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Winer LK, Fredette JD, Hasler J, Akumuo R, Greco SH, von Mehren M, Bartholomew AJ, Blazer DG, Gabrielova L, Adamkova D, Bernard-Bedard E, Nessim C, Kollár A, Olariu R, Cencelj-Arnez R, Hompes D, Ford SJ, Cardona K, Sato K, Iwata S, Farma JM, Villano AM. Examining the Impact of Biopsy Technique on Clinical and Pathologic Outcomes in Dermatofibrosarcoma Protuberans: An International, Multi-Institutional Study. Ann Surg Oncol 2025:10.1245/s10434-025-17011-7. [PMID: 40014211 DOI: 10.1245/s10434-025-17011-7] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/01/2024] [Accepted: 01/28/2025] [Indexed: 02/28/2025]
Abstract
BACKGROUND The National Comprehensive Cancer Network (NCCN) guidelines recommend preoperative biopsy for diagnosing dermatofibrosarcoma protuberans (DFSP) but limited data support this approach. We characterized DFSP diagnostic practices and compared clinical outcomes based on technique. METHODS Data were collected for adult patients who underwent resection for initial DFSP presentation between 2003 and 2021 at 10 international institutions. Patients were categorized by excisional versus preoperative biopsy (incisional, punch, core needle biopsies, or fine needle aspiration), and univariate and multivariable analyses were performed. RESULTS The cohort included 321 patients, with excisional biopsy performed in 51.4% and preoperative biopsy performed in 48.6% of patients. Biopsy type was stable throughout the study period (p = 0.08). There were no differences in sex, disease presentation, or preoperative imaging. In unadjusted analysis, biopsy varied by practitioner specialty, with general surgeons performing nearly 50% of excisional biopsies. Despite similar planned circumferential margins and anatomic location, preoperative biopsy was associated with higher index R0 rate (60.1% vs. 78.6%), fewer total excisions, and fewer complications (38.2% vs. 25.6%, all p < 0.05). However, adjuvant radiotherapy (11.7% vs. 6.0%) and final R0 rates (91.5% vs. 88.4%) were comparable regardless of technique (p > 0.05). In adjusted analysis, excisional biopsy was associated with extremity tumors (odds ratio [OR] 1.79, confidence interval [CI] 1.21-2.66, p = 0.004), treatment in non-academic settings (OR 2.28, CI 1.10-4.73, p = 0.03), and inversely with preoperative imaging (OR 0.47, CI 0.24-0.93, p = 0.03). CONCLUSION Preoperative biopsy is associated with margin-negative resection, fewer re-excisions, and reduced complications. Clinical suspicion of DFSP is paramount, and preoperative imaging may critically inform biopsy selection prior to index resection.
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Affiliation(s)
- Leah K Winer
- Division of Surgical Oncology, Fox Chase Cancer Center, Philadelphia, PA, USA
| | - Jordan D Fredette
- Division of Biostatistics and Bioinformatics, Fox Chase Cancer Center, Philadelphia, PA, USA
| | - Jill Hasler
- Division of Biostatistics and Bioinformatics, Fox Chase Cancer Center, Philadelphia, PA, USA
| | - Rita Akumuo
- Department of General Surgery, Temple University Hospital, Philadelphia, PA, USA
| | - Stephanie H Greco
- Division of Surgical Oncology, Fox Chase Cancer Center, Philadelphia, PA, USA
| | - Margaret von Mehren
- Division of Hematology and Medical Oncology, Fox Chase Cancer Center, Philadelphia, PA, USA
| | | | - Dan G Blazer
- Department of Surgery, Duke University Hospital, Durham, NC, USA
| | - Lucie Gabrielova
- Department of Surgery and Clinic of Comprehensive Cancer Care, Masaryk Memorial Cancer Institute, Brno, Czech Republic
| | - Dagmar Adamkova
- Department of Surgery and Clinic of Comprehensive Cancer Care, Masaryk Memorial Cancer Institute, Brno, Czech Republic
| | | | - Carolyn Nessim
- Division of Surgical Oncology, Ottawa Hospital, Ottawa, ON, Canada
| | - Attila Kollár
- Department of Medical Oncology, Bern University Hospital, University of Bern, Bern, Switzerland
| | - Radu Olariu
- Department of Plastic and Hand Surgery, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland
| | - Romi Cencelj-Arnez
- Division of Surgery, Institute of Oncology Ljubljana, Ljubljana, Slovenia
| | - Daphne Hompes
- Department of Surgical Oncology, University Hospitals Leuven, Leuven, Belgium
| | - Samuel J Ford
- Sarcoma Unit, Queen Elizabeth Hospital, Birmingham, UK
| | - Kenneth Cardona
- Division of Surgical Oncology, Emory University Hospital, Atlanta, GA, USA
| | - Kenji Sato
- Department of Musculoskeletal Oncology, National Cancer Center Hospital, Tokyo, Japan
| | - Shintaro Iwata
- Department of Musculoskeletal Oncology, National Cancer Center Hospital, Tokyo, Japan
| | - Jeffrey M Farma
- Division of Surgical Oncology, Fox Chase Cancer Center, Philadelphia, PA, USA
| | - Anthony M Villano
- Division of Surgical Oncology, Fox Chase Cancer Center, Philadelphia, PA, USA.
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Winer LK, Akumuo R, Fredette JD, Deng M, Hasler JS, Greco SH, von Mehren M, Bartholomew AJ, Blazer DG, Gabrielova L, Adamkova D, Bernard-Bedard E, Nessim C, Kollár A, Olariu R, Cencelj-Arnez R, Hompes D, Ford SJ, Cardona K, Sato K, Iwata S, Farma JM, Villano AM. Current patterns of care and outcomes for dermatofibrosarcoma protuberans: An international multi-institutional collaborative. Cancer 2025; 131:e35468. [PMID: 38985726 DOI: 10.1002/cncr.35468] [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] [Received: 01/20/2024] [Revised: 04/26/2024] [Accepted: 05/31/2024] [Indexed: 07/12/2024]
Abstract
BACKGROUND Dermatofibrosarcoma protuberans (DFSP) is a cutaneous sarcoma with an infiltrative growth pattern that makes it challenging to clear margins. High quality data regarding DFSP natural history, management, and outcomes are limited. METHODS Data were retrospectively collected for adult DFSP patients who underwent resection at 10 institutions in eight countries. Demographics, tumor characteristics, treatment strategies, and outcomes were analyzed. RESULTS Analysis included 347 patients consisting of young (median, 42 years), White (76.2%), males (54.2%) with truncal lesions (57.3%). The majority (76.8%) were symptomatic at presentation. Preoperative imaging was used in 55.9% of cases. Diagnosis was established with excisional biopsy in 50.9% versus incisional biopsy in 25.0% of cases. Despite planned margins of >1.0 cm in 67.4% of cases, only 69.0% of patients achieved R0 resection. Twenty-two percent of patients underwent at least one re-excision. R0 resection was achieved at a second procedure in 80.2% and a third procedure in 86.2%. Ultimately, R0 resection was feasible in 89.5% of all patients. Fibrosarcomatous transformation (FST) was observed in 12.6%. In total, 6.6% (N = 23) recurred (17 local, six distant). Of the six distant recurrences, 50.0% had FST. With a median follow-up of 47.0 months, disease-specific survival rate was 98.8%. In multivariable analysis, R0 margins at index resection were associated with wider circumferential margins and non-FST histology. CONCLUSIONS In this international, multicenter collaborative, DFSP practice patterns were heterogeneous but achieved favorable recurrence rates and survival. Multiple excisions to clear margins remain commonplace and can inform future efforts to optimize margin selection.
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Affiliation(s)
- Leah K Winer
- Division of Surgical Oncology, Fox Chase Cancer Center, Philadelphia, Pennsylvania, USA
| | - Rita Akumuo
- Department of General Surgery, Temple University Hospital, Philadelphia, Pennsylvania, USA
| | - Jordan D Fredette
- Division of Biostatistics and Bioinformatics, Fox Chase Cancer Center, Philadelphia, Pennsylvania, USA
| | - Mengying Deng
- Division of Biostatistics and Bioinformatics, Fox Chase Cancer Center, Philadelphia, Pennsylvania, USA
| | - Jill S Hasler
- Division of Biostatistics and Bioinformatics, Fox Chase Cancer Center, Philadelphia, Pennsylvania, USA
| | - Stephanie H Greco
- Division of Surgical Oncology, Fox Chase Cancer Center, Philadelphia, Pennsylvania, USA
| | - Margaret von Mehren
- Division of Medical Oncology, Fox Chase Cancer Center, Philadelphia, Pennsylvania, USA
| | - Alex J Bartholomew
- Department of Surgery, Duke University Hospital, Durham, North Carolina, USA
| | - Dan G Blazer
- Department of Surgery, Duke University Hospital, Durham, North Carolina, USA
| | - Lucie Gabrielova
- Department of Surgery, Masaryk Memorial Cancer Institute, Brno, Zluty kopec, Czech Republic
| | - Dagmar Adamkova
- Department of Surgery, Masaryk Memorial Cancer Institute, Brno, Zluty kopec, Czech Republic
| | | | - Carolyn Nessim
- Division of Surgical Oncology, Ottawa Hospital, Ottawa, Ontario, Canada
| | - Attila Kollár
- Department of Medical Oncology, Inselspital, Bern University Hospital, Univesity of Bern, Bern, Switzerland
| | - Radu Olariu
- Department of Plastic and Hand Surgery, Inselspital, Bern University Hospital, Freiburgstrasse, Switzerland
| | - Romi Cencelj-Arnez
- Division of Surgery, Institute of Oncology Ljubljana, Ljubljana, Slovenia
| | - Daphne Hompes
- Department of Surgical Oncology, University Hospitals Leuven, Leuven, Belgium
| | - Samuel J Ford
- Sarcoma Unit, Queen Elizabeth Hospital, Birmingham, United Kingdom
| | - Kenneth Cardona
- Division of Surgical Oncology, Emory University Hospital, Atlanta, Georgia, USA
| | - Kenji Sato
- Department of Musculoskeletal Oncology, National Cancer Center Hospital, Tokyo, Japan
| | - Shintaro Iwata
- Department of Musculoskeletal Oncology, National Cancer Center Hospital, Tokyo, Japan
| | - Jeffrey M Farma
- Division of Surgical Oncology, Fox Chase Cancer Center, Philadelphia, Pennsylvania, USA
| | - Anthony M Villano
- Division of Surgical Oncology, Fox Chase Cancer Center, Philadelphia, Pennsylvania, USA
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Sanabria A, Pinillos P, Chiesa-Estomba C, Guntinas-Lichius O, Kowalski LP, Mäkitie AA, Rao KN, Ferlito A. Comparing Mohs micrographic surgery and wide local excision in the management of head and neck dermatofibrosarcoma protuberans: a scoping review. J DERMATOL TREAT 2024; 35:2295816. [PMID: 38146660 DOI: 10.1080/09546634.2023.2295816] [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: 11/10/2023] [Accepted: 12/11/2023] [Indexed: 12/27/2023]
Abstract
Dermatofibrosarcoma protuberans (DFSP) is a rare, locally aggressive cutaneous sarcoma with a propensity for recurrence. Its management, particularly in the head and neck (H&N) region, presents unique challenges. This study aimed to evaluate the effectiveness of Mohs micrographic surgery (MMS) compared to wide local excision (WLE) in treating H&N DFSP and its impact on recurrence rates and tissue preservation. A comprehensive search was conducted in PubMed/MEDLINE, yielding 29 relevant studies. We included studies comparing MMS and WLE in adult patients with H&N DFSP and reporting local recurrence outcomes. Data were analyzed using random effects analysis, with a meta-analysis performed for comparative studies. Analysis of studies demonstrated a lower recurrence for MMS. Comparative analysis of five studies involving 117 patients showed a significantly lower recurrence rate in the MMS group (2%) compared to the WLE group (19%). Margin status varied between studies, with some achieving negative margins at shorter distances. In the management of H&N DFSP, MMS has emerged as a superior surgical technique, consistently associated with reduced recurrence rates and the potential for tissue preservation. The adoption of MMS should be considered for its capacity to achieve negative margins with fewer processing steps, particularly in anatomically complex regions like the H&N.
