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Sasi A, Ganguly S, Thakar A, Sikka K, Agarwala S, Pushpam D, Kumar A, Biswas B, Meel R, Biswas A, Barwad A, Mridha AR, Bakhshi S. Nuances in the Treatment of Ewing Sarcoma of the Head and Neck in a Low-Middle-Income Country Setting: A Multi-Disciplinary Approach. Head Neck 2025; 47:1690-1698. [PMID: 39844767 DOI: 10.1002/hed.28086] [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: 10/26/2024] [Revised: 01/14/2025] [Accepted: 01/15/2025] [Indexed: 01/24/2025] Open
Abstract
BACKGROUND Ewing sarcoma of the head and neck (ES-HN) is a rare subsite of ES, where therapeutic outcomes need to be explored further. METHODS This retrospective study includes ES-HN patients registered at our center between 2003 and 2019. Demographic details and treatment outcomes were recorded from the hospital database. Prognostic factors for survival were identified by Cox regression. RESULTS Eighty-five patients were included. Metastatic disease was seen in nine patients (10.59%). Local therapy included radiotherapy alone (n = 38; 44.7%), surgery plus radiotherapy (n = 15; 17.6%), or surgery alone (n = 8; 9.4%). The median overall survival (OS) was 37.4 months. On multivariable analysis, osseous primary (HR 0.40; p = 0.009) and male sex (HR 0.43; p = 0.023) were associated with superior OS. Leucocytosis (HR 3.46; p = 0.001) was associated with inferior OS. CONCLUSIONS ES-HN has favorable biology with metastases being rare at baseline. However, leucocytosis, extra-osseous disease, and female sex are poor prognostic factors. In resource-challenged settings, difficulties in administering local therapy may contribute to inferior outcomes.
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Affiliation(s)
- Archana Sasi
- Division of Leukemia, Dana Farber Cancer Institute, Brookline, Massachusetts, USA
| | - Shuvadeep Ganguly
- Department of Medical Oncology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India
| | - Alok Thakar
- Department of Otorhinolaryngology, All India Institute of Medical Sciences, New Delhi, India
| | - Kapil Sikka
- Department of Otorhinolaryngology, All India Institute of Medical Sciences, New Delhi, India
| | - Sandeep Agarwala
- Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India
| | - Deepam Pushpam
- Department of Medical Oncology, Dr. B.R.A. Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India
| | - Akash Kumar
- Department of Medical Oncology, National Cancer Institute, Jhajjar, Haryana, India
| | - Bivas Biswas
- Department of Medical Oncology, Apollo Gleneagles Hospitals, Kolkata, India
| | - Rachna Meel
- Department of Ophthalmology, Dr Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India
| | - Ahitagni Biswas
- Department of Radiation Oncology, Dr. B.R.A. Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India
| | - Adarsh Barwad
- Department of Pathology, All India Institute of Medical Sciences, New Delhi, India
| | - Asit Ranjan Mridha
- Department of Pathology, All India Institute of Medical Sciences, New Delhi, India
| | - Sameer Bakhshi
- Department of Medical Oncology, Dr. B.R.A. Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India
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Callan AK, Alexander JH, Montgomery NI, Lindberg AW, Scharschmidt TJ, Binitie O. Contemporary surgical management of osteosarcoma and Ewing sarcoma. Pediatr Blood Cancer 2025; 72 Suppl 2:e31374. [PMID: 39410791 DOI: 10.1002/pbc.31374] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/16/2024] [Revised: 09/23/2024] [Accepted: 09/25/2024] [Indexed: 04/08/2025]
Abstract
The incidence of malignant bone tumors has remained relatively stable over the past two decades between 8% and 9% per 1,000,000 in North America. Multidisciplinary treatment is paramount for optimal care combining surgical resection, chemotherapy, and rehabilitation. Surgical treatment aims for a negative margin resection of the sarcoma with a personalized reconstruction plan. Limb salvage surgery (LSS) is possible in the majority of cases; however, amputation (including rotationplasty) may be required or preferred. Reconstruction can be achieved utilizing endoprostheses, allograft, autograft, or a combination of these techniques. Emerging technologies such as 3D printing of implants and cutting guides, and intraoperative navigation have helped to improve options for LSS.
