistochemical staining, and electron microscopic examination. The patient underwent four cycles of adriamycin-ifosfamide chemotherapy and received radiation therapy. Multiple bone metastases occurred 9 months after surgery and palliative treatment was given. Follow up was lost at post-operative 22 months. CONCLUSIONS We report a rare case of intermediate grade primary angiosarcoma with detailed radiological and histological findings. Despite postoperative chemoradiation therapy, multiple metastases suggest that intermediate grade may have a more aggressive behavior.
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Affiliation(s)
- Soyoung Im
- Department of Pathology, St. Vincent’s Hospital, The Catholic University of Korea, College of MedicineSuwon, Republic of Korea
| | - Byung Joo Chae
- Department of Surgery, Seoul St. Mary’s Hospital, The Catholic University of Korea, College of MedicineSeoul, Republic of Korea
| | - Sung Hun Kim
- Department of Radiology, Seoul St. Mary’s Hospital, The Catholic University of Korea, College of MedicineSeoul, Republic of Korea
| | - Bong-Joo Kang
- Department of Radiology, Seoul St. Mary’s Hospital, The Catholic University of Korea, College of MedicineSeoul, Republic of Korea
| | - Byung Joo Song
- Department of Surgery, Bucheon St. Mary’s Hospital, The Catholic University of Korea, College of MedicineBucheon, Republic of Korea
| | - Ahwon Lee
- Department of Hospital Pathology, Seoul St. Mary’s Hospital, The Catholic University of Korea, College of MedicineSeoul, Republic of Korea
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Amajoud Z, Vertongen AS, Weytens R, Hauspy J. Radiation induced angiosarcoma of the breast: case series and review of the literature. Facts Views Vis Obgyn 2018; 10:215-220. [PMID: 31367294 PMCID: PMC6658205] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/21/2022] Open
Abstract
Radiation therapy (RT) is an essential adjuvant treatment in early stage breast cancer decreasing the risk of local recurrence. One of the rare late complications of RT is the development of a second primary tumor in the form of radiation-induced angiosarcoma (RIAS). In this report, we present a series of cases of RIAS at a single center and discuss the presentation, management and outcome of this rare iatrogenic malignancy. We conducted a retrospective data analysis of all diagnosed RIAS at the GZA Sint Augustinus Hospital between 2008 and 2018 (n=10). Additionally, a literature search was done. The women were between 64 to 86 years old (mean 73 years). Median follow up was 13,0 months [range 6-96 months] The latency period till RIAS ranged from 4.1 to 14.9 years (average 7.3 years). All tumors, with various clinical presentations were located in the radiation field with sizes from 1 to 10 cm. Nine patients had surgery. Disease-free interval for first recurrence of RIAS was 2-51 months (median 4 months). Overall survival for 1, 2 and five years is respectively 80, 69 and 46%. Comparable numbers were found in the literature. In conclusion, RIAS can occur beyond the conventional 5-year oncological follow-up. Long-term follow-up is necessary with particular attention to post irradiation skin lesions to ensure early detection and prompt therapeutic intervention. Surgery is the golden standard, however the role of chemotherapy and/or RT remains ambiguous. Further investigation is needed.
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Affiliation(s)
- Z Amajoud
- Departement of gynecology and obstetrics, GZA Sint Augustinus, Antwerp
| | | | - R Weytens
- Departement of radiotherapy, GZA Sint Augustinus, Antwerp
| | - J Hauspy
- Departement of gynecologic oncology, GZA Sint Augustinus, Antwerp
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Abstract
RATIONALE As a very rare vascular tumor, breast angiosarcoma (AS) can be divided into primary and second breast AS. However, the latter is slightly more commonly detected in clinical practice. Radiation post mastectomy is the common cause for the secondary breast AS, and although there are other reasons, it is still quite rare. In the present study, we reported a rare case of breast AS and summarized the relevant literatures so that to conduce to diagnose AS. PATIENT CONCERNS A 50-year-old female with a history of right breast neoplasm was treated with repeat lumpectomy for 4 times during 8 years. DIAGNOSES Mammogram and ultrasound examination demonstrated a possible malignancy (BIRADS-4B and BI-RADS-4C, respectively). Immunohistochemically positive for endothelial markers CD31, CD34, ERG, and FVIII-R-Ag. INTERVENTIONS The patient underwent a right mastectomy with sentinel lymph node biopsy by our multidisciplinary team and no other therapy was given postsurgery. OUTCOMES The patient had no recurrence after 3 months. LESSONS Based on our findings, we concluded that repeated resection might be a risk factor for the breast AS, especially for a gradual pathological evolution from benign to malignant. This case showed a very rare cause for angiomatosis of breast, and the patient had a successful outcome after a simple mastectomy.