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Affiliation(s)
- Alvaro Sanabria
- Department of Surgery, School of Medicine, Universidad de Antioquia, Medellin, Colombia
- CEXCA Centro de Excelencia en Enfermedades de Cabeza y Cuello, Medellin, Colombia
| | - Pilar Pinillos
- Department of Surgery, School of Medicine, Universidad de Antioquia, Medellin, Colombia
| | - Carlos Chiesa-Estomba
- Department of Otorhinolaryngology-Head & Neck Surgery, Hospital Universitario Donostia-Biodonostia Research Institute, San Sebastian, Spain
| | | | - Luiz P Kowalski
- Head and Neck Surgery and Otorhinolaryngology Department, A C Camargo Cancer Center, Sao Paulo, Brazil
- Head and Neck Surgery Department, University of Sao Paulo Medical School, Sao Paulo, Brazil
| | - Antti A Mäkitie
- Department of Otorhinolaryngology-Head and Neck Surgery, Faculty of Medicine, Helsinki University Hospital, University of Helsinki, Helsinki, Finland
- Research Program in Systems Oncology, University of Helsinki, Helsinki, Finland
| | - Karthik N Rao
- Department of Head Neck Surgical Oncology, All India Institute of Medical Sciences, Raipur, India
| | - Alfio Ferlito
- Coordinator of the International Head and Neck Scientific Group, Padua, Italy
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Liu Y, Huang K, Chen M, Zhao S, He Z, Lu L, Wei T. High-frequency ultrasound-assisted Mohs micrographic surgery for the treatment of dermatofibrosarcoma protuberans. J Plast Reconstr Aesthet Surg 2024; 96:186-195. [PMID: 39094373 DOI: 10.1016/j.bjps.2024.07.013] [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: 10/24/2023] [Revised: 05/29/2024] [Accepted: 07/08/2024] [Indexed: 08/04/2024]
Abstract
BACKGROUND Dermatofibrosarcoma protuberans (DFSP) is a superficial sarcoma characterized by infiltrative growth with tentacle-like borders. Mohs micrographic surgery (MMS) is the preferred treatment option for DFSP. However, the imprecise boundary localization in MMS leads to an increased number of Mohs layers required and a longer surgery time. High-frequency ultrasound has excellent tissue recognition capability for DFSP, allowing for precise boundary marking. MATERIALS AND METHODS In this study, we retrospectively analyzed 14 cases of DFSP treated with MMS using preoperative ultrasound localization and three-dimensional reconstruction at Xiangya Hospital over the past 5 years. We also reviewed previous studies on MMS for DFSP treatment. RESULTS It was found that the average number of Mohs layers for patients after preoperative ultrasound localization was 1.57, ranging from 1 to 3, which was less than the previously reported 1.86 layers, ranging from 1 to 12. This effectively reduced the number of Mohs layers required. CONCLUSIONS By utilizing preoperative high-frequency ultrasound to determine the boundaries and depth of DFSP, the number of Mohs layers can be effectively reduced, leading to less workload for pathological examination, shorter operation time, and reduced surgical risks for patients. Ultrasound imaging data can be used for three-dimensional reconstruction, enabling less experienced Mohs surgeons to have a visual understanding of the morphology and extent of infiltration of the lesions. This aids in developing optimal surgical plans, smoothing the learning curve, and promoting the wider adoption of MMS.
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Affiliation(s)
- Yuancheng Liu
- Department of Dermatology, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China; National Engineering Research Center of Personalized Diagnostic and Therapeutic Technology, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China; Hunan Engineering Research Center of Skin Health and Disease, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China; Hunan Key Laboratory of Skin Cancer and Psoriasis, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China; National Clinical Research Center of Geriatric Disorders, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China
| | - Kai Huang
- Department of Dermatology, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China; National Engineering Research Center of Personalized Diagnostic and Therapeutic Technology, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China; Hunan Engineering Research Center of Skin Health and Disease, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China; Hunan Key Laboratory of Skin Cancer and Psoriasis, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China; National Clinical Research Center of Geriatric Disorders, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China
| | - Mingliang Chen
- Department of Dermatology, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China; National Engineering Research Center of Personalized Diagnostic and Therapeutic Technology, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China; Hunan Engineering Research Center of Skin Health and Disease, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China; Hunan Key Laboratory of Skin Cancer and Psoriasis, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China; National Clinical Research Center of Geriatric Disorders, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China
| | - Shuang Zhao
- Department of Dermatology, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China; National Engineering Research Center of Personalized Diagnostic and Therapeutic Technology, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China; Hunan Engineering Research Center of Skin Health and Disease, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China; Hunan Key Laboratory of Skin Cancer and Psoriasis, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China; National Clinical Research Center of Geriatric Disorders, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China
| | - Zhiyou He
- National Engineering Research Center of Personalized Diagnostic and Therapeutic Technology, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China; Hunan Engineering Research Center of Skin Health and Disease, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China; Hunan Key Laboratory of Skin Cancer and Psoriasis, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China; National Clinical Research Center of Geriatric Disorders, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China; Department of Burn, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China
| | - Lixia Lu
- Department of Dermatology, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China; National Engineering Research Center of Personalized Diagnostic and Therapeutic Technology, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China; Hunan Engineering Research Center of Skin Health and Disease, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China; Hunan Key Laboratory of Skin Cancer and Psoriasis, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China; National Clinical Research Center of Geriatric Disorders, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China.
| | - Tianhong Wei
- National Engineering Research Center of Personalized Diagnostic and Therapeutic Technology, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China; Hunan Engineering Research Center of Skin Health and Disease, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China; Hunan Key Laboratory of Skin Cancer and Psoriasis, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China; National Clinical Research Center of Geriatric Disorders, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China; Department of Ultrasound, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China
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Hanna M, Alkhatib ASA, Alassri R, Awada R, Daboura D, Martini N. A challenging diagnosis of dermatofibrosarcoma protuberans of the middle finger in an infant: A case report. Int J Surg Case Rep 2024; 120:109890. [PMID: 38865945 PMCID: PMC11258620 DOI: 10.1016/j.ijscr.2024.109890] [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: 03/24/2024] [Revised: 06/04/2024] [Accepted: 06/06/2024] [Indexed: 06/14/2024] Open
Abstract
INTRODUCTION Dermatofibrosarcoma protuberans (DFSP) is a rare sarcoma, accounting for less than 0.1 % of tumors. While it predominantly occurs in adults, pediatric cases are unusual. This case report aims to highlight the diagnostic and therapeutic challenges posed by DFSP in infants due to its rarity and slow-growing nature, emphasizing the importance of early diagnosis and prompt intervention. CASE PRESENTATION We report the case of an 8-month-old infant presenting with a progressive finger mass, initially mistakenly diagnosed as a dermatofibroma. Local excision was done, but the tumor recurred after one year. Subsequent re-excision and skin grafting were performed, and histopathology confirmed DFSP. Despite middle finger amputation three weeks later, a new mass emerged on the adjacent ring finger after one year. This tested negative for DFSP. The fibrous mass has persisted for five years without significant changes. CLINICAL DISCUSSION DFSP is a rare sarcoma with a higher prevalence in adults. It typically presents as a painless, slow-growing mass and is usually diagnosed by biopsy and immunohistochemistry. Surgical excision with negative margins is the preferred treatment. The rarity and slow-growing nature of DFSP pose challenges in diagnosis and treatment. CONCLUSION Early diagnosis and prompt surgical intervention are crucial in managing DFSP, especially given its high recurrence potential. Maintaining a high index of suspicion is essential even in very young children. Aggressive resection with negative margins and diligent post-operative surveillance are key strategies to mitigate metastasis risk and improve prognosis in such challenging cases.
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Affiliation(s)
- Majd Hanna
- Damascus university, Faculty of Medicine, Damascus, Syrian Arab Republic; Stemosis for Scientific Research, Damascus, Syrian Arab Republic.
| | | | - Riffa Alassri
- Stemosis for Scientific Research, Damascus, Syrian Arab Republic; Hama University, Faculty of Medicine, Hama, Syrian Arab Republic
| | - Rim Awada
- Stemosis for Scientific Research, Damascus, Syrian Arab Republic; Faculty of Medical Sciences, Lebanese University, Rafic Hariri University Campus, Hadath, Lebanon
| | - Dalaa Daboura
- Damascus university, Faculty of Medicine, Damascus, Syrian Arab Republic; Stemosis for Scientific Research, Damascus, Syrian Arab Republic
| | - Nafiza Martini
- Damascus university, Faculty of Medicine, Damascus, Syrian Arab Republic; Stemosis for Scientific Research, Damascus, Syrian Arab Republic
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Zeaiter N, Aoun CB, Sfeir J, Ghandour M, Hreibe W. Dermatofibrosarcoma Protuberans Arising From a Chronic Wound in the Left Shoulder: A Case Report. Cureus 2024; 16:e55638. [PMID: 38586739 PMCID: PMC10995738 DOI: 10.7759/cureus.55638] [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] [Accepted: 03/06/2024] [Indexed: 04/09/2024] Open
Abstract
Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing soft tissue sarcoma, typically presenting as a cutaneous lesion. However, its occurrence in chronic wounds is infrequently documented, posing diagnostic and therapeutic challenges. This report details the case of a 59-year-old female with no significant medical history, presenting with a chronic, non-healing wound on the left shoulder, persisting for three years. Initially a small nodule, it progressed into an ulcerating lesion. Physical examination revealed a contracted scar with restricted shoulder mobility. After obtaining informed consent, a surgical excision of the lesion was performed by an electrocautery. Histopathology confirmed DFSP, characterized by spindle fibrous cells, with skin ulceration and deep dermal infiltration. A split-thickness skin graft achieved successful closure. This case underscores the importance of considering DFSP in chronic, non-healing wounds. Timely intervention and appropriate surgical management are crucial for favorable outcomes.
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Affiliation(s)
- Nancy Zeaiter
- Plastic and Reconstructive Surgery, Lebanese University, Beirut, LBN
- Plastic and Reconstructive Surgery, Al-Zahraa Hospital University Medical Center, Beirut, LBN
| | - Charbel B Aoun
- Plastic and Reconstructive Surgery, Lebanese University, Beirut, LBN
- Plastic and Reconstructive Surgery, Al-Zahraa Hospital University Medical Center, Beirut, LBN
| | - Joseph Sfeir
- Plastic and Reconstructive Surgery, Lebanese University, Beirut, LBN
- Plastic and Reconstructive Surgery, Al-Zahraa Hospital University Medical Center, Beirut, LBN
| | | | - Walid Hreibe
- Plastic and Reconstructive Surgery, Lebanese University, Beirut, LBN
- Plastic and Reconstructive Surgery, Al-Zahraa Hospital University Medical Center, Beirut, LBN
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7
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Saifuddin H, Yan M, Jakub J, Martinez-Jorge J, Roenigk R, Vijayasekaran A. Wide local excision, Mohs micrographic surgery, and reconstructive options for treatment of dermatofibrosarcoma protuberans of the breast: A retrospective case series from Mayo Clinic. World J Surg Oncol 2023; 21:141. [PMID: 37147611 PMCID: PMC10163742 DOI: 10.1186/s12957-023-03022-9] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/11/2022] [Accepted: 09/23/2022] [Indexed: 05/07/2023] Open
Abstract
BACKGROUND Dermatofibrosarcoma protuberans (DFSP) of the breast is a dermal fibroblastic neoplasm requiring wide excisional margins due to recurrence rates ranging from 26 to 60%. The current literature on reconstructive options and utility of Mohs micrographic surgery for DFSP of the breast is scarce. We describe surgical management of DFSP of the breast at our institution with the largest case series reported to date. METHODS A retrospective review was performed of women who underwent surgery for DFSP of the breast at our institution between 1990 and 2019. Continuous data was summarized using mean, median, and range; categorical data was summarized with frequency count and percentage. Preoperative lesion size and postoperative defect size were evaluated using 2-sided Fisher exact test, and p-values < 0.05 were considered statistically significant. RESULTS Nine patients underwent wide local excision (WLE) with reconstruction including pedicled latissimus dorsi flaps (n = 2), local flap advancement (n = 2), mastectomy with implant (n = 1), oncoplastic breast reduction (n = 1), and skin grafts (n = 3). Nine underwent Mohs micrographic surgery (MMS) with complex primary closure. Mean postoperative maximum wound defect size for WLE was 10.8 cm versus 7.0 cm for MMS with no statistical significance (p = 0.77). Mean preoperative maximum lesion size for WLE was 6.4 cm versus 3.3 cm for MMS with no statistical significance (p = 0.07). Complications with WLE included wound dehiscence in three patients and seroma in one patient. No complications were reported with MMS and primary closure. Recurrence was reported in one WLE patient, which was successfully detected despite flap coverage and resected without complications. Median follow-up for the patients without recurrence was 5.0 years, with two patients in MMS cohort lost to follow-up. Five-year overall survival was 100%. CONCLUSIONS MMS and WLE are both viable surgical options for managing DFSP of the breast. MMS could potentially minimize reconstructive needs due to smaller average defect size and result in fewer complications but may also result in asymmetry. Immediate flap reconstruction, especially in larger defects, can achieve excellent aesthetic outcomes for patients with DFSP of the breast without compromising detection of disease recurrence.