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Affiliation(s)
- Alexandra K Callan
- Department of Orthopaedics, University of Texas Southwestern Medical Center, Dallas, Texas, USA
| | - John H Alexander
- Department of Orthopaedics, The Ohio State University, Columbus, Ohio, USA
| | | | | | | | - Odion Binitie
- Department of Sarcoma, Moffitt Cancer Center, Tampa, Florida, USA
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Hahn D, Velasquez L, Muller N, Lawless M. Retroperitoneal Ewing Sarcoma Masquerading as an Intraabdominal Abscess in a Teenager: A Rare Diagnostic Challenge. Cureus 2025; 17:e80853. [PMID: 40255709 PMCID: PMC12007975 DOI: 10.7759/cureus.80853] [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: 03/19/2025] [Indexed: 04/22/2025] Open
Abstract
Retroperitoneal Ewing sarcoma is a rare and aggressive extraskeletal variant of Ewing sarcoma, often presenting with nonspecific symptoms that can delay diagnosis. We discuss the case of a 19-year-old male who presented to the emergency room with progressive left abdominal and flank pain. Imaging revealed a retroperitoneal, necrotic mass and biopsy with immunohistochemistry confirmed retroperitoneal Ewing sarcoma with NKX2.2 and CD99 positivity. Fluorescence in situ hybridization (FISH) analysis showed that 98% of cells had an EWSR1 gene rearrangement. For treatment, the patient underwent a CT-guided biopsy and chemotherapy was initiated. The sometimes-insidious progression of retroperitoneal Ewing sarcoma, coupled with the retroperitoneal space's ability to accommodate tumors without significant symptoms, often leads to late-stage diagnosis and poor prognosis. This report emphasizes the importance of early recognition and highlights the need for further research into optimal diagnostic protocols and treatment strategies.
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Affiliation(s)
- Daniel Hahn
- Internal Medicine, Touro College of Osteopathic Medicine, New York, USA
| | - Lauren Velasquez
- Internal Medicine, Touro College of Osteopathic Medicine, New York, USA
| | - Nickolas Muller
- Internal Medicine, Touro College of Osteopathic Medicine, New York, USA
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Xiao F, Wang W. Development and verification of prognostic nomogram for extraskeletal Ewing's sarcoma based on the SEER database. Heliyon 2025; 11:e40854. [PMID: 39801991 PMCID: PMC11719304 DOI: 10.1016/j.heliyon.2024.e40854] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/14/2024] [Revised: 11/29/2024] [Accepted: 11/29/2024] [Indexed: 01/16/2025] Open
Abstract
Background Extraskeletal Ewing's sarcoma (EES) is a rare tumor, and there is currently no predictive model for overall survival of EES patients. This study sought to use data from the Surveillance, Epidemiology, and End Results (SEER) database to develop a clinical predictive model that could be used to assess the prognosis of EES patients. Methods We selected and downloaded prognostic data on 356 patients diagnosed with extraskeletal Ewing's sarcoma based on screening criteria, These patients were distributed between 2004 and 2015. 356 patients were randomly divided into a training cohort (70 %) and an internal validation cohort (30 %). After univariate or multifactor Cox regression analysis, the relevant variables were screened and a nomogram was constructed. The consistency index (C-index), calibration chart and receiver operating characteristic (ROC) curve were used to evaluate the established nomogram. The clinical utility of the model was verified by clinical decision curve.Study conducted and outcomes reported according to STROBE statement. Results After multifactor regression analysis, we identified five factors that were significantly associated with EES prognosis, and subsequently established a nomogram. Verification data showed that the C-index of this nomogram was 0.829 (95 % CI 0.774-0.884). the AUCs of the nomogram for predicting the 3- and 5-year OS were 0.91 and 0.863. the calibration curves and Decision curve analysis showed that nomogram could predict the prognosis of EES patients. Conclusion Stage, age, tumour size, chemotherapy, and surgery may be independent prognostic factors for EES. our study produced a survival nomogram that can be used to predict the prognosis of patients with EES and validated its performance, which may help clinicians evaluate the condition of patients with EES and choose personalised treatment.