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Primary Angiosarcoma of the Breast after Bilateral Breast Reduction. Case Rep Surg 2018; 2018:7390987. [PMID: 29977639 PMCID: PMC6011124 DOI: 10.1155/2018/7390987] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/29/2018] [Accepted: 04/15/2018] [Indexed: 11/17/2022] Open
Abstract
Angiosarcoma of the breast is a rare malignancy of endothelial cell origin, representing less than 1% of all breast malignancy. Primary angiosarcomas can occur in the setting of chronic lymphedema, but it also may occur spontaneously without any preceding treatment. Surgery is the primary therapeutic intervention for breast angiosarcomas with radiation and chemotherapy as adjuvant treatment. Angiosarcomas are aggressive and tend to have a high risk of local and metastatic recurrence. We present a case of primary angiosarcoma that developed in a patient who had bilateral breast reduction surgery in the past.
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Abstract
OPINION STATEMENT Angiosarcomas are rare vascular neoplasms that are among the most aggressive subtypes of soft tissue sarcomas. Surgical resection is often challenging even in localized disease, as the infiltrative nature of these cancers leads to frequent local and metastatic recurrences. Cytotoxic chemotherapy, including anthracycline-based regimens and taxanes can produce significant responses in a subset of patients but durability is limited with most patients ultimately succumbing to metastatic disease. Targeted therapy with tyrosine kinase inhibitors is usually well-tolerated but prone to development of resistance. Few head-to-head trials have addressed the optimal sequence of therapies, or demonstrated conclusive benefits of one therapy over another based on clinical and etiologic factors. Novel therapies in clinical trials, including antibodies to endoglin and checkpoint inhibitors have demonstrated exciting early activity in patients with angiosarcoma. Improved understanding of the genetic heterogeneity within various angiosarcoma subtypes may identify predictive biomarkers to match patients to effective existing and future therapies. Overall, angiosarcoma patients with optimal performance status are best served in clinical trials that incorporate novel combinations of cytotoxic chemotherapy, targeted therapies, and immunotherapies.
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Kunkiel M, Maczkiewicz M, Jagiełło-Gruszfeld A, Nowecki Z. Primary angiosarcoma of the breast-series of 11 consecutive cases-a single-centre experience. Curr Oncol 2018; 25:e50-e53. [PMID: 29507495 PMCID: PMC5832291 DOI: 10.3747/co.25.3816] [Citation(s) in RCA: 21] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/21/2022] Open
Abstract
Angiosarcomas of the breast are rare parenchymal malignancies of the chest wall. Surgery is the main treatment modality with chemotherapy and radiotherapy used in case of recurrence. With generally unfavourable prognosis and lack of clear treatment guidelines due to its rarity and scarcity of available data, angiosarcoma of the breast is a challenging clinical situation for both oncologist and patient. We present here the results of a series of 11 consecutive primary angiosarcoma cases treated at our institute between 2000 and 2015.
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Affiliation(s)
- M. Kunkiel
- Department of Breast Cancer and Reconstructive Surgery, Maria Sklodowska-Curie Memorial Cancer Center and Institute of Oncology, Warsaw, Poland
| | - M. Maczkiewicz
- Department of Breast Cancer and Reconstructive Surgery, Maria Sklodowska-Curie Memorial Cancer Center and Institute of Oncology, Warsaw, Poland
| | - A. Jagiełło-Gruszfeld
- Department of Breast Cancer and Reconstructive Surgery, Maria Sklodowska-Curie Memorial Cancer Center and Institute of Oncology, Warsaw, Poland
| | - Z. Nowecki
- Department of Breast Cancer and Reconstructive Surgery, Maria Sklodowska-Curie Memorial Cancer Center and Institute of Oncology, Warsaw, Poland
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45
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Gambini D, Visintin R, Locatelli E, Galassi B, Bareggi C, Runza L, Onida F, Tomirotti M. Paclitaxel-Dependent Prolonged and Persistent Complete Remission Four Years from First Recurrence of Secondary Breast Angiosarcoma. TUMORI JOURNAL 2018; 95:828-31. [DOI: 10.1177/030089160909500631] [Citation(s) in RCA: 20] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/12/2023]
Abstract
Among angiosarcomas, radiation-induced breast sarcomas (RIBS) represent a well-known entity generally characterized by a poor outcome, especially in patients with advanced disease. Despite the unfavorable prognosis, some chemotherapeutic agents have been used to treat these malignancies, occasionally with success. Treatments with demonstrated activity against sarcomas include ifosfamide-based regimens and, more recently, taxane derivatives. We report a case of a patient having a secondary breast angiosarcoma recurring early after surgery, who achieved complete remission following treatment with weekly paclitaxel. After 4 years of maintenance therapy, with an interval between consecutive administrations of no longer than 3 weeks, the patient is still in complete remission. A locoregional recurrence was documented twice during this period, the first as a consequence of a brief treatment interruption and the second because of a treatment delay. Nonetheless, in both instances a new complete remission was rapidly achieved with the resumption of the same treatment, without evidence of any significant adverse effects. We discuss the highly unusual behavior of this malignancy and the possible role of the two different mechanisms of action of paclitaxel-antiangiogenic versus cytotoxic-depending on the schedule of administration, with evidence of “false” drug-resistance.