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Affiliation(s)
- Hiba Saifuddin
- Division of Plastic Surgery, Mayo Clinic, Rochester, MN, USA
| | - Maria Yan
- Division of Plastic Surgery, Mayo Clinic, Rochester, MN, USA
| | - James Jakub
- Department of Surgery, Mayo Clinic, Jacksonville, FL, USA
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8
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Espinoza AF, Onwuka E, Hicks MJ, Masand P, Monson L, Okcu MF, Pinglay N, Vidimos A, Vasudevan SA. Dermatofibrosarcoma protuberans case report: Aggressive tumor in a preadolescent child. JOURNAL OF PEDIATRIC SURGERY CASE REPORTS 2023. [DOI: 10.1016/j.epsc.2023.102612] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 03/30/2023] Open
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9
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Pardo D, Scarola D, Weston GK, Falcone TE. Dermatofibrosarcoma Protuberans of the Face. EAR, NOSE & THROAT JOURNAL 2023:1455613231172321. [PMID: 37082813 DOI: 10.1177/01455613231172321] [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: 04/22/2023] Open
Abstract
Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive tumor with high rate of local recurrence but low metastatic potential. Its high-grade fibrosarcomatous variant and occurrence on the head and neck are rare findings associated with increased morbidity and mortality. The most significant prognostic feature of DFSP is obtaining tumor free surgical margins. As such, accurate recognition and proper management of this uncommon and locally aggressive malignancy is especially crucial in head and neck surgery.
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Affiliation(s)
- Daniel Pardo
- University of Connecticut School of Medicine, Farmington, CT, USA
| | - Danielle Scarola
- Divsion of Otolaryngology - Head & Neck Surgery, University of Connecticut Health Center, Farmington, CT, USA
| | - Gillian K Weston
- Department of Dermatology; Dermatopathology, University of Connecticut Health Center, Farmington, CT, USA
| | - Todd E Falcone
- Divsion of Otolaryngology - Head & Neck Surgery, University of Connecticut Health Center, Farmington, CT, USA
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10
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Mani S, Kumar R, Kakkar A, Barwad A, Dheeraj K, Sagar P, Kumar R. Recurrent Dermatofibrosarcoma Protuberans of the Head and Neck: a Case Series. Indian J Surg Oncol 2023; 14:128-136. [PMID: 36065236 PMCID: PMC9435430 DOI: 10.1007/s13193-022-01636-1] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/27/2022] [Accepted: 08/23/2022] [Indexed: 12/05/2022] Open
Abstract
Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous sarcoma that develops from dermal fibroblasts and spreads within the dermis and subcutaneous fat. It is locally aggressive, with a high local recurrence rate after excision but has extremely low metastatic potential. In the case of recurrent tumors, surgical excision with adequate margins is the gold standard treatment and may require adjuvant radiotherapy or chemotherapy in some cases. We conducted a retrospective analysis of individuals with dermatofibrosarcoma protuberance of the head and neck region that had treatment at our facility between 2016 and 2021. We gathered the data on the surgical techniques, reconstructive techniques used, histopathological features, adjuvant therapy, and outcomes. We treated three patients with head and neck dermatofibrosarcoma protuberance: one scalp lesion and two on the cheek. All three patients had recurrent tumors, two of whom were treated elsewhere for the primary lesion. One patient underwent surgery for a benign spindle cell tumor of the right cheek, but a final histopathological examination revealed dermatofibrosarcoma protuberance, and the tumor recurred within 3 months. The duration of recurrence is between 3 and 24 months. The size of the tumor ranges from 7.2 to 10.5 cm. The wide local excision margins range from 2 to 4 cm. Reconstruction ranges from split skin graft to regional flap. Inadequate margins raise the possibility of local recurrence in dermatofibrosarcoma protuberance.
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Affiliation(s)
- Suresh Mani
- Department of Head and Neck Surgery, CMC, Vellore, India
| | - Rajeev Kumar
- Department of ENT & Head and Neck Surgery, AIIMS, New Delhi, India
| | | | | | | | - Prem Sagar
- Department of ENT & Head and Neck Surgery, AIIMS, New Delhi, India
| | - Rakesh Kumar
- Department of ENT & Head and Neck Surgery, AIIMS, New Delhi, India
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Sheidaei S, Salehi M, Abedian kenari F, Jafari HR. Dermatofibrosarcoma protuberans challenges: a case series and review of the literature. J Med Case Rep 2023; 17:18. [PMID: 36653860 PMCID: PMC9850584 DOI: 10.1186/s13256-022-03728-6] [Citation(s) in RCA: 4] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/07/2022] [Accepted: 12/20/2022] [Indexed: 01/20/2023] Open
Abstract
BACKGROUND Dermatofibrosarcoma protuberans (DFSP) is a rare variant of skin sarcoma which is characterized by proliferation of spindle cells in a storiform pattern. Although it is mostly benign in its primary stages, it can cause a high burden of morbidity unless it is thoroughly excised. CASE PRESENTATION Here, we review six cases of DFSP which were characterized by skin lesions in various parts of the body. Patients were from 26 to 51 years old; four were Asian men and two were Asian women. Wide surgical excision was performed for all these patients and no extra treatment was considered. Samples were studied by hematoxylin and eosin (H&E) staining and immunohistochemical (IHC) tests. Only one of our patients experienced recurrence after the initial surgery. CONCLUSION Determining the best surgical method is still a dilemma in the treatment of DFSP lesions. There are numerous studies to prove the efficacy of various surgical interventions. Although DFSP is not commonly known as a malignant skin lesion, delay in treatment will have a catastrophic impact on patients' lives. Thus, applying an in-time surgical method (wide local excision in our cases) in treating DFSP is crucial in preventing recurrence as well as decreasing the morbidity burden of DFSP.
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Affiliation(s)
- Somayeh Sheidaei
- grid.411623.30000 0001 2227 0923Pathology, Mazandaran University of Medical Sciences, Mazandaran, Iran
| | - Mahsa Salehi
- grid.411623.30000 0001 2227 0923Pathology, Mazandaran University of Medical Sciences, Mazandaran, Iran
| | - Fatemeh Abedian kenari
- grid.411623.30000 0001 2227 0923Pathology, Mazandaran University of Medical Sciences, Mazandaran, Iran
| | - Hamid Reza Jafari
- grid.411036.10000 0001 1498 685XGeneral surgery, Isfahan University of Medical Sciences, Isfahan, Iran
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12
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Evidence from Clinical Studies Related to Dermatologic Surgeries for Skin Cancer. Cancers (Basel) 2022; 14:cancers14153835. [PMID: 35954498 PMCID: PMC9367341 DOI: 10.3390/cancers14153835] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/07/2022] [Revised: 07/22/2022] [Accepted: 08/03/2022] [Indexed: 01/20/2023] Open
Abstract
Simple Summary Although significant progress in pharmacotherapy for skin cancer has been made in the past several years, surgical removal of primary skin cancer is still the first choice of treatment unless distant metastases are evident. In the surgical treatment of primary skin tumors, the surgical margin is critical not only for reducing the possibility of tumor recurrence but also for minimizing the cosmetic and functional complications associated with wide local excision. In contrast, dermatologic surgeries including lymph node dissection and skin graft can cause various complications, and these complications are frequently associated with significant morbidity and discomfort. In this review, we summarize the evidence from previous clinical studies regarding the optimal surgical margin for skin cancer and the methods for diminishing the complications associated with dermatologic surgery. Abstract Despite the significant progress made in the past several years in pharmacotherapies for skin cancer, such as BRAF/MEK inhibitors, immune checkpoint inhibitors, and Hedgehog pathway inhibitors, surgical removal of primary skin cancer is still the first choice of treatment unless distant metastases are evident. In cases of lymph node metastases with clinically palpable lymphadenopathy, lymph node dissection (LND) is typically performed for most skin cancers. In the surgical treatment of primary skin tumors, the surgical margin is critical not only for reducing the possibility of tumor recurrence but also for minimizing the cosmetic and functional complications associated with wide local excision. In contrast, dermatologic surgery can cause various complications. Although skin graft is frequently used for reconstruction of the surgical defect, extensive graft necrosis may develop if optimal stabilization of the graft is not obtained. LND also sometimes causes complications such as intraoperative or postoperative bleeding and postoperative lymphoceles. Moreover, as in other types of surgery, surgical site infection, intraoperative anxiety, and intraoperative and postoperative pain may also develop. These complications are frequently associated with significant morbidity and discomfort. In this review, we summarize the evidence from previous clinical studies regarding the optimal surgical margin for skin cancer and the methods for diminishing the complications associated with dermatologic surgery.
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Abstract
Dermatofibrosarcoma protuberans (DFSP) is a rare, locally aggressive dermal-based sarcoma. Metastatic potential is extremely low, primarily in the setting of fibrosarcomatous transformation. DFSP is characterized by a t(17;22) (q22;q13) translocation that results in active PDGFB signaling. Surgical resection with negative margins (typically including the underlying fascia) is the potentially curative treatment. Delayed wound closure should be considered for cases requiring extensive resection or tissue rearrangement. Tyrosine kinase inhibitors, such as imatinib, have shown response rates of 50% to 60% in patients with locally advanced or metastatic disease. Radiation can be useful for residual or recurrent diseases.
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Muhammad R, Jamaluddin NN, Abdullah SSN, Wong YP. A Collision Tumor of Dermatofibrosarcoma Protuberance with Anaplastic Thyroid Carcinoma. WORLD JOURNAL OF ENDOCRINE SURGERY 2022; 14:31-33. [DOI: 10.5005/jp-journals-10002-1426] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 09/12/2023]
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Al Laham O, Atia F, Shaheen J, Alkhoury L, Ibrahim D, Alchikh Omar M. A unique case of Dermatofibrosarcoma Protuberans arising from an inguinal hernial repair scar in a Middle Eastern male - A Case Report. Int J Surg Case Rep 2022; 96:107334. [PMID: 35777334 PMCID: PMC9284050 DOI: 10.1016/j.ijscr.2022.107334] [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: 05/20/2022] [Revised: 06/15/2022] [Accepted: 06/18/2022] [Indexed: 02/07/2023] Open
Abstract
INTRODUCTION AND IMPORTANCE Dermatofibrosarcoma Protuberans (DFSP) is a rare and fatal variant of Spindle Cell Sarcoma. It has an annual incidence rate of 0.8 to 4.5 cases per one million individuals. It's locally aggressive and has vague and masquerading clinical presentations. Misdiagnosis is devastating as it can lead to time wasting, expenditure of unnecessary resources, and possibly raise morbidity and mortality for patients. It is warranted to raise preoperative clinical awareness to achieve prompt surgical therapeutic interventions to reach an up-to-par prognosis. CASE PRESENTATION We demonstrate the case of a 50-year-old previously healthy Middle Eastern male patient, who was referred to our General Surgery clinic with the chief complaint of an expansive bulge in his left iliac fossa. Preoperative imaging could not exclude a neoplastic cause behind the presentation. Based on the clinical picture, a surgical intervention was decided. CLINICAL DISCUSSION Our patient's treatment was consummated by means of classical surgical resection of the lesion with adequate negative margins and referring him to an oncologist specialized in DFSP to undergo the necessary adjuvant treatment. Definitive diagnosis was firmly entrenched postoperatively after finalization of the histopathological and immunohistochemical analyses of the resected protuberance. CONCLUSION DFSP is an eminently rare entity, especially DFSPs which originate from a surgical scar -as was our patient's- and fluctuates in its clinical presentation, thus, it is our responsibility to depict, study this malignant tumor, and document its incidence, so that we can make ironclad clinical decrees to plummet the morbidity and mortality of this relentless neoplasia.
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Affiliation(s)
- Omar Al Laham
- Department of Surgery, Al-Mouwasat University Hospital, Damascus, Syria,Department of Surgery, Al Assad University Hospital, Damascus, Syria,Corresponding author at: Department of Surgery, Al-Mouwasat University Hospital, Damascus, Syria.
| | - Fareed Atia
- Department of Surgery, Al-Mouwasat University Hospital, Damascus, Syria,Department of Surgery, Al Assad University Hospital, Damascus, Syria
| | - Jack Shaheen
- Department of Surgery, Al-Mouwasat University Hospital, Damascus, Syria,Department of Surgery, Al Assad University Hospital, Damascus, Syria
| | - Lama Alkhoury
- Department of Laboratory Diagnosis, Damascus University, Damascus, Syria
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Martin ECS, Vyas KS, Batbold S, Erwin PJ, Brewer JD. Dermatofibrosarcoma Protuberans Recurrence After Wide Local Excision Versus Mohs Micrographic Surgery: A Systematic Review and Meta-Analysis. Dermatol Surg 2022; 48:479-485. [PMID: 35353755 DOI: 10.1097/dss.0000000000003411] [Citation(s) in RCA: 13] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
BACKGROUND Local recurrence (LR) rates of dermatofibrosarcoma protuberans (DFSP) treated with different surgical modalities are unknown. OBJECTIVE To evaluate the differences in LR rates of DFSP treated with wide local excision (WLE) versus Mohs micrographic surgery (MMS). MATERIALS AND METHODS Pertinent studies of DFSP treated with either WLE or MMS were identified through a search of multiple databases, including Ovid MEDLINE (1946-2018), Embase (1988-2018), Web of Science (1975-2018), and Scopus (1970-2018). Comparative 2-arm and noncomparative single-arm studies were assessed through meta-analyses. RESULTS Of the 517 studies identified, 88 met inclusion criteria (12 comparative studies; 76 single-arm studies). In the 12 comparative studies, 352 patients with DFSP underwent MMS and 777 patients with DFSP underwent WLE. The LR rate was 1.7% after MMS and 3.7% after WLE (odds ratio, 1.549; 95% CI, 0.710-3.381; p = .27). In the 76 noncomparative studies, 980 patients underwent MMS (LR rate, 1.5%; 95% CI, 0.9%-2.1%; p < .001), and 2,215 patients underwent WLE (LR rate, 9.4%; 95% CI, 7.5%-11.3%; p < .001). CONCLUSION The LR rate of DFSP in patients treated with MMS is lower than in patients treated with WLE. Because of high rates of postoperative DFSP LR, MMS should be strongly considered when available.