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Affiliation(s)
- Feipeng Xiao
- Affiliated Hengyang Hospital, Hunan Normal University (Hengyang Central Hospital), Hengyang, 421001, China
| | - Weizhen Wang
- Hunan Cancer Hospital, Affiliated Cancer Hospital of Xiangya School of Medicine, Central South University, Changsha, 410000, China
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Ziomek M, Placzke J, Urbanek K, Skóra T, Rutkowski P, Spałek MJ. Case report: successful treatment of primary intradural extramedullary extraskeletal Ewing sarcoma in adult patient with intralesional surgery, chemotherapy, and proton beam therapy of the cerebrospinal axis. Ther Adv Med Oncol 2024; 16:17588359241297868. [PMID: 39552637 PMCID: PMC11569501 DOI: 10.1177/17588359241297868] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/23/2024] [Accepted: 10/19/2024] [Indexed: 11/19/2024] Open
Abstract
Ewing sarcoma is a rare malignant neoplasm that primarily affects bone in children. Extraskeletal location is less common, while intradural extramedullary Ewing sarcoma (IEES) in adults is a casuistic phenomenon. Due to its rarity, a standardized treatment strategy for IEES has not been established. The clinical use of proton beam therapy (PBT) for craniospinal irradiation (CSI) in the treatment of IEES has not been reported in the literature. A 41-year-old previously healthy man presented with disabling gluteal and lower extremity pain, decreased sensation, and progressive paraparesis without sphincter dysfunction. Imaging showed intradural extramedullary spinal lesions. The patient underwent urgent surgery. Histology and immunohistochemistry suggested a poorly differentiated neuroendocrine tumor. Negative chromogranin staining and a high Ki67 index prompted further investigation. Next-generation sequencing later confirmed an EWSR1/FLI1 translocation, leading to the diagnosis of extraskeletal Ewing sarcoma. The patient received standardized chemotherapy with marked clinical improvement. PBT CSI was initiated but was interrupted due to COVID-19 and other complications. At 20 months follow-up, no recurrence was observed, and the patient reported an active life. Despite intra-spinal spread and multiple complications, intensive chemotherapy combined with PBT CSI led to a favorable outcome. CSI rather than focal radiotherapy should be considered for patients with IEES limited to the cerebrospinal axis. PBT may be used as an alternative to photon radiotherapy to better spare organs at risk.
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Affiliation(s)
- Mateusz Ziomek
- Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Skłodowska-Curie National Research Institute of Oncology, Warsaw, Poland
| | - Joanna Placzke
- Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Skłodowska-Curie National Research Institute of Oncology, Warsaw, Poland
| | - Konrad Urbanek
- Department of Radiotherapy, Maria Skłodowska-Curie National Research Institute of Oncology, Kraków Branch, Kraków, Poland
| | - Tomasz Skóra
- Department of Radiotherapy, Maria Skłodowska-Curie National Research Institute of Oncology, Kraków Branch, Kraków, Poland
| | - Piotr Rutkowski
- Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Skłodowska-Curie National Research Institute of Oncology, Warsaw, Poland
| | - Mateusz Jacek Spałek
- Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Skłodowska-Curie National Research Institute of Oncology, Roentgena 5, Warsaw 02-781, Poland
- Department of Radiotherapy I, Maria Skłodowska-Curie National Research Institute of Oncology, Warsaw, Poland
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Yang G, Huang L, Wu J, Huang B, Zhang C, Li S, Wang F, Jiang X. Case report and literature review: A young man with giant intra-abdominal Ewing sarcoma. Medicine (Baltimore) 2024; 103:e39983. [PMID: 39465729 PMCID: PMC11460867 DOI: 10.1097/md.0000000000039983] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/19/2024] [Indexed: 10/29/2024] Open
Abstract
RATIONALE Extraosseous Ewing sarcoma (EES) is a rare manifestation within the Ewing sarcoma tumor family (ESFT). Its clinical manifestations lack specificity, intestinal obstruction is the main symptom but can also present with abdominal pain, gastrointestinal bleeding, and other discomforts, making it prone to misdiagnosis as intestinal mesenchymal tumor. PATIENT CONCERNS A 29-year-old male was admitted to the hospital with intestinal obstruction symptoms and abdominal CT suggesting "left abdominal occupation." DIAGNOSIS The patient was initially misdiagnosed as intestinal mesenchymal tumor, and was later definitively diagnosed as abdominal Ewing sarcoma by postoperative pathology and genetic testing. INTERVENTIONS Due to the patient's surgical indication, surgical resection with exploratory laparotomy was performed and then the patient underwent systemic chemotherapy. OUTCOMES Intraoperatively, we found a 15-cm tumor originating from the proximal jejunum, with invasion into the peritoneum, duodenum, jejunum, and colon. Finally, the pathological report revealed Ewing sarcoma. LESSONS Giant abdominal Ewing sarcoma with a diameter of 15 cm is rare. Considering postoperative pathology and genetic testing, abdominal Ewing sarcoma was suspected. The patient was successfully treated using surgery.