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Affiliation(s)
- Donatella Gambini
- Medical Oncology Unit, IRCCS Foundation “Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena”, Milan
| | - Roberto Visintin
- Medical Oncology Unit, IRCCS Foundation “Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena”, Milan
| | - Elisa Locatelli
- Medical Oncology Unit, IRCCS Foundation “Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena”, Milan
| | - Barbara Galassi
- Medical Oncology Unit, IRCCS Foundation “Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena”, Milan
| | - Claudia Bareggi
- Medical Oncology Unit, IRCCS Foundation “Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena”, Milan
| | - Letterio Runza
- Pathology Unit, IRCCS Foundation “Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena”, Milan
| | - Francesco Onida
- Department of Medical Sciences, University of Milan, Milan, Italy
| | - Maurizio Tomirotti
- Medical Oncology Unit, IRCCS Foundation “Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena”, Milan
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Lokanatha D, Anand A, Lakshmaiah KC, Govind Babu K, Jacob LA, Suresh Babu MC, Lokesh KN, Rudresha AH, Rajeev LK, Saldanha SC, Giri GV, Koppaka D, Kumar RV. Primary breast angiosarcoma - a single institution experience from a tertiary cancer center in South India. Breast Dis 2018; 37:133-138. [PMID: 29286911 DOI: 10.3233/bd-170291] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 06/07/2023]
Abstract
INTRODUCTION Primary angiosarcoma of the breast is a rare entity with incidence of less than 0.05% of all malignant breast neoplasms. It occurs in young females without any associated risk factors. The tumor behaves aggressively and has a poor prognosis compared to invasive ductal carcinoma. METHOD It was a retrospective observational study done at a tertiary cancer center from January 2012 to December 2016. The medical records of patients diagnosed with primary breast angiosarcoma were reviewed for the study. Clinicopathological profile, treatment, and the outcomes were analyzed. RESULTS Four patients were diagnosed with primary breast angiosarcoma out of 2560 breast cancer patients seen over a period of 5 years. Two had metastatic disease at presentation. Among four patients, two underwent surgery of the primary tumor, whereas, all received chemotherapy either as adjuvant or palliative setting. One patient received adjuvant radiation therapy. Three patients received 2nd line and one received 3rd line chemotherapy on disease progression. After a median follow-up of 18 months one patient was surviving on 3rd line chemotherapy with trabectedin. Other three succumbed to disease after progression. CONCLUSION Due to a small number of this malignancy randomized studies are difficult to perform and optimum treatment strategy still need to be defined.