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Affiliation(s)
- Emma C St Martin
- Mayo Clinic Alix School of Medicine, Minnesota campus, Rochester, Minnesota
| | - Krishna S Vyas
- Mayo Clinic School of Graduate Medical Education, Mayo Clinic College of Medicine and Science, Rochester, Minnesota
| | - Sarah Batbold
- Mayo Clinic Alix School of Medicine, Minnesota campus, Rochester, Minnesota
| | | | - Jerry D Brewer
- Department of Dermatology, Division of Dermatologic Surgery, Mayo Clinic, Rochester, Minnesota
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Score for the Overall Survival Probability Scores of Fibrosarcoma Patients after Surgery: A Novel Nomogram-Based Risk Assessment System. JOURNAL OF ONCOLOGY 2022; 2021:4533175. [PMID: 34976057 PMCID: PMC8716234 DOI: 10.1155/2021/4533175] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 08/10/2021] [Accepted: 12/01/2021] [Indexed: 12/29/2022]
Abstract
Background The primary purpose of this study was to determine the risk factors affecting overall survival (OS) in patients with fibrosarcoma after surgery and to develop a prognostic nomogram in these patients. Methods Data were collected from the Surveillance, Epidemiology, and End Results database on 439 postoperative patients with fibrosarcoma who underwent surgical resection from 2004 to 2015. Independent risk factors were identified by performing Cox regression analysis on the training set, and based on this, a prognostic nomogram was created. The accuracy of the prognostic model in terms of survival was demonstrated by the area under the curve (AUC) of the receiver operating characteristic curves. In addition, the prediction consistency and clinical value of the nomogram were validated by calibration curves and decision curve analysis. Results All included patients were divided into a training set (n = 308) and a validation set (n = 131). Based on univariate and multivariate analyses, we determined that age, race, grade, and historic stage were independent risk factors for overall survival after surgery in patients with fibrosarcoma. The AUC of the receiver operating characteristic curves demonstrated the high predictive accuracy of the prognostic nomogram, while the decision curve analysis revealed the high clinical application of the model. The calibration curves showed good agreement between predicted and observed survival rates. Conclusion We developed a new nomogram to estimate 1-year, 3-year, and 5-year OS based on the independent risk factors. The model has good discriminatory performance and calibration ability for predicting the prognosis of patients with fibrosarcoma after surgery.
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Pediatric Pigmented Fibrosarcomatous Dermatofibrosarcoma Protuberans: Answer. Am J Dermatopathol 2022; 44:75-76. [PMID: 34889817 DOI: 10.1097/dad.0000000000002101] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
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Mohammed SY, Sadiq Q, Mcgregor D, Khan F. Diagnostic Challenges of Dermatofibrosarcoma Protuberans (DFSP), a Rare Spindle Cell Tumor of Breast. Cureus 2021; 13:e20643. [PMID: 35106202 PMCID: PMC8788893 DOI: 10.7759/cureus.20643] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 12/23/2021] [Indexed: 11/05/2022] Open
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Nam S, Park JB, Kim HC. Dermatofibrosarcoma Protuberans in Active Duty Military Personnel: A Case Report. Mil Med 2021; 188:usab423. [PMID: 34626190 DOI: 10.1093/milmed/usab423] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/04/2021] [Revised: 09/27/2021] [Accepted: 10/01/2021] [Indexed: 11/14/2022] Open
Abstract
Dermatofibrosarcoma protuberans (DFSP) is an uncommon, soft tissue sarcoma with a high rate of local recurrence, low rate of distant metastasis, and infiltrative growth. We report two cases of DFSP in young active duty military personnel. Both cases underwent primary excision at the Armed Forces Capital Hospital with positive surgical margins and underwent re-excision at Samsung Medical Center. Dermatofibrosarcoma protuberans is a rare finding, and wide local excision with or without skin flap remains the procedure of choice. Awareness of DFSP in young patients is important for proper diagnosis and treatment and to achieve local control. Complete excision of the tumor with a clear surgical margin and thorough surveillance after surgery are recommended for service members to maintain their active duty status and to prevent ineffective combat conditions.
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Affiliation(s)
- Soyun Nam
- Department of Surgery, Armed Forces Capital Hospital, Medical Corps, Republic of Korea Army, Seongnam 13754, Republic of Korea
| | - Jae Berm Park
- Department of Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul 06351, Republic of Korea
| | - Hyun-Chul Kim
- Department of Surgery, Armed Forces Capital Hospital, Medical Corps, Republic of Korea Army, Seongnam 13754, Republic of Korea
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21
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Dermatofibrosarcoma- An uncommon entity, commonly mismanaged: a case report. Int J Surg Case Rep 2021; 87:106385. [PMID: 34563815 PMCID: PMC8479619 DOI: 10.1016/j.ijscr.2021.106385] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/16/2021] [Revised: 08/10/2021] [Accepted: 09/02/2021] [Indexed: 12/03/2022] Open
Abstract
Introduction & importance Dermatofibrosarcoma protuberans (DFS) is a slow-growing, recurrent, cutaneous soft tissue sarcoma with low metastatic potential. It is not uncommon for DFS to get misdiagnosed and treated like commoner parietal wall swellings, viz., epidermal cysts, lipomas and fibromas. Suboptimal management often leads to recurrence, which may be difficult to manage. Case report A 33-year male was referred to us with a lump in his lower abdomen for 15 years, without any symptoms. The patient underwent medical management for 6 years, followed by excision. The swelling recurred four years later and progressed in size till the next 5 years. At our centre, the patient underwent imaging and aspiration cytology, which established diagnosis of DFS, followed by wide local excision. Biopsy was conclusive and margins negative. Discussion DFS presents commonly as an asymptomatic indurated plaque that slowly enlarges over months to years. Untreated, DFS can attain massive dimensions, producing the large “protuberant” nodules, and hence the name. In the current report, 15 years elapsed before the correct diagnosis was established. In this case, recurrence first appeared after four years of excision. The possibility of primary wound closure after wide excision decreases with every subsequent excision, and reconstructive options may not be readily available. Conclusion The current report highlights a diagnostic delay of 15 years in a case of DFS. Asymptomatic, indolent nature combined with low awareness among community doctors contribute to delay in timely diagnosis. Community doctors should consider DFS as a differential in any long-standing, indolent, asymptomatic parietal wall swelling, especially with a history of recurrence.
It is not uncommon for DFS to get misdiagnosed . Suboptimal management often leads to recurrence, which may be difficult to manage. We report a case that highlights a diagnostic delay of 15 years in the case of DFS. Indolent nature low awareness among doctors contribute to delay in the diagnosis.
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22
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Zhou AE, Hoegler KM, Khachemoune A. Review of Perineural Invasion in Keratinocyte Carcinomas. Am J Clin Dermatol 2021; 22:653-666. [PMID: 34105084 DOI: 10.1007/s40257-021-00615-6] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 05/28/2021] [Indexed: 12/19/2022]
Abstract
Perineural invasion is an infiltrative process of peripheral nerves by the primary neoplasm within the immediate vicinity. Aggressive forms of keratinocyte carcinomas, such as basal cell and squamous cell carcinomas, may feature perineural invasion, which is often associated with tumor recurrence and poorer prognosis. Diagnosis requires a high clinical suspicion. Imaging and histopathology are used to assess for extent of disease while surgical excision with complete circumferential peripheral and margin assessment is the treatment goal. However, there is still significant uncertainty about adjuvant chemotherapy and definitive management guidelines. Here, we summarize the current understanding of this complex pathogenic process, the clinical presentation, and the significance of perineural inflammation. We also discuss the recommendations about staging, prognosis, adjuvant radiotherapy, and general guidelines for managing keratinocyte carcinomas with perineural invasion. A better understanding of perineural invasion is essential to improve diagnosis, tailor interventions, and mitigate patient morbidity and mortality.
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Badhey AK, Tikhtman R, Tang AL. Management of dermatofibrosarcoma protuberans. Curr Opin Otolaryngol Head Neck Surg 2021; 29:278-282. [PMID: 33993132 DOI: 10.1097/moo.0000000000000721] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
PURPOSE OF REVIEW Provide an up to date review of the diagnosis, workup and treatment of dermatofibrosarcoma protuberans (DFSP). DFSP can be a challenging disease to manage and adequate understanding of the most up to date literature can help provide comprehensive treatment strategies. RECENT FINDINGS DFSP is an infiltrative cutaneous sarcoma. It tends to have deep local invasion with a high risk of local recurrence, but a low risk of distant metastasis. It presents typically as a slow growing, asymptomatic skin lesion. It presents rarely in the head and neck, only 15% of the time. Recent data has discussed the role of wide local excision (WLE) vs. Mohs surgery. In addition, for unresectable disease the role of systemic therapy and immunomodulatory agents such as Imatinib has shown success. SUMMARY Typically, surgical management is the first line for DFSP, however the risk for local recurrence still remains high with negative margins. Due to this risk, lifelong surveillance is required after initial diagnosis and management. Similar to other head and neck tumors, most recurrences happen within the first 3 years after treatment. DFSP can be treated with WLE or Mohs. For aggressive disease that is considered unresectable systemic therapy does exist, including molecular targeted therapies.
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Affiliation(s)
- Arvind K Badhey
- Department of Otolaryngology, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA
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Xiong JX, Cai T, Hu L, Chen XL, Huang K, Chen AJ, Wang P. Risk factors related to postoperative recurrence of dermatofibrosarcoma protuberans: A retrospective study and literature review. World J Clin Cases 2021; 9:5442-5452. [PMID: 34307598 PMCID: PMC8281415 DOI: 10.12998/wjcc.v9.i20.5442] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/19/2021] [Revised: 04/20/2021] [Accepted: 05/19/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Dermatofibrosarcoma protuberans (DFSP) is a rare low-grade malignant soft tissue tumor characterized by rosette-like infiltrative growth. Postoperative recurrence of this tumor is very common.
AIM To evaluate the risk factors related to recurrence after wide local excision (WLE) of DFSP and to guide clinical diagnosis and treatment.
METHODS The medical records of 44 DFSP patients confirmed by pathology at our hospital from 2012 to 2019 were retrospectively reviewed. The relationship between clinical features, tumor characteristics, treatment, and recurrence risk were analyzed, and the possible risk factors for postoperative tumor recurrence were evaluated.
RESULTS There were 44 patients in total, including 21 males and 23 females. The median progression free survival was 36 mo (range, 1-240 mo). Twenty patients were treated for the first time, while 24 had previous treatment experience. Forty-two cases were followed for 25.76 ± 22.0 mo, among whom four (9.52%) experienced recurrence after WLE (rate was 9.52%). The recurrence rate in the recurrent group was higher than that in the patients with primary tumor (19.05% vs 0%, P = 0.028). Eighteen cases had a history of misdiagnosis (rate was 40.91%). The recurrence rate among patients with previous experience of misdiagnosis was significantly higher than in patients without (68% vs 36.84%, P = 0.04). The tumor diameter in patients with a history of treatment was larger than in patients treated for the first time (4.75 ± 0.70 cm vs 2.25 ± 0.36 cm, P = 0.004).
CONCLUSION To sum up, the clinical manifestations of DFSP are not specific and are easily misdiagnosed, thus commonly causing the recurrence of DFSP. After incomplete resection, the tumor may rapidly grow. Previous recurrence history may be a risk factor for postoperative recurrence, and tumor location may have an indirect effect on postoperative recurrence; however, we found no significant correlation between sex, age, course of the disease, or tumor size and postoperative recurrence.