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Affiliation(s)
- Guang Yang
- Gusu School, Nanjing Medical University, Nanjing, China
| | - Lining Huang
- Department of Hepatobiliary Surgery, the Affiliated Suzhou Hospital of Nanjing Medical University, Suzhou, China
| | - Jianwu Wu
- Department of Hepatobiliary Surgery, the Affiliated Suzhou Hospital of Nanjing Medical University, Suzhou, China
| | - Bo Huang
- Department of Hepatobiliary Surgery, the Affiliated Suzhou Hospital of Nanjing Medical University, Suzhou, China
| | - Cong Zhang
- Gusu School, Nanjing Medical University, Nanjing, China
| | - Song Li
- Department of Hepatobiliary Surgery, the Affiliated Suzhou Hospital of Nanjing Medical University, Suzhou, China
| | - Feng Wang
- Department of Hepatobiliary Surgery, the Affiliated Suzhou Hospital of Nanjing Medical University, Suzhou, China
| | - Xinwei Jiang
- Department of Hepatobiliary Surgery, the Affiliated Suzhou Hospital of Nanjing Medical University, Suzhou, China
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Nitsch A, Qarqash S, Römer S, Schoon J, Singer D, Bekeschus S, Ekkernkamp A, Wassilew GI, Tzvetkov MV, Haralambiev L. Effective combination of cold physical plasma and chemotherapy against Ewing sarcoma cells in vitro. Sci Rep 2024; 14:6505. [PMID: 38499701 PMCID: PMC10948386 DOI: 10.1038/s41598-024-56985-4] [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: 06/11/2023] [Accepted: 03/13/2024] [Indexed: 03/20/2024] Open
Abstract
Ewing's sarcoma (ES) is the second most common bone tumor in children and adolescents and is highly malignant. Although the new chemotherapy has significantly improved the survival rate for ES from about 10 to 75%, the survival rate for metastatic tumors remains around 30%. This treatment is often associated with various side effects that contribute to the suffering of the patients. Cold physical plasma (CPP), whether used alone or in combination with current chemotherapy, is considered a promising adjunctive tool in cancer treatment. This study aims to investigate the synergistic effects of CPP in combination with cytostatic chemotherapeutic agents that are not part of current ES therapy. Two different ES cell lines, RD-ES and A673, were treated with the determined IC20 concentrations of the chemotherapeutic agents cisplatin and methotrexate (MTX) in combination with CPP. The effects on population doubling, cell viability, and apoptotic processes within these cell lines were assessed. This combination therapy has led to a reduction of population doubling and cell viability, as well as an increase in apoptotic activity in cells compared to CPP monotherapy. The results of this study provide evidence that combining CPP with non-common chemotherapy drugs such as MTX and CIS in the treatment of ES enhances the anticancer effects of these drugs. These findings open up new possibilities for the effective use of these drugs against ES.
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Affiliation(s)
- Andreas Nitsch
- Center for Orthopedics, Trauma Surgery and Rehabilitation Medicine, University Medicine Greifswald, Ferdinand-Sauerbruch-Straße, 17475, Greifswald, Germany
| | - Sara Qarqash
- Center for Orthopedics, Trauma Surgery and Rehabilitation Medicine, University Medicine Greifswald, Ferdinand-Sauerbruch-Straße, 17475, Greifswald, Germany
| | - Sarah Römer
- Department of General Pharmacology, Institute of Pharmacology, Center of Drug Absorption and Transport (C_DAT), University Medicine Greifswald, Felix-Hausdorff-Straße 3, 17489, Greifswald, Germany
| | - Janosch Schoon
- Center for Orthopedics, Trauma Surgery and Rehabilitation Medicine, University Medicine Greifswald, Ferdinand-Sauerbruch-Straße, 17475, Greifswald, Germany
| | - Debora Singer
- Clinic and Polyclinic for Dermatology and Venerology, Strempelstr. 13, 18057, Rostock, Germany
- Leibniz Institute for Plasma Science and Technology (INP), ZIK Plasmatis, Felix-Hausdorff-Str. 2, 17489, Greifswald, Germany
| | - Sander Bekeschus
- Clinic and Polyclinic for Dermatology and Venerology, Strempelstr. 13, 18057, Rostock, Germany
- Leibniz Institute for Plasma Science and Technology (INP), ZIK Plasmatis, Felix-Hausdorff-Str. 2, 17489, Greifswald, Germany
| | - Axel Ekkernkamp
- Center for Orthopedics, Trauma Surgery and Rehabilitation Medicine, University Medicine Greifswald, Ferdinand-Sauerbruch-Straße, 17475, Greifswald, Germany
- Department of Trauma and Orthopaedic Surgery, BG Klinikum Unfallkrankenhaus Berlin, Warener Straße 7, 12683, Berlin, Germany
| | - Georgi I Wassilew
- Center for Orthopedics, Trauma Surgery and Rehabilitation Medicine, University Medicine Greifswald, Ferdinand-Sauerbruch-Straße, 17475, Greifswald, Germany
| | - Mladen V Tzvetkov
- Department of General Pharmacology, Institute of Pharmacology, Center of Drug Absorption and Transport (C_DAT), University Medicine Greifswald, Felix-Hausdorff-Straße 3, 17489, Greifswald, Germany
| | - Lyubomir Haralambiev
- Center for Orthopedics, Trauma Surgery and Rehabilitation Medicine, University Medicine Greifswald, Ferdinand-Sauerbruch-Straße, 17475, Greifswald, Germany.