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Affiliation(s)
- D Lokanatha
- Department of Medical Oncology, Kidwai Cancer Institute, Bangalore, 560029, India
| | - Abhishek Anand
- Department of Medical Oncology, Kidwai Cancer Institute, Bangalore, 560029, India
| | - K C Lakshmaiah
- Department of Medical Oncology, Kidwai Cancer Institute, Bangalore, 560029, India
| | - K Govind Babu
- Department of Medical Oncology, Kidwai Cancer Institute, Bangalore, 560029, India
| | - Linu Abraham Jacob
- Department of Medical Oncology, Kidwai Cancer Institute, Bangalore, 560029, India
| | - M C Suresh Babu
- Department of Medical Oncology, Kidwai Cancer Institute, Bangalore, 560029, India
| | - K N Lokesh
- Department of Medical Oncology, Kidwai Cancer Institute, Bangalore, 560029, India
| | - A H Rudresha
- Department of Medical Oncology, Kidwai Cancer Institute, Bangalore, 560029, India
| | - L K Rajeev
- Department of Medical Oncology, Kidwai Cancer Institute, Bangalore, 560029, India
| | - Smitha C Saldanha
- Department of Medical Oncology, Kidwai Cancer Institute, Bangalore, 560029, India
| | - G V Giri
- Department of Medical Oncology, Kidwai Cancer Institute, Bangalore, 560029, India
| | - Deepak Koppaka
- Department of Medical Oncology, Kidwai Cancer Institute, Bangalore, 560029, India
| | - Rekha V Kumar
- Department of Pathology, Kidwai Cancer Institute, Bangalore, 560029, India
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Investigation of Prognostic Features in Primary Cutaneous and Soft Tissue Angiosarcoma After Surgical Resection: A Retrospective Study. Ann Plast Surg 2017; 78:S41-S46. [PMID: 28118230 DOI: 10.1097/sap.0000000000001004] [Citation(s) in RCA: 16] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/04/2023]
Abstract
Primary cutaneous and soft tissue angiosarcoma is a rare but highly aggressive malignancy. To date, surgical resection is the mainstay of treatment, but poor prognosis is expected. To investigate whether there are factors associated with poor prognosis after surgical resection and to develop a treatment guideline for current therapy, we retrospectively collected data on 28 patients who underwent surgery as initial treatment and reviewed patient demographics, tumor characteristics, disease courses, and prognoses from September 1996 to May 2013. Of these 28 patients, 17 (60.7%) were men and the mean age at first diagnosis was 66.57 ± 18.57 years. Anatomically, 17 (60.7%) tumors were in the scalp and 11 (39.3%) were in other sites of the body. Of the 28 patients, 23 (82.1%) had achieved negative surgical margins, 24 (85.7%) received adjuvant radiation therapy, and 17 (60.7%) received adjuvant chemotherapy. Twenty-one patients (75%) died during a mean follow-up time of 35.86 ± 28.91 months, and all deaths were caused by angiosarcoma. The 5-year overall survival rate was 17.86%. Sixteen (57.1%) patients had locoregional tumor recurrence, and 20 (71.4%) had distant metastases, with a median of 9.17 (range, 1.9-98.07) months to recurrence or metastasis. Possible predictors of poor prognosis (P < 0.05) in terms of disease-free survival after surgical resection were male sex, cardiovascular disease, smoking, and scalp angiosarcomas, those in terms of overall survival were older than 70 years, male sex, cardiovascular disease, smoking, scalp angiosarcomas, distant metastases, and not receiving adjuvant chemotherapy. In conclusion, although multimodal treatments are used, the overall prognosis after surgical resection is still poor, especially for patients with the above predictive factors. An early diagnosis and complete resection of the primary tumor with or without adjuvant radiotherapy and chemotherapy are suggested for a potential better outcome. For those who have a diffuse lesion pattern with the involvement of vital structures, recurrence, or metastasis, palliative resection could be an alternative treatment choice.
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Wang L, Lao IW, Yu L, Wang J. Clinicopathological features and prognostic factors in angiosarcoma: A retrospective analysis of 200 patients from a single Chinese medical institute. Oncol Lett 2017; 14:5370-5378. [PMID: 29113171 PMCID: PMC5656021 DOI: 10.3892/ol.2017.6892] [Citation(s) in RCA: 19] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/18/2015] [Accepted: 05/23/2017] [Indexed: 12/20/2022] Open
Abstract
Angiosarcoma is a rare soft tissue sarcoma, and the data about its clinicopathological features and prognostic factors are limited. The purpose of the present study was to report a large series of angiosarcoma at a single institution. Clinical data from 200 cases of angiosarcoma from the Shanghai Cancer Center (Shanghai, China) between March 2006 and March 2014 were retrospectively analyzed. The study population included 97 males and 103 females with ages between 4 and 91 years (median, 53 years). According to the tumor location, 200 cases were divided into 4 groups: i) Tumors involving the head and neck; ii) breast; iii) viscera (including internal organs and bone); and iv) soft tissue (including trunk and extremities). Of the 113 patients with follow-up data, 46 patients succumbed to the disease with a median interval of 10 months. Tumor recurrence/metastasis was identified in 66 patients with a median interval of 4 months. The disease-free survival (DFS) rate at 5-years was 19.3% and the overall survival (OS) rate at 5-years was 40.8%. Site of tumor origin, size (≥5 cm) and histological differentiation influenced DFS (P=0.032, 0.038 and <0.001, respectively), and OS (P<0.001, 0.008 and <0.001, respectively) rates. Age (<65 years) and multimodal treatment correlated with improved OS (P=0.003 and <0.001, respectively). Tumor differentiation and treatment modality were identified to be independent determinants of OS (P<0.001 and 0.038, respectively). Tumor recurrence/metastasis was an independent predictor of DFS (P<0.001). The prognosis of angiosarcoma is poor and the mortality rate is high. The site of tumor origin, size, histological differentiation, age, treatment modality and tumor recurrence/metastasis are all significant prognostic factors. In the present study, multimodal treatment may improve the clinical outcome of patients with angiosarcoma.