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Affiliation(s)
- Jian-Xia Xiong
- Department of Dermatology, The First Affiliated Hospital of Chongqing Medical University, Chongqing 400010, China
| | - Tao Cai
- Department of Dermatology, The First Affiliated Hospital of Chongqing Medical University, Chongqing 400010, China
| | - Li Hu
- Department of Dermatology, The First Affiliated Hospital of Chongqing Medical University, Chongqing 400010, China
| | - Xiao-Li Chen
- Department of Dermatology, The First Affiliated Hospital of Chongqing Medical University, Chongqing 400010, China
| | - Kun Huang
- Department of Dermatology, The First Affiliated Hospital of Chongqing Medical University, Chongqing 400010, China
| | - Ai-Jun Chen
- Department of Dermatology, The First Affiliated Hospital of Chongqing Medical University, Chongqing 400010, China
| | - Ping Wang
- Department of Dermatology, The First Affiliated Hospital of Chongqing Medical University, Chongqing 400010, China
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Chen Y, Jiang G. Association between surgical excision margins and outcomes in patients with dermatofibrosarcoma protuberans: A meta-analysis. Dermatol Ther 2021; 34:e14954. [PMID: 33835635 DOI: 10.1111/dth.14954] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/05/2020] [Revised: 03/09/2021] [Accepted: 04/06/2021] [Indexed: 11/29/2022]
Abstract
Dermatofibrosarcoma protuberans (DFSP) is a tumor with low-grade malignancy and high recurrence rates. Wide local excision (WLE) is essential for DFSP, but its surgical excision margin is controversial. This study aimed to examine whether resection margin is associated with the prognosis of patients with DFSP by systematically reviewing related literature. Studies that evaluated the relationship between excision margins and outcomes after WLE were retrieved from the Cochrane Library, PubMed, Embase, and Web of Science databases. Relative risk (RR) estimates and 95% confidence intervals (CIs) were measured via a random-effect model to evaluate the subsequent outcomes: recurrence rate, mortality, and positive surgical margin rate. Sensitivity and subgroup analyses were simultaneously carried out. The meta-analysis of eight observational literatures demonstrated a significant positive correlation of ≥3 cm margin with the recurrence rate (RR = 0.17, 95% CI 0.09-0.31) and the positive surgical margin rate (RR = 0.09, 95% CI 0.02-0.46). The same result was observed in the recurrence rate in all subgroups. The stability of the above outcomes was authenticated via sensitivity analyses. Our meta-analysis illustrated that excision with <3 cm surgical margin exhibited an increased risk of poor DFSP prognosis (high recurrence rate and increasing positive margin rate).
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Affiliation(s)
- Yiyin Chen
- Department of Dermatology, Affiliated Hospital of Xuzhou Medical University, Xuzhou, China.,Department of Dermatology, Xuzhou Medical University, Xuzhou, China
| | - Guan Jiang
- Department of Dermatology, Affiliated Hospital of Xuzhou Medical University, Xuzhou, China.,Department of Dermatology, Xuzhou Medical University, Xuzhou, China
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Li Y, Liang J, Xu X, Jiang X, Wang C, Chen S, Xiang B, Ji Y. Clinicopathological features of fibrosarcomatous dermatofibrosarcoma protuberans and the construction of a back-propagation neural network recognition model. Orphanet J Rare Dis 2021; 16:48. [PMID: 33499900 PMCID: PMC7836157 DOI: 10.1186/s13023-021-01698-4] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/07/2020] [Accepted: 01/19/2021] [Indexed: 02/08/2023] Open
Abstract
BACKGROUND Fibrosarcomatous dermatofibrosarcoma protuberans (FS-DFSP) is a form of tumor progression of dermatofibrosarcoma protuberans (DFSP) with an increased risk of metastasis and recurrence. Few studies have compared the clinicopathological features of FS-DFSP and conventional DFSP (C-DFSP). OBJECTIVES To better understand the epidemiological and clinicopathological characteristics of FS-DFSP. METHODS We conducted a cohort study of 221 patients diagnosed with DFSP and built a recognition model with a back-propagation (BP) neural network for FS-DFSP. RESULTS Twenty-six patients with FS-DFSP and 195 patients with C-DFSP were included. There were no differences between FS-DFSP and C-DFSP regarding age at presentation, age at diagnosis, sex, size at diagnosis, size at presentation, and tumor growth. The negative ratio of CD34 in FS-DFSP (11.5%) was significantly lower than that in C-DFSP (5.1%) (P = 0.005). The average Ki-67 index of FS-DFSP (18.1%) cases was significantly higher than that of C-DFSP (8.1%) cases (P < 0.001). The classification accuracy of the BP neural network model training samples was 100%. The correct rates of classification and misdiagnosis were 84.1% and 15.9%. CONCLUSIONS The clinical manifestations of FS-DFSP and C-DFSP are similar but have large differences in immunohistochemistry. The classification accuracy and feasibility of the BP neural network model are high in FS-DFSP.
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Affiliation(s)
- Yanan Li
- Division of Oncology, Department of Pediatric Surgery, West China Hospital of Sichuan University, #37 Guo-Xue-Xiang, Chengdu, 610041 China
| | - Jiaqi Liang
- Shaanxi Zhongtian Rocket Technology Co, Ltd, Xi’an, 710025 China
| | - Xuewen Xu
- Department of Burns and Plastic Surgery, West China Hospital of Sichuan University, Chengdu, 610041 China
| | - Xian Jiang
- Department of Dermatology, West China Hospital of Sichuan University, Chengdu, 610041 China
| | - Chuan Wang
- Division of Oncology, Department of Pediatric Surgery, West China Hospital of Sichuan University, #37 Guo-Xue-Xiang, Chengdu, 610041 China
| | - Siyuan Chen
- Pediatric Intensive Care Unit, Department of Critical Care Medicine, West China Hospital of Sichuan University, Chengdu, 610041 China
| | - Bo Xiang
- Division of Oncology, Department of Pediatric Surgery, West China Hospital of Sichuan University, #37 Guo-Xue-Xiang, Chengdu, 610041 China
| | - Yi Ji
- Division of Oncology, Department of Pediatric Surgery, West China Hospital of Sichuan University, #37 Guo-Xue-Xiang, Chengdu, 610041 China
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A Rare Malignant Disease, Dermatofibrosarcoma Protuberans of the Breast: A Retrospective Analysis and Review of Literature. BIOMED RESEARCH INTERNATIONAL 2020; 2020:8852182. [PMID: 33224981 PMCID: PMC7671798 DOI: 10.1155/2020/8852182] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 08/26/2020] [Revised: 10/19/2020] [Accepted: 10/25/2020] [Indexed: 11/17/2022]
Abstract
Dermatofibrosarcoma protuberans (DFSP) is a rare low-grade fibroblastic mesenchymal tumor derived from the dermis. The aim of this retrospective analysis was to summarize the clinicopathological data from our cases and published cases to offer more evidence for the recognition of dermatofibrosarcoma protuberans (DFSP). A total of 6 breast DFSP patients who had received treatment in our hospital were retrospectively enrolled, and detailed clinicopathological data were gathered for analysis. The median age was 29.5 years (ranging from 17 to 42 years). Most cases presented a red or brown-red, mobile, well-circumscribed, protruding, breast mass (ranging from 1 to 3 cm). For histopathology, all cases (6/6) showed a storiform pattern of spindle cells that were positive for CD34 (6/6) and Vimentin (5/6) and negative for smooth muscle actin (0/6) and S-100 protein (0/6). The majority of patients (5/6) underwent wide local excision, with 2 cases treated with radiotherapy. With a median follow-up of 36 months, all 6 patients survived without recurrence or metastasis. The PubMed database was used to search for similar cases. Eventually, 36 cases were included in this review, while cases without detailed clinical information or not reported in English were excluded from the analysis. To summarize, DFSP of the breast is an extremely rare malignancy characterized by spindle tumor cells arranged in a storiform pattern and positivity for CD34. The core needle biopsy is one of the crucial methods for its preoperative diagnosis. Management of DFSP is mainly based on surgical excision. It is prone to local recurrence, so long-term follow-up is required.
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González A, Etchichury D, Rivero JM, Adamo L. Treatment of dermatofibrosarcoma of the head and neck with Mohs surgery with paraffin sections. J Plast Reconstr Aesthet Surg 2020; 74:1061-1070. [PMID: 33317985 DOI: 10.1016/j.bjps.2020.10.062] [Citation(s) in RCA: 13] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/01/2020] [Revised: 06/20/2020] [Accepted: 10/20/2020] [Indexed: 11/28/2022]
Abstract
INTRODUCTION Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade mesenchymal skin tumor. Complete surgical excision remains the mainstay of treatment. Head and neck (HN) DFSP features high local recurrence (LR) rates with wide local excision (WLE) due to both anatomical limitations and functional and cosmetic concerns. Mohs micrographic surgery (MMS) allows complete margin control and is particularly suitable for HN-DFSPs. PATIENTS AND METHODS Between 2000 and 2018, 103 DFSP were managed with MMS; 41 (39.8%) were in the HN. Our case series included 23 females (56.1%); mean age: 44.5 yrs. The most frequent sites were the supraclavicular area (10) and forehead (9). Mean size: 5.1 cm (6-16). Previous surgery: 43.9%. We have used permanent-tissue MMS (27 cases) since 2006. RESULTS Mean first layer lateral margin: 1.6 cm. Negative margins after first and second stage: 51.2% and 92.7%, respectively. Mean follow-up: 92.6 months (16-225). LR: 1(2.4%). We observed no regional or distant metastasis. DISCUSSION We conducted a literature search in PubMed for HN-DFSP and found that this seems to be the largest published series to date. In terms of LR, a substantial difference between WLE 62.5% (9-73%) and MMS 0.8% (0-2.4%) was seen. Forty-one HN-DFSP with unfavorable prognostic factors (mean size: 5.1 cm, previous treatment: 43.9%) were treated. LR: 1(2.4%). Mean follow-up: 92.6 months. Randomized controlled trials are needed, though not likely to be conducted, mainly because of the rarity of HN-DFSP, the need for a long follow-up, and the lack of MMS availability in some institutions. We consider MMS to be the standard treatment for HN-DFSP due to its high cure rate and tissue sparing. Modified WLE with complete margin control appears to be a comparable strategy.
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Affiliation(s)
- Abel González
- Instituto Alexander Fleming, Buenos Aires, Argentina; Instituto Oncología Angel H. Roffo, Buenos Aires, Argentina.
| | - Dardo Etchichury
- Instituto Alexander Fleming, Buenos Aires, Argentina; Instituto Oncología Angel H. Roffo, Buenos Aires, Argentina
| | - Juan Mario Rivero
- Instituto Alexander Fleming, Buenos Aires, Argentina; Instituto Oncología Angel H. Roffo, Buenos Aires, Argentina
| | - Lucia Adamo
- Instituto Alexander Fleming, Buenos Aires, Argentina; Instituto Oncología Angel H. Roffo, Buenos Aires, Argentina
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Al-Farsi A, Al-Brashdi A, Al-Salhi S, Al-Rahbi N, Al-Rahbi S, Al-Masqari M, Al-Manji Z, Al-Umairi RS. Dermatofibrosarcoma Protuberans Mimicking Primary Breast Neoplasm: A case report and literature review. Sultan Qaboos Univ Med J 2020; 20:e368-e371. [PMID: 33110655 PMCID: PMC7574798 DOI: 10.18295/squmj.2020.20.03.019] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/04/2020] [Revised: 03/30/2020] [Accepted: 04/30/2020] [Indexed: 11/16/2022] Open
Abstract
Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing mesenchymal neoplasm of the dermis and subcutaneous tissues that has a low-to intermediate-grade malignancy. DFSP commonly involves the trunk and extremities, and very rarely the breast skin, mimicking a primary breast neoplasm with few reported cases in the literature. We report a 35-year old female patient who was referred to the Royal Hospital, Muscat, Oman in 2017, with a two-year history of a slow growing left breast lump. Assessment of the breasts with mammography revealed a lobulated lesion in the left-upper-inner quadrant with neither microcalcification nor architectural distortion, mimicking a benign lesion. However, on ultrasound, the lesion had suspicious features with increased vascularity and hence, it was categorised as breast imaging reporting and data system (BIRAD) IV. The patient underwent left breast wide local excision and the histopathological diagnosis was dermatofibrosarcoma protuberans.
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Affiliation(s)
- Abeer Al-Farsi
- Department of Radiology, Oman Medical Specialty Board, Muscat, Oman
| | - Aza Al-Brashdi
- Department of Radiology, Oman Medical Specialty Board, Muscat, Oman
| | | | - Nasser Al-Rahbi
- Department of Histopathology, the Royal Hospital, Muscat, Oman
| | | | | | - Zainab Al-Manji
- Department of Histopathology, the Royal Hospital, Muscat, Oman
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Zargham H, Khachemoune A. Systematic review of Mohs micrographic surgery in children: Identifying challenges and practical considerations for successful application. J Am Acad Dermatol 2020; 85:152-161. [PMID: 33011324 DOI: 10.1016/j.jaad.2020.09.052] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/15/2020] [Revised: 09/20/2020] [Accepted: 09/21/2020] [Indexed: 10/23/2022]
Abstract
BACKGROUND Few data exist to guide the application of Mohs micrographic surgery (MMS) in the pediatric population. OBJECTIVE We sought to summarize the clinical characteristics of children undergoing MMS, identify challenges that limit the use of MMS in this population, and examine how these challenges can be overcome. METHODS A systematic review of PubMed and EMBASE, from inception of databases to November 2, 2019, identified all cases of pediatric skin lesions treated with MMS. RESULTS A total of 111 patients were included. The median patient age was 11 years (range 6 weeks to 17 years). The most commonly treated tumor was dermatofibrosarcoma protuberans (n = 62), followed by basal cell carcinoma (n = 30). The most common location was the head and neck (n = 34), followed by the trunk (n = 28) and the extremities (n = 23). The most commonly cited challenges in the application of MMS in children included patient cooperation, concerns for the safety of prolonged general anesthesia, availability of a MMS service in the pediatric setting, and access to a histopathology laboratory experienced in MMS sectioning. LIMITATIONS Many articles did not report specific patient characteristics. CONCLUSION Multiple obstacles limit the application of MMS in pediatric patients. This review describes practical methods to circumvent these obstacles to facilitate the appropriate use of MMS in children.