- Department of Trauma and Orthopaedic Surgery, BG Klinikum Unfallkrankenhaus Berlin, Warener Straße 7, 12683, Berlin, Germany.
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Ghandour M, Semaan K, Saad E, Horsch A, Abdallah R, Semaan D. Clinicodemographic characteristics of extraosseous ewing sarcoma: A comparative meta-analysis of pediatric and adult patients. J Orthop 2023; 44:86-92. [PMID: 37731676 PMCID: PMC10507075 DOI: 10.1016/j.jor.2023.09.002] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/30/2023] [Accepted: 09/04/2023] [Indexed: 09/22/2023] Open
Abstract
Background Evidence suggests different presentation patterns and prognosis of extraosseous Ewing Sarcoma (EES) based on age. Thus, we carried out this study to test the difference between children and adult EES cases regarding clinicodemographic characteristics and prognosis. Methods A total of 4 databases were explored yielding 18 relevant studies for data synthesis. Outcomes included the comparison of demographic and clinical characteristics as well as prognosis between children and adults with EES. Log odds ratio (logOR) and its 95% confidence interval (CI) were pooled across studies. Statistical models/methods were selected based on heterogeneity. Results Our analysis included a total of 1261 children and 1256 adults. When we compared these two age categories, we did not observe a significant difference in the risk of developing EES [logOR = -0.13; 95% CI: -0.65: 0.39; I2 = 88.42%]. No significant differences regarding gender, tumor location, and size (≤5 vs. >5 cm), EWSR1 positivity, or management modality. We did not observe significant difference regarding clinical outcomes, such as 5-year overall survival and event-free survival, recurrence, mortality, no evidence of disease, and secondary metastasis. Conclusions Our findings highlight the absence of an association between the age category of patients and the incidence of EES, as well as its clinical outcomes.
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Affiliation(s)
- Maher Ghandour
- Orthopedics Department, Heidelberg University Hospital, Germany
- Orthopedics Department, Lebanese University, Lebanon
| | - Karl Semaan
- Medicine, Université Saint Joseph, Beirut, Lebanon
| | - Eddy Saad
- Medicine, Université Saint Joseph, Beirut, Lebanon
| | - Axel Horsch
- Orthopedics Department, Heidelberg University Hospital, Germany
| | | | - Doumit Semaan
- Orthopedics Department, Lebanese University, Lebanon
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Dupuy M, Lamoureux F, Mullard M, Postec A, Regnier L, Baud’huin M, Georges S, Brounais-Le Royer B, Ory B, Rédini F, Verrecchia F. Ewing sarcoma from molecular biology to the clinic. Front Cell Dev Biol 2023; 11:1248753. [PMID: 37752913 PMCID: PMC10518617 DOI: 10.3389/fcell.2023.1248753] [Citation(s) in RCA: 8] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/27/2023] [Accepted: 08/30/2023] [Indexed: 09/28/2023] Open
Abstract
In Europe, with an incidence of 7.5 cases per million, Ewing sarcoma (ES) is the second most common primary malignant bone tumor in children, adolescents and young adults, after osteosarcoma. Since the 1980s, conventional treatment has been based on the use of neoadjuvant and adjuvant chemotherapeutic agents combined with surgical resection of the tumor when possible. These treatments have increased the patient survival rate to 70% for localized forms, which drops drastically to less than 30% when patients are resistant to chemotherapy or when pulmonary metastases are present at diagnosis. However, the lack of improvement in these survival rates over the last decades points to the urgent need for new therapies. Genetically, ES is characterized by a chromosomal translocation between a member of the FET family and a member of the ETS family. In 85% of cases, the chromosomal translocation found is (11; 22) (q24; q12), between the EWS RNA-binding protein and the FLI1 transcription factor, leading to the EWS-FLI1 fusion protein. This chimeric protein acts as an oncogenic factor playing a crucial role in the development of ES. This review provides a non-exhaustive overview of ES from a clinical and biological point of view, describing its main clinical, cellular and molecular aspects.