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Affiliation(s)
- Lei Wang
- Department of Pathology, Fudan University Shanghai Cancer Center, Shanghai 200032, P.R. China.,Department of Oncology, Shanghai Medical College, Fudan University, Shanghai 200433, P.R. China
| | - I Weng Lao
- Department of Pathology, Fudan University Shanghai Cancer Center, Shanghai 200032, P.R. China.,Department of Oncology, Shanghai Medical College, Fudan University, Shanghai 200433, P.R. China
| | - Lin Yu
- Department of Pathology, Fudan University Shanghai Cancer Center, Shanghai 200032, P.R. China.,Department of Oncology, Shanghai Medical College, Fudan University, Shanghai 200433, P.R. China
| | - Jian Wang
- Department of Pathology, Fudan University Shanghai Cancer Center, Shanghai 200032, P.R. China.,Department of Oncology, Shanghai Medical College, Fudan University, Shanghai 200433, P.R. China
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Core Biopsy of Vascular Neoplasms of the Breast: Pathologic Features, Imaging, and Clinical Findings. Am J Surg Pathol 2017; 40:1424-34. [PMID: 27340752 DOI: 10.1097/pas.0000000000000668] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
Abstract
Vascular lesions (VLs) of the breast present a diagnostic challenge on breast core biopsy (BCBx). We report on 27 VLs presenting on BCBx. The mean patient age was 60 years, and mean size was 7.5 mm (range, 1.6 to 16 mm). Presentation included palpable mass in 6 (22%), incidental in 6 (22%), and an imaging abnormality in 15 (56%) cases. Imaging impression included hematoma (24%), lymph node (10%), fat necrosis (10%), tortuous vessel (5%), and not provided in 52%. The lesions were classified on the basis of BCBx or BCBx and excision (available in 16 pts) as follows: 1 low-grade angiosarcoma, 8 angiolipomas, 6 capillary hemangiomas, 4 cavernous hemangiomas, 2 hemangiomas (not otherwise specified), 1 papillary endothelial hyperplasia, and 5 perilobular hemangiomas. The angiosarcoma was 9 mm, detected incidentally by magnetic resonance imaging, and showed dissection of stromal collagen, infiltration of glands, high cellularity, moderate cytologic atypia, scant mitotic activity, and Ki-67 reactivity of 10%. Among the 26 benign VLs, worrisome histologic features were noted in 14 on BCBx, including anastomosing vascular channels in 9, moderate cytologic atypia in 4, high cellularity in 2, Ki-67>10% in 2, mitotic activity in 1, and infiltration of glands in 1. Of the 12 VLs without worrisome features, the lesion extended to edge of core in 8, precluding complete evaluation. BCBx of VLs presents diagnostic challenges due to overlapping clinicopathologic and radiologic features with low-grade angiosarcoma. If completeness of removal is documented on BCBx, and cytoarchitectural changes are not worrisome, follow-up could be considered rather than excision. However, only 4 of these cases fulfilled those criteria.
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Vascular tumours of the breast: a comprehensive review with focus on diagnostic challenges encountered in the core biopsy setting. Pathology 2016; 49:197-214. [PMID: 28049578 DOI: 10.1016/j.pathol.2016.11.003] [Citation(s) in RCA: 17] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/04/2016] [Revised: 11/04/2016] [Accepted: 11/06/2016] [Indexed: 12/20/2022]
Abstract
Vascular proliferations of the breast comprise a spectrum of benign and malignant lesions. In limited samples, such as core needle biopsies (CNB), these lesions may be difficult to distinguish due to significant overlap in morphological features. As the treatment and prognosis of these entities vary widely, it is important for pathologists to consider a complete differential diagnosis and correctly synthesise histological features, results of adjunctive immunohistochemical studies, and pertinent clinical and imaging information, to render an accurate diagnosis in such limited samples. The diagnostic pitfalls of under- or overdiagnosis of vascular lesions sampled in CNB will also be discussed.
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