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Affiliation(s)
- Hanieh Zargham
- Division of Dermatology, McGill University Health Center, Montreal, Quebec, Canada; Department of Dermatology, University of New York Downstate and Veterans Affairs Medical Center, Brooklyn, New York
| | - Amor Khachemoune
- Division of Dermatology, McGill University Health Center, Montreal, Quebec, Canada; Department of Dermatology, University of New York Downstate and Veterans Affairs Medical Center, Brooklyn, New York.
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31
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Durack A, Gran S, Gardiner MD, Jain A, Craythorne E, Proby CM, Marsden J, Harwood CA, Matin RN. A 10-year review of surgical management of dermatofibrosarcoma protuberans. Br J Dermatol 2020; 184:731-739. [PMID: 32599647 DOI: 10.1111/bjd.19346] [Citation(s) in RCA: 18] [Impact Index Per Article: 3.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 06/23/2020] [Indexed: 11/29/2022]
Abstract
BACKGROUND Dermatofibrosarcoma protuberans (DFSP) is a rare skin cancer. Standard treatment in the UK is either wide local excision (WLE) or Mohs micrographic surgery (MMS). It is unclear which approach has the lower recurrence rate. OBJECTIVES We undertook a retrospective comparative review of surgical management of DFSP in the UK National Health Service in order to define (i) current surgical practice for primary and recurrent DFSP, (ii) local recurrence rates for primary DFSP and (iii) survival outcomes for DFSP. METHODS A retrospective clinical case-note review of patients with histologically confirmed DFSP (January 2004 to December 2013) who have undergone surgical treatment. RESULTS The surgical management of 483 primary and 64 recurrent DFSP in 11 plastic surgery and 15 dermatology departments was analysed. Almost 75% of primary DFSP (n = 362) were treated with WLE and 20% (n = 97) with MMS. For recurrent DFSP, 69% (n = 44) and 23% (n = 15) of patients underwent WLE and MMS, respectively. Recurrent primary DFSP occurred in six patients after WLE and none after MMS. The median follow-up time was 25·5 months (interquartile range 6·8-45·1) for new and 19·8 (IQR 4·5-44·5) for recurrent DFSP [Correction added on 1 Feb 2021, after first online publication: 4.8 years (interquartile range 3.5-5.8) was incorrect], with eight reported deaths during the follow-up analysis period (one confirmed to be DFSP related). CONCLUSIONS WLE was the most common surgical modality used to treat DFSP across the UK. The local recurrence rate was very low, occurring only after WLE. Although a prospective randomized controlled trial may provide more definitive outcomes, in the absence of a clearly superior surgical modality, treatment decisions should be based on patient preference, clinical expertise and cost.
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Affiliation(s)
- A Durack
- Department of Dermatology, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK
| | - S Gran
- Centre of Evidence Based Dermatology, University of Nottingham, Nottingham, UK
| | - M D Gardiner
- Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Sciences, University of Oxford, Oxford, UK.,Department of Plastic and Reconstructive Surgery, Frimley Health Foundation NHS Trust, Frimley, UK
| | - A Jain
- Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Sciences, University of Oxford, Oxford, UK.,Department of Plastic and Reconstructive Surgery, Imperial College NHS Trust, London, UK
| | - E Craythorne
- Department of Dermatology, Guy's and St Thomas' NHS Foundation Trust, London, UK
| | - C M Proby
- Department of Dermatology, Ninewells Hospital and Medical School, University of Dundee, Dundee, UK
| | - J Marsden
- Department of Dermatology, Queen Elizabeth Hospital Birmingham, Birmingham, UK
| | - C A Harwood
- Centre for Cell Biology and Cutaneous Research, Barts Health NHS Trust, Queen Mary University of London, London, UK
| | - R N Matin
- Department of Dermatology, Oxford University Hospitals NHS Foundation Trust, Oxford, UK
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Nangole WF, Muhinga M. Multidisciplinary Approach to the Management of Extensive Dermato-fibro-sarcomas Involving the Chest Wall. Indian J Plast Surg 2020; 54:82-85. [PMID: 33814747 PMCID: PMC8012793 DOI: 10.1055/s-0040-1714975] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/01/2022] Open
Abstract
Dermato-fibro-sarcomas are known for high-recurrence rates. The gold standard of management is surgical excision with clear margins. Such margins on the chest results in large defects which require complex reconstructive procedures. We report a case series of patients managed by a multidisciplinary team with good outcomes. A total of 12 patients with extensive dermato-fibro-sarcoma of the anterior chest wall were treated over a period of 5 years in our setting. The age range was 25 to 54 years. Skeletal defects were reconstructed with Prolene mesh and methyl acrylate cement in 10 of the 12 patients. Pedicle flaps were used in nine patients. All margins were clear of tumors, with the nearest margin being 1.5 cm. One patient had a recurrence. No donor-site morbidity was recorded in any of the patients. In conclusion, a multidisciplinary approach provides improved outcomes in the management of large dermato-fibro-sarcomas of the chest wall. With this approach, extensive dissection of the tumor is achieved, and reconstruction is performed with minimal complication.
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Affiliation(s)
| | - M Muhinga
- Department of Surgery, University of Nairobi, Nairobi, Kenya
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33
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Akhtar A, Khan AH, Rashid M, Eitezaz F, Ur Rashid H. Resection and Reconstructive Options in the Management of Dermatofibrosarcoma Protuberans of the Head and Neck. Cureus 2020; 12:e9423. [PMID: 32864249 PMCID: PMC7450883 DOI: 10.7759/cureus.9423] [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: 11/05/2022] Open
Abstract
Objective To discuss resection and various reconstructive options in patients with dermatofibrosarcoma protuberans (DFSP). Methods This study was conducted at Shifa International Hospital, Islamabad, Pakistan, from May 2018 to December 2019. All patients aged 20 years or above of either gender who were diagnosed to have DFSP over this period were included in the study. All the patients underwent wide local excision of the tumor under general anesthesia. A peroperative frozen section was conducted in all the cases to confirm complete excision. Immediate reconstruction was performed following the tumor excision. The choice of reconstruction, i.e. free, regional, or local flap was based on the size of the resultant defect. Results The mean age of the patients was 37.11 ±10.91 years. There were 12 (66.7%) males and six (33.3%) females. The mean duration of the disease was 11.22 ±2.94 months. The affected anatomical site showed that the face was involved in the majority, nine (50%) patients, followed by the scalp in four (22.2%), nape of the neck in three (16.7%), and supraclavicular region in two (11.1%) patients. In most of the cases, the free flap was observed, i.e. (n=9, 50%), followed by a regional flap in seven (38.9%), and the local flap in two (10.1%) patients. Conclusion Wide local excision of the disease, confirmed on frozen section, offers improved survival. Among DFSP of the head and neck, the face was found to be the affected anatomical site in half the cases. Also, reconstruction following tumor excision with a free flap is the most favorable option among patients with DFSP.
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Affiliation(s)
- Aqsa Akhtar
- Plastic Surgery, Shifa International Hospital, Islamabad, PAK
| | | | - Mamoon Rashid
- Plastic Surgery, Shifa International Hospital, Islamabad, PAK
| | - Farhan Eitezaz
- Plastic Surgery, Shifa International Hospital, Islamabad, PAK
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Asilian A, Honarjou N, Faghihi G, Saber M, Mozafarpoor S, Hafezi H. An experience of slow‐Mohs micrographic surgery for the treatment of Dermatofibrosarcoma protuberans: A long‐term cohort study. J Cosmet Dermatol 2020; 19:2701-2705. [DOI: 10.1111/jocd.13319] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/05/2019] [Revised: 12/31/2019] [Accepted: 01/21/2020] [Indexed: 01/05/2023]
Affiliation(s)
- Ali Asilian
- Department of Dermatology School of Medicine Isfahan University of Medical Sciences Isfahan Iran
| | - Navid Honarjou
- Faculty of Medicine Isfahan University of Medical Sciences Isfahan Iran
| | - Gita Faghihi
- Department of Dermatology School of Medicine Isfahan University of Medical Sciences Isfahan Iran
| | - Mina Saber
- Department of Dermatology School of Medicine Isfahan University of Medical Sciences Isfahan Iran
| | - Samaneh Mozafarpoor
- Skin Diseases and Leishmaniasis Research Center School of Medicine Isfahan University of Medical Sciences Isfahan Iran
| | - Hossein Hafezi
- Skin Diseases and Leishmaniasis Research Center School of Medicine Isfahan University of Medical Sciences Isfahan Iran
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Malan M, Xuejingzi W, Quan SJ. The efficacy of Mohs micrographic surgery over the traditional wide local excision surgery in the cure of dermatofibrosarcoma protuberans. Pan Afr Med J 2019; 33:297. [PMID: 31692830 PMCID: PMC6815477 DOI: 10.11604/pamj.2019.33.297.17692] [Citation(s) in RCA: 21] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/15/2018] [Accepted: 07/09/2019] [Indexed: 12/11/2022] Open
Abstract
Usually most patients with dermatofibrosarcoma protuberans (DFSP) may present rather late when the tumor is in protuberant phase due to its rarity and indolent onset. It has a high propensity for local recurrence and destructive nature. Management of DFSP requires a biopsychosocial and Multidisplinary approach regardless of the clinical or immunohistochemical variant. Surgery is the Gold standard management of localized disease. DFSP rarely exhibits any lymphatic or hematogenous dissemination. It is because of its high recurrence rate associated with Wide Local Excision (WLE), the introduction of Mohs micrographic surgery (MMS) has really helped in reducing the rates of recurrence of DFSP. Thus, the aim of this meta-analysis and systemic review is to advocate for MMS over WLE for DFSP and other cutaneous malignancies using DFSP as a prototype. The objective of this study were to conduct a meta-analysis on comparative surgical methods used in the cure of DFSP with regards to WLE verses MMS, to evaluate the cure rates with relation to recurrence rates, offer a recommendation on the various treatment modalities based on the location of lesion, and use of adjuvant therapy in different clinical-medical setups. A comprehensive retrospective analysis search in EMBASE, Google Scholar and Medline (PubMed) for studies published from 2008 to 2018 containing the surgical management of DFSP with WLE verses MMS were reviewed. Five studies of moderate-quality evidence (level B) with a pooled patient load of 684 was analyzed and found for recurrence of DFSP after WLE and MMS to be 9.10% and 2.72% respectively after an average follow-up time for both groups of 5.32 years with a female predominance of 1.58. The trunk is the commonest site for the DFSP lesion which was at 52.80% then the upper and lower extremities zones and the head and neck zones at 31.75% and 15.45% respectively. The pooled adjusted odds ratio (OR) analysis indicated that there was a direct relationship with regards the reduced recurrence rate of DFSP in the MMS group compared to the WLE group (OR:0.31;95%; CI :0.17-0.56). Furthermore, there was significant association between the reduced recurrence rate with the MMS in DFSP patients with a statistical P-value of 0.0001 at 95% CI. The expected increased recurrence rate by zones was in WLE head and neck zone at 38.19% then trunk and extremities zone at 13.34%. In the MMS group it was at of 23.4% as compared to 16.0% in the head and neck zone. Mohs Micrographic Surgery (MMS) is more efficacious in the cure rate and recurrence reduction of DFSP and should be advocated for as first line therapy especially in high recurrence prone zones.