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Affiliation(s)
- Maryne Dupuy
- Nantes Université, Inserm UMR 1307, CNRS UMR 6075, CRCI2NA, Université d'Angers, Nantes, France
| | | | | | | | | | | | | | | | | | | | - Franck Verrecchia
- Nantes Université, Inserm UMR 1307, CNRS UMR 6075, CRCI2NA, Université d'Angers, Nantes, France
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El Harras Y, Choayb S, Laasri K, Allali N, Chat L, El Haddad S. Extra-skeletal Ewing's sarcoma of the leg with multiple skeletal and pulmonary metastases: A rare pediatric case report. SAGE Open Med Case Rep 2023; 11:2050313X231194815. [PMID: 37654555 PMCID: PMC10467293 DOI: 10.1177/2050313x231194815] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/22/2023] [Accepted: 07/28/2023] [Indexed: 09/02/2023] Open
Abstract
Being the second most common malignant bone tumor in children and young adults, Ewing's sarcoma can also occur as a primary soft-tissue tumor called extraosseous or extra-skeletal Ewing's sarcoma. It is a rare entity, especially in the pediatric population. We report the case of an adolescent who presented to our department for lower extremity magnetic resonance imaging to explore leg swelling. It revealed an extra-skeletal Ewing's sarcoma with multiple bone metastases. By reporting this case, we also review the literature on this rare abnormality.
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Affiliation(s)
- Yahya El Harras
- Pediatric and Gynecology Radiology Department, Children’s Hospital, University Mohammed V, Rabat, Morroco
| | - Safaa Choayb
- Pediatric and Gynecology Radiology Department, Children’s Hospital, University Mohammed V, Rabat, Morroco
| | - Khadija Laasri
- Pediatric and Gynecology Radiology Department, Children’s Hospital, University Mohammed V, Rabat, Morroco
| | - Nazik Allali
- Pediatric and Gynecology Radiology Department, Children’s Hospital, University Mohammed V, Rabat, Morroco
| | - Latifa Chat
- Pediatric and Gynecology Radiology Department, Children’s Hospital, University Mohammed V, Rabat, Morroco
| | - Siham El Haddad
- Pediatric and Gynecology Radiology Department, Children’s Hospital, University Mohammed V, Rabat, Morroco
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11
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Ganguly S, Sasi A, Khan SA, Kumar VS, Kapoor L, Sharma MC, Mridha A, Barwad A, Thulkar S, Pushpam D, Bakhshi S. Formulation and validation of a baseline prognostic score for osteosarcoma treated uniformly with a non-high dose methotrexate-based protocol from a low middle income healthcare setting: a single centre analysis of 594 patients. Front Oncol 2023; 13:1148480. [PMID: 37188186 PMCID: PMC10175811 DOI: 10.3389/fonc.2023.1148480] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/20/2023] [Accepted: 04/05/2023] [Indexed: 05/17/2023] Open
Abstract
INTRODUCTION The outcomes of osteosarcoma in low middle income countries (LMICs) are different due to patients presenting in advanced stages, resource constraints and the use of non-high-dose-methotrexate (HDMTX)-based regimens. This study derived and validated a prognostic score for osteosarcoma that integrates biologic and social factors and is tailored for patients from an LMIC setting using a non-HDMTX-based protocol. MATERIALS AND METHODS A retrospective study including osteosarcoma patients enrolled for treatment at a single tertiary care centre in India between 2003-19 was conducted. Baseline biologic and social characteristics were extracted from medical records and survival outcomes were noted. The cohort was randomised into a derivation and validation cohort. Multivariable Cox regression was used to identify baseline characteristics that were independently prognostic for survival outcomes in the derivation cohort. A score was derived from the prognostic factors identified in the derivation cohort and further validated in the validation cohort with estimation of its predictive ability. RESULTS 594 patients with osteosarcoma were eligible for inclusion in the study. Around one-third of the cohort had metastatic disease with 59% of the patients residing in rural areas. The presence of metastases at baseline (HR 3.39; p<0.001; score=3), elevated serum alkaline phosphatase (SAP) >450 IU/L (HR 1.57; p=0.001; score=1) and baseline tumour size > 10 cm (HR 1.68; p<0.001; score=1) were identified to be independent factors predicting inferior event free survival (EFS) and were included in development of the prognostic score. Patients were categorized as low risk (score 0), intermediate risk (score 1-3) and high risk (4-5). Harrell's c-indices for the score were 0.682, 0.608 and 0.657 respectively for EFS in the derivation, validation and whole cohort respectively. The timed AUC of ROC was 0.67 for predicting 18-month EFS in the derivation, validation and whole cohorts while that for 36-month EFS were 0.68, 0.66 and 0.68 respectively. CONCLUSIONS The study describes the outcomes among osteosarcoma patients from an LMIC treated uniformly with a non-HDMTX-based protocol. Tumor size, baseline metastases and SAP were prognostic factors used to derive a score with good predictive value for survival outcomes. Social factors did not emerge as determinants of survival.
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Affiliation(s)
- Shuvadeep Ganguly
- Department of Medical Oncology, Dr. B.R.A. Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India
| | - Archana Sasi
- Department of Medical Oncology, Dr. B.R.A. Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India
| | - Shah Alam Khan
- Department of Orthopaedics, All India Institute of Medical Sciences, New Delhi, India
| | | | - Love Kapoor
- Department of Orthopaedics, All India Institute of Medical Sciences, New Delhi, India
| | - Mehar Chand Sharma
- Department of Pathology, All India Institute of Medical Sciences, New Delhi, India
| | - Asit Mridha
- Department of Pathology, All India Institute of Medical Sciences, New Delhi, India
| | - Adarsh Barwad
- Department of Pathology, All India Institute of Medical Sciences, New Delhi, India
| | - Sanjay Thulkar
- Department of Radiodiagnosis, Dr. B.R.A. Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India
| | - Deepam Pushpam
- Department of Medical Oncology, Dr. B.R.A. Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India
| | - Sameer Bakhshi
- Department of Medical Oncology, Dr. B.R.A. Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India
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12
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Chowdhary RL, Chufal KS, Pahuja AK, Ahmad I, Sharma M, Jwala M, Kumar L, Sharma A. An institutional review of treatment outcomes in extraosseous Ewing's sarcoma- the largest Asian experience. Cancer Radiother 2023; 27:50-56. [PMID: 35973914 DOI: 10.1016/j.canrad.2022.06.008] [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: 09/18/2021] [Revised: 05/07/2022] [Accepted: 06/01/2022] [Indexed: 02/06/2023]
Abstract
PURPOSE To analyze the treatment strategies, outcomes and factors impacting these outcomes in extraosseous ewings sarcoma (EOES). MATERIAL AND METHODS A search of the hospital database yielded a total of 109 EOES patients registered in last 10 years out of which 25 patients were excluded from analysis due to incomplete medical records. Demographic and clinical characteristics were reported using descriptive statistics. Overall survival (OS) was taken from the time of diagnosis to death. Patients who were alive or lost to follow up were censored from the survival analysis. A total of 12 clinical and treatment related variables were taken into univariate analysis and those showing significance or a trend towards significance were taken up for multivariate analysis. Further a cluster analysis was done in a quest to find a subgroup which would have a better survival outcome as compared to other clusters. SPSS version 23 was used for statistical analysis. RESULTS Chest wall (n=26), lower extremity (n=22) and paraspinal area (n=14) were the common sites involved. Localised swelling (n=43) was the most common presenting symptom and the median time to presentation was 2 months. Overall survival of the entire cohort at 5 years was 52 percent. Stage at presentation had a significant impact (P value<0.001) on estimated median OS (localised 70 months versus 36 months in metastatic stage). Cluster analysis showed that, patients with localised stage at presentation, good response to chemotherapy, negative resection margin and no adjuvant RT had a median survival of 69 months. CONCLUSION Judicious use of trimodality treatment in EOES yields optimal results and it also adds significantly onto the scarce literature on this subtype of sarcoma.
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Affiliation(s)
- R L Chowdhary
- Department of Radiation Oncology, Rajiv Gandhi Cancer Institute and Research Center, New Delhi, India.