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Affiliation(s)
- Malumani Malan
- Department of Dermatology and Venereology at Zhongnan Hospital of Wuhan University, Wuhan City, Hubei Province, Peoples Republic of China
- Department of Dermatology and Venereology at Zhongnan Hospital of Wuhan University, Wuhan City, Hubei Province, Peoples Republic of China
| | - Wu Xuejingzi
- Department of Dermatology and Venereology at Zhongnan Hospital of Wuhan University, Wuhan City, Hubei Province, Peoples Republic of China
| | - Song Ji Quan
- Head of Department of Dermatology and Venereology at Zhongnan Hospital of Wuhan University, Wuhan City, Hubei Province, People's Republic of China
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David MP, Funderburg A, Selig JP, Brown R, Caliskan PM, Cove L, Dicker G, Hoffman L, Horne T, Gardner JM. Perspectives of Patients With Dermatofibrosarcoma Protuberans on Diagnostic Delays, Surgical Outcomes, and Nonprotuberance. JAMA Netw Open 2019; 2:e1910413. [PMID: 31469398 PMCID: PMC6724159 DOI: 10.1001/jamanetworkopen.2019.10413] [Citation(s) in RCA: 28] [Impact Index Per Article: 4.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/28/2022] Open
Abstract
IMPORTANCE Dermatofibrosarcoma protuberans (DFSP) may have a deceptively benign clinical appearance, including a nonprotuberant presentation. Patients with DFSP often perceive misdiagnoses and delays in receiving a diagnosis. Use of existing, patient-designed Facebook patient support groups (FBSGs) to recruit large numbers of patients with rare diseases may be an effective novel research method. OBJECTIVES To collaborate with patients with rare disease through social media and answer questions important to both patients and the medical field, including sources of diagnostic delay, risk of recurrence, and flat presentation of DFSP. DESIGN, SETTING, AND PARTICIPANTS A multiple-choice survey created by a team of medical practitioners and patients with DFSP was administered to 214 patients with DFSP or family members from international DFSP FBSGs and a nonprofit foundation patient database via Lime Survey from October 30 to November 20, 2015. The survey asked questions designed to determine risk of recurrence and metastasis, surgical outcomes, sources of diagnostic delay, symptoms of recurrence, number of recurrences, scar size, and number of clinicians seen before biopsy. Statistical analysis was performed from January 1, 2016, to April 1, 2019. MAIN OUTCOMES AND MEASURES The study goal was to collect at least 200 survey responses. RESULTS Of 214 survey respondents (169 females and 45 males; mean [SD] age, 40.7 [12.1] years; range, <1 to 72 years), 199 were patients with DFSP and 15 were family members. Delays occurred between the patient noticing the DFSP lesion and receiving a diagnosis of DFSP (median, 4 years; range, <1 to 42 years). Most patients (112 [52.3%]) believed that they received a misdiagnosis at some point: by dermatologists (35 of 107 [32.7%]), primary care clinicians (80 of 107 [74.8%]), or another type of physician (27 of 107 [25.2%]). The most frequent prebiopsy clinical suspicion included cyst (101 [47.2%]), lipoma (30 [14.0%]), and scar (17 [7.9%]). Many patients first noticed their DFSP as a flat plaque (87 of 194 [44.8%]). Of these lesions, 73.6% (64 of 87) became protuberant eventually. Surgical treatments included Mohs micrographic surgery (56 of 194 [28.9%]), wide local excision (122 of 194 [62.9%]), and conservative excision (16 of 194 [8.2%]). The reported rate of recurrence was 5.4% (3 of 56) for Mohs micrographic surgery, 7.4% (9 of 122) for wide local excision, and 37.5% (6 of 16) for conservative excision. The higher rate of recurrence for conservative excision was significant (P = .001); there was no significant difference in the rate of recurrence between Mohs micrographic surgery and wide local excision (P = .76). CONCLUSIONS AND RELEVANCE This study reports what appears to be disease-relevant statistics from the largest survey of patients with DFSP to date. Because of the dissonance between the name of the neoplasm and its clinical presentation, the alternative term dermatofibrosarcoma, often protuberant is proposed. This study suggests that FBSGs are useful tools in medical research, providing rapid access to large numbers of patients with rare diseases and enabling synergistic collaborations between patients and medical researchers.
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Affiliation(s)
- Marjorie Parker David
- Department of Pathology and Laboratory Medicine, University of Texas Health Science Center at San Antonio
- Department of Pathology, University of Arkansas for Medical Sciences, Little Rock
| | - Ashley Funderburg
- College of Nursing, University of Arkansas for Medical Sciences, Little Rock
| | - James P. Selig
- College of Public Health, University of Arkansas for Medical Sciences, Little Rock
| | - Rebecca Brown
- Dermatofibrosarcoma Protuberans Patient Advisory Board, International Group, College of Nursing, University of Arkansas for Medical Sciences, Little Rock
| | - Pip M. Caliskan
- Dermatofibrosarcoma Protuberans Patient Advisory Board, International Group, College of Nursing, University of Arkansas for Medical Sciences, Little Rock
| | - Lee Cove
- Dermatofibrosarcoma Protuberans Patient Advisory Board, International Group, College of Nursing, University of Arkansas for Medical Sciences, Little Rock
| | - Gayle Dicker
- Dermatofibrosarcoma Protuberans Patient Advisory Board, International Group, College of Nursing, University of Arkansas for Medical Sciences, Little Rock
| | - Lori Hoffman
- Public Policy and External Affairs, Sarcoma Foundation of America, Damascus, Maryland
| | - Tammi Horne
- Dermatofibrosarcoma Protuberans Patient Advisory Board, International Group, College of Nursing, University of Arkansas for Medical Sciences, Little Rock
| | - Jerad M. Gardner
- Department of Pathology, University of Arkansas for Medical Sciences, Little Rock
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Role of postoperative radiotherapy in dermatofibrosarcoma protuberans: a propensity score-matched analysis. Radiat Oncol 2019; 14:20. [PMID: 30696463 PMCID: PMC6350344 DOI: 10.1186/s13014-019-1226-z] [Citation(s) in RCA: 15] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/11/2018] [Accepted: 01/20/2019] [Indexed: 12/03/2022] Open
Abstract
Objective This study aimed to evaluate the role of postoperative radiotherapy (RT) in dermatofibrosarcoma protuberans (DFSP) and identify the prognostic factors influencing the disease-free survival (DFS). Methods A total of 184 patients with DFSP were analyzed from 2000 to 2016. The regression model was used to examine the prognostic factors for DFS. Baseline covariates were balanced using a propensity score model. The role of RT was assessed by comparing the DFS of the surgery + RT group with that of the surgery group. Results The median follow-up was 58 months (range, 6–203 months). The 5-year DFS rate was 89.8%. The univariate analysis showed that age ≥ 50 years, presence of fibrosarcoma, margins < 2 cm, and tumor size ≥5 cm were associated with worse DFS (P = 0.002, P < 0.001, P = 0.030, and P = 0.032, respectively). The multivariate Cox regression model revealed that age, margin width, lesion number, and histological subtype independently affected DFS. The Ki-67 expression was related to age and histological subtype. Patients with Ki-67 ≥ 17% showed a worse DFS than those with Ki-67 < 17% (35.8% vs 87.8%, P = 0.002). In the matched cohort, DFS was significantly higher in the S + RT group than in the S group (5-year DFS, 88.1% vs 56.2%, P = 0.044). Conclusions Age, margin width, lesion number, and histological subtype were independent risk factors for DFS in patients with DFSP. The high expression of Ki-67 could predict a poor prognosis. Postoperative RT could improve DFS for patients with DFSP.
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Llombart B, Serra C, Requena C, Alsina M, Morgado-Carrasco D, Través V, Sanmartín O. Guidelines for Diagnosis and Treatment of Cutaneous Sarcomas: Dermatofibrosarcoma Protuberans. ACTAS DERMO-SIFILIOGRAFICAS 2018. [DOI: 10.1016/j.adengl.2018.05.034] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022] Open
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Penel N, El Bedoui S, Robin YM, Decanter G. Dermatofibrosarcome : prise en charge. Bull Cancer 2018; 105:1094-1101. [DOI: 10.1016/j.bulcan.2018.08.008] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/03/2018] [Accepted: 08/16/2018] [Indexed: 01/14/2023]
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Lee S, Oh Y, Nam K, Oh B, Roh M, Chung K. Mohs micrographic surgery for dermatofibrosarcoma protuberans: comparison of frozen and paraffin techniques. J Eur Acad Dermatol Venereol 2018; 32:2171-2177. [DOI: 10.1111/jdv.15201] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/11/2018] [Accepted: 07/12/2018] [Indexed: 11/28/2022]
Affiliation(s)
- S.H. Lee
- Department of Dermatology; Seoul National University Hospital; Seoul Korea
- Institute of Human Environment Interface Biology; Seoul National University; Seoul Korea
| | - Y. Oh
- Department of Dermatology; Cutaneous Biology Research Institute; Yonsei University College of Medicine; Seoul Korea
| | - K.A. Nam
- Department of Dermatology; Cutaneous Biology Research Institute; Yonsei University College of Medicine; Seoul Korea
| | - B. Oh
- Department of Dermatology; Cutaneous Biology Research Institute; Yonsei University College of Medicine; Seoul Korea
| | - M.R. Roh
- Department of Dermatology; Cutaneous Biology Research Institute; Yonsei University College of Medicine; Seoul Korea
| | - K.Y. Chung
- Department of Dermatology; Cutaneous Biology Research Institute; Yonsei University College of Medicine; Seoul Korea
- Brain Korea 21 Project for Medical Science; Seoul Korea
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Llombart B, Serra C, Requena C, Alsina M, Morgado-Carrasco D, Través V, Sanmartín O. Guidelines for Diagnosis and Treatment of Cutaneous Sarcomas: Dermatofibrosarcoma Protuberans. ACTAS DERMO-SIFILIOGRAFICAS 2018; 109:868-877. [PMID: 30539729 DOI: 10.1016/j.ad.2018.05.006] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/06/2017] [Revised: 04/21/2018] [Accepted: 05/23/2018] [Indexed: 12/26/2022] Open
Abstract
Sarcomas comprise a broad group of tumors, many of whose biological behavior and aggressiveness differ from one type to another. The therapeutic approach is generally multidisciplinary and often complex. Developments in surgical and oncological dermatology during the last few decades have positioned dermatologists as specialists in the diagnosis and treatment of skin cancer. The aim of this article is to review the main soft tissue sarcomas that typically affect the skin. Dermatofibrosarcoma protuberans is a low-grade malignant sarcoma. It exhibits slow-growth, is locally invasive, and has low metastatic potential (<3%). Mohs micrographic surgery is the treatment of choice. The COL1A1-PDGFB translocation should be analyzed in cases of unclear diagnosis and when it is necessary to identify candidates for tyrosine kinase inhibitors. Imatinib is indicated for the treatment of locally advanced and metastatic dermatofibrosarcoma protuberans.
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Affiliation(s)
- B Llombart
- Servicio de Dermatología, Instituto Valenciano de Oncología, Valencia, España.
| | - C Serra
- Servicio de Dermatología, Instituto Valenciano de Oncología, Valencia, España
| | - C Requena
- Servicio de Dermatología, Instituto Valenciano de Oncología, Valencia, España
| | - M Alsina
- Servicio de Dermatología, Hospital Clínic de Barcelona, Universitat de Barcelona, Barcelona, España
| | - D Morgado-Carrasco
- Servicio de Dermatología, Hospital Clínic de Barcelona, Universitat de Barcelona, Barcelona, España
| | - V Través
- Servicio de Anatomía Patológica, Instituto Valenciano de Oncología, Valencia, España
| | - O Sanmartín
- Servicio de Dermatología, Instituto Valenciano de Oncología, Valencia, España
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Lyu A, Wang Q. Dermatofibrosarcoma protuberans: A clinical analysis. Oncol Lett 2018; 16:1855-1862. [PMID: 30008876 DOI: 10.3892/ol.2018.8802] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/21/2017] [Accepted: 04/24/2018] [Indexed: 01/13/2023] Open
Abstract
Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous tumor with a high incidence of misdiagnosis. DFSP has a high rate of recurrence but a low rate of metastasis. In the present study, retrospective analyses were performed on the clinical features, differential diagnosis and treatment of patients with DFSP to improve our understanding of the disease and allow more effective treatment measures to be implemented. The present study investigated the clinicopathological features of 70 pathologically confirmed cases of DFSP at the First Affiliated Hospital of Zhengzhou University (Zhengzhou, China) between March 2012 and 2017. The primary endpoint was recurrence rate. Three cases were analyzed in detail. The results revealed that 7 of the 41 primary patients (follow-up at 2.7 years) had recurrence (17.1%), compared with 11 (37.9%) of the 29 recurrent patients (follow-up at 2.0 years, P=0.049). Of the 59 patients with DFSP (follow-up at 2.6 years), 12 had recurrence (20.3%) compared with 6 (54.6%) of the 11 patients with fibrosarcomatous DFSP (FS-DFSP; follow-up at 2.1 years, P=0.045). DFSP requires diagnosis by pathological examination, and surgical resection is the main treatment. DFSP demonstrated a high recurrence rate, with the degree of malignancy increasing following multiple recurrences. FS-DFSP had a higher risk of local recurrence and distant metastasis, and a higher degree of malignancy than classic DFSP. These data may be useful to guide clinicians to improve decisions in the treatment of patients with DFSP.