| | - K S Chufal
- Department of Radiation Oncology, Rajiv Gandhi Cancer Institute and Research Center, New Delhi, India
| | - A K Pahuja
- Department of Radiation Oncology, Rajiv Gandhi Cancer Institute and Research Center, New Delhi, India
| | - I Ahmad
- Department of Radiation Oncology, Rajiv Gandhi Cancer Institute and Research Center, New Delhi, India
| | - M Sharma
- Department of Radiation Oncology, Rajiv Gandhi Cancer Institute and Research Center, New Delhi, India
| | - M Jwala
- Department of Radiation Oncology, Rajiv Gandhi Cancer Institute and Research Center, New Delhi, India
| | - L Kumar
- Department of Radiation Oncology, Rajiv Gandhi Cancer Institute and Research Center, New Delhi, India
| | - A Sharma
- Department of biostatistics, Rajiv Gandhi Cancer Institute and Research Center, New Delhi, India
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13
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Mathew J, Arjunan R, Dasappa A, Namachivayam A. Prognostic Factors and Clinical Outcomes in Extraskeletal Ewing Sarcoma: A Cohort Study. Ann Surg Oncol 2022; 30:3084-3094. [PMID: 36564656 DOI: 10.1245/s10434-022-12992-1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/15/2022] [Accepted: 12/07/2022] [Indexed: 12/24/2022]
Abstract
BACKGROUND Extraskeletal Ewing sarcoma (ESE) is a lesser-known, rarer counterpart of Ewing sarcoma of bone. This single-center study sought to evaluate the prognosticators and outcomes following multimodality therapy in patients with ESE. METHODS Forty-seven patients with ESE, treated between 2013 and 2018 with a standardized protocol and multimodality therapy using established doxorubicin-based regimens, were followed-up to assess outcomes. RESULTS Median age at diagnosis was 20 (range 7-56) years, and 57.4% were male. Median tumor size was 7 (range 2-21) cm. The symptom-duration ranged from 1 to 8 (median 4) months. Tumor-site was trunkal in 61.7%, extremity in 23.4%, and head and neck 14.9%. Of the 35 patients with nonmetastatic disease at presentation, 13 underwent upfront surgery. The rest received chemotherapy followed by local treatment, which was surgical in 15 and radiotherapy in 5. At median follow-up of 24 (range 5-98) months, 55.3% patients had experienced events, and 29.8% had died of progressive disease. Three-year event-free survival was 41.1%, and overall survival was 53%. On univariate analysis, trunkal location, upfront surgery, and positive surgical margins were associated with inferior EFS. Trunkal tumors and upfront surgery were also associated with poorer OS. On multivariate analysis, trunkal location and margin-positive resections retained statistical significance for adverse EFS. CONCLUSIONS Unless clearly resectable upfront, ESE should be downstaged with chemotherapy before local treatment. A margin-negative resection should be the objective when performing surgery. Definitive radiotherapy is an alternative in tumors not amenable for complete excision or when anticipated postoperative morbidity precludes radical surgery.
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Affiliation(s)
- Joseph Mathew
- Department of Surgical Oncology, Kidwai Memorial Institute of Oncology, Bangalore, India.
| | - Ravi Arjunan
- Department of Surgical Oncology, Kidwai Memorial Institute of Oncology, Bangalore, India
| | - Ashwathappa Dasappa
- Department of Surgical Oncology, Kidwai Memorial Institute of Oncology, Bangalore, India
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14
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Ghandour M, Lehner B, Klotz M, Geisbüsch A, Bollmann J, Renkawitz T, Horsch A. Extraosseous Ewing Sarcoma in Children: A Systematic Review and Meta-Analysis of Clinicodemographic Characteristics. CHILDREN (BASEL, SWITZERLAND) 2022; 9:children9121859. [PMID: 36553303 PMCID: PMC9776445 DOI: 10.3390/children9121859] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Subscribe] [Scholar Register] [Received: 10/17/2022] [Revised: 11/22/2022] [Accepted: 11/26/2022] [Indexed: 12/04/2022]
Abstract
Background: We conducted this systematic review to provide comprehensive evidence on the prevalence, clinical features and outcomes of young extraosseous Ewing sarcoma (EES) cases. Methods: PubMed, Scopus, Web of Science, and Google Scholar were searched for articles reporting the occurrence of EES among children and adolescents (<21 years). The primary outcome included the rate of occurrence of EES among children and adolescents, while the secondary outcomes included the descriptive analyses of the demographic characteristics, tumor characteristics, and clinical outcomes of the affected cases. The data are reported as the effect size (ES) and its corresponding 95% confidence interval (CI). Results: A total of 29 studies were included. Twenty-four reported instances of childhood disease among all the EES cases [ES = 30%; 95%CI: 29−31%], while five studies reported extraosseous cases among the pediatric EES cases [ES = 22%; 95%CI: 13−31%]. The thorax is the most common location of childhood EES [33%; 95%CI: 20−46%] followed by the extremities [31%; 95%CI: 22−40%]. Concurrent chemotherapy and radiotherapy [57%; 95%CI: 25−84%] was the most commonly implemented management protocol in the pediatric