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Affiliation(s)
- Anqi Lyu
- Department of Plastic Surgery, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan 450052, P.R. China
| | - Qiying Wang
- Department of Plastic Surgery, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan 450052, P.R. China
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Dermatofibrosarcoma Protuberans of the Neck: A Brief Review of the Literature. Indian J Otolaryngol Head Neck Surg 2018; 71:369-372. [PMID: 31741988 DOI: 10.1007/s12070-018-1314-7] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/03/2018] [Accepted: 03/20/2018] [Indexed: 10/17/2022] Open
Abstract
Dermatofibrosarcome protuberance (DFSP) is a rare, slow growing soft tissue sarcoma with fibroblastic origin, which has a great, potential of local invasion and recurrence. Because of its unspecific appearance and slow growth features it is commonly misdiagnosed as other benign lesions that leads to a late accurate diagnosis or an inadequate initial treatment. A young female patient with DFSP on the neck who was initially misdiagnosed as lipoma and then underwent inadequate initial resection is presented in this case.
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Verbruggen C, Ricard A, Cogrel O, Bondaz M, Carrier S. Marges d’exérèse des dermatofibrosarcomes cervico-faciaux par technique de Slow-Mohs : étude clinique rétrospective sur 20 cas. ANN CHIR PLAST ESTH 2018; 63:47-53. [DOI: 10.1016/j.anplas.2017.06.005] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/25/2017] [Accepted: 06/21/2017] [Indexed: 12/11/2022]
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Lin JY, Sheen-Chen SM, Hsu W, Huang CC, Ko SF, Eng HL. Dermatofibrosarcoma Protuberans of the Breast. TUMORI JOURNAL 2018; 94:861-3. [DOI: 10.1177/030089160809400616] [Citation(s) in RCA: 14] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Abstract
Background Dermatofibrosarcoma protuberans is an uncommon, locally aggressive but rarely metastasizing tumor of the deep dermis and subcutaneous tissue. It may occur at almost any site but is most common in the trunk and extremities. Dermatofibrosarcoma protuberans of the breast has been rarely reported. Case Presentation A 49-year-old woman without a significant medical history presented with a right breast mass that had been present for more than 10 years. Recently, progressive enlargement of the mass was noted. Physical examination showed a firm, well-defined, non-tender, mobile, erythematous 2 × 2 cm mass in the upper outer part of the right breast. Breast sonography showed a 2.3 × 1 cm slightly heteroechoic nodule with uneven anterior contours located in the dermal layer, highly suggestive of a dermal lesion. Wide excision of the mass was performed. Microscopically, the hypercellular tumor was composed of relatively monomorphic spindle cells forming a storiform pattern around the sweat glands of the deep dermis and the subcutaneous fat vacuoles. The tumor cells showed strong immunoreactivity for CD34. The final histological diagnosis was dermatofibrosarcoma protuberans. Conclusions Dermatofibrosarcoma protuberans of the breast, though uncommon, does exist. With the awareness of this entity can a prompt diagnosis be made and the disease be properly managed.
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Affiliation(s)
- Jong-Yueh Lin
- Department of Surgery, Chang Gung Memorial Hospital, Kaohsiung Medical Center College of Medicine, Chang Gung University, Niao-Sung Hsiang, Kaohsiung Hsien, Taiwan
| | - Shyr-Ming Sheen-Chen
- Department of Surgery, Chang Gung Memorial Hospital, Kaohsiung Medical Center College of Medicine, Chang Gung University, Niao-Sung Hsiang, Kaohsiung Hsien, Taiwan
| | - Wayne Hsu
- Department of Surgery, Chang Gung Memorial Hospital, Kaohsiung Medical Center College of Medicine, Chang Gung University, Niao-Sung Hsiang, Kaohsiung Hsien, Taiwan
| | - Chao-Cheng Huang
- Department of Pathology, Chang Gung Memorial Hospital, Kaohsiung Medical Center College of Medicine, Chang Gung University, Niao-Sung Hsiang, Kaohsiung Hsien, Taiwan
| | - Sheung-Fat Ko
- Department of Diagnostic Radiology, Chang Gung Memorial Hospital, Kaohsiung Medical Center College of Medicine, Chang Gung University, Niao-Sung Hsiang, Kaohsiung Hsien, Taiwan
| | - Hock-Liew Eng
- Department of Diagnostic Radiology, Chang Gung Memorial Hospital, Kaohsiung Medical Center College of Medicine, Chang Gung University, Niao-Sung Hsiang, Kaohsiung Hsien, Taiwan
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Wollina U, Langner D, Schönlebe J, França K, Lotti T, Tchernev G. Dermatofibrosarcoma Protuberans: Retrospective Single Center Analysis Over 16 Years. Open Access Maced J Med Sci 2018; 6:35-37. [PMID: 29483976 PMCID: PMC5816309 DOI: 10.3889/oamjms.2018.030] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/02/2017] [Revised: 09/22/2017] [Accepted: 09/24/2017] [Indexed: 11/23/2022] Open
Abstract
Dermatofibrosarcoma protuberans (DFSP) is rare mesenchymal neoplasia with a high risk of local recurrence but a low risk of metastatic spread. Tumor cells express CD34 and show a characteristic translocation t(17;22)(q22;q13). We analysed the documented cases at the Department of Dermatology and Allergology between 08/2001 and 08/2017. The diagnosis had been confirmed by histology and immunohistology in all cases. We identified four adults and a pediatric patient with DFSP. All patients were treated by wide surgical excision and controlled by three-dimensional histologic margin control. We observed no recurrence and no metastatic spread. We discuss prognostic factors and emerging treatments.
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Affiliation(s)
- Uwe Wollina
- Städtisches Klinikum Dresden - Department of Dermatology and Allergology, Dresden, Sachsen, Germany
| | - Dana Langner
- Städtisches Klinikum Dresden - Department Dermatology and Allergology, Dresden, Germany
| | - Jacqueline Schönlebe
- Städtisches Klinikum Dresden - Institute of Pathology "Georg Schmorl", Dresden, Germany
| | - Katlein França
- Department of Dermatology and Cutaneous Surgery, Department of Psychiatry & Behavioral Sciences; Institute for Bioethics and Health Policy, University of Miami Miller School of Medicine, Miami, FL, USA
| | - Torello Lotti
- University of Rome, Institute of Deramtology, Rome, Italy
| | - Georgi Tchernev
- Department of Dermatology, Venereology and Dermatologic Surgery, Medical Institute of Ministry of Interior, Sofia, Bulgaria.,Onkoderma Policlinic for Dermatology and Dermatologic Surgery, Sofia, Bulgaria
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Dermatofibrosarcoma protuberans of the cheek. JOURNAL OF CANCER RESEARCH AND PRACTICE 2017. [DOI: 10.1016/j.jcrpr.2017.03.001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022] Open
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Abstract
OPINION STATEMENT Dermatofibrosarcoma protuberans (DFSP) is a slow growing tumor with a very low metastatic potential but with significant subclinical extension and great capacity for local destruction. Thus, the first surgeon approached with such challenging tumor must attempt to cure the patient with a method that spares healthy tissue and ensures an optimal oncological, functional, and esthetic result. The treatment of DFSP often requires a multidisciplinary approach. Depending on location, dermatologic surgeons, surgical oncologists, head and neck surgeons, neurosurgeons, plastic surgeons, and occasionally medical oncologists may be involved with the management. Mohs micrographic surgery (MMS) is the preferred method when available. In our institution, most of the DFSP cases are often advanced cases; thus, dermatologic surgeons obtain clear margins peripherally and other surgical specialties assist with resection of the fascia and any critical deeper structures. When MMS is not available, wide local excision (at least 2- to 3-cm margins of resection) with exhaustive pathologic assessment of margin status is recommended, and it is best to confirm tumor extirpation prior to any reconstruction. Subclinical extension of the tumor could be related to the size; how long it has been growing or histological markers that are unknown right now. No clinical trials comparing MMS vs WLE are available, and further research should be focused on these subjects as well as the use of imatinib and other targeted therapies for recurrent and metastatic tumors and for neoadjuvant treatment.
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Affiliation(s)
- Alvaro E Acosta
- Dermatology Department, Instituto Nacional de Cancerología, Universidad Nacional de Colombia, Carrera 19C No. 90-14, Bogotá, Colombia.
| | - Catalina Santa Vélez
- Dermatology Oncology Universidad Militar Nueva Granada, Instituto Nacional de Cancerología, Calle 1a No. 9-85, Bogotá, Colombia
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Veronese F, Boggio P, Tiberio R, Gattoni M, Fava P, Caliendo V, Colombo E, Savoia P. Wide local excision vs. Mohs Tübingen technique in the treatment of dermatofibrosarcoma protuberans: a two-centre retrospective study and literature review. J Eur Acad Dermatol Venereol 2017; 31:2069-2076. [PMID: 28573714 DOI: 10.1111/jdv.14378] [Citation(s) in RCA: 42] [Impact Index Per Article: 5.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/28/2016] [Accepted: 05/09/2017] [Indexed: 12/23/2022]
Abstract
BACKGROUND Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade mesenchymal skin tumour, characterized by slow infiltrative growth and common local recurrence, with infrequent distant metastases. OBJECTIVE The aim of this study is to better clarify clinicopathological characteristics of this tumour and to evaluate the cure rates of Mohs Tübingen technique (MTT) and wide local excision (WLE). Eventually, we perform a literature review to compare our experience with published data. METHODS A retrospective review was conducted on 135 patients diagnosed, treated and followed up between 1997 and 2014 at two different institutions. Sixty-two patients underwent to WLE and 73 to MTT. The primary end-points were the following: percentage of recurrences, time to progression and recurrence annual risk rate. Then, the PubMed database was searched for DFSP case series treated with standard surgical resection (SSR), WLE, Mohs' micrographic surgery (MMS) and MTT. The annual risk rate of recurrence calculated and reported for the four separate procedures was pooled to compare them. RESULTS Five of the 62 patients with WLE (8.1%) experienced recurrences after a mean follow-up of 4.7 years; the percentage of recurred patients 9 years after MTT was 5.5%, and the annual recurrence risk rate of 0.6%. Pooling these data with those from literature, the recurrence rate varies from 26% to 60% for SSR, from 0% and 41% for WLE, from 0% and 8.3% for MMS and from 0% to 5.5% for MTT. The lowest annual recurrence risk rate was found for MTT. CONCLUSION Significantly lower recurrence rates were recorded in patients treated with classic or Tübingen Mohs' technique. To the best of our knowledge, our case series is the widest treated with MTT ever described in the literature; these data may be useful to guide clinicians in the choice of the gold standard treatment for Dermatofibrosarcoma protuberans.
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Affiliation(s)
- F Veronese
- Department of Health Science, University of Eastern Piedmont, Novara, Italy
| | - P Boggio
- SCDU Dermatology, AOU Maggiore della Carità, Novara, Italy
| | - R Tiberio
- SCDU Dermatology, AOU Maggiore della Carità, Novara, Italy
| | - M Gattoni
- SSVD Dermatology P.O. S. Andrea, Vercelli, Italy
| | - P Fava
- SCDU Dermatology, AOU Città della Salute e della Scienza, Torino, Italy
| | - V Caliendo
- SCDU Dermatology, AOU Città della Salute e della Scienza, Torino, Italy
| | - E Colombo
- Department of Translational Medicine, University of Eastern Piedmont, Novara, Italy
| | - P Savoia
- Department of Health Science, University of Eastern Piedmont, Novara, Italy
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A Comparison of Mohs Micrographic Surgery and Wide Local Excision for Treatment of Dermatofibrosarcoma Protuberans With Long-Term Follow-up: The Mayo Clinic Experience. Dermatol Surg 2017; 43:98-106. [PMID: 27749444 DOI: 10.1097/dss.0000000000000910] [Citation(s) in RCA: 81] [Impact Index Per Article: 10.1] [Reference Citation Analysis] [Abstract] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
Abstract
BACKGROUND Dermatofibrosarcoma protuberans (DFSP) is a soft tissue tumor with slow infiltrative growth and local recurrence if inadequately excised. OBJECTIVE To compare long-term outcomes after Mohs micrographic surgery (MMS) and wide local excision (WLE). MATERIALS AND METHODS Records of patients with DFSP surgically treated with WLE or MMS from January 1955 through March 2012 were retrospectively reviewed. RESULTS Mean follow-up for patients treated with MMS (n = 67) and WLE (n = 91) was 4.8 and 5.7 years, respectively. Twenty-eight patients (30.8%) with WLE had recurrence (mean, 4.4 years), whereas only 2 (3.0%) with MMS had recurrence (1.0 and 2.6 years). Recurrence-free survival rates at 1, 5, 10, and 15 years were significantly higher with MMS (p < .001). Mean preoperative lesion sizes were similar (5-6 cm) between the 2 groups, whereas mean (standard deviation) postoperative defect sizes were 10.7 (4.3) cm and 8.8 (5.5) cm for WLE and MMS, respectively (p = .004). Primary closure was used for 73% of MMS cases, whereas WLE more commonly used flaps, grafts, or other closures (52%). Two Mohs layers typically were required for margin control. CONCLUSION Surgical excision with meticulous histologic evaluation of all surgical margins is needed for DFSP treatment to achieve long-term high cure rates and low morbidity.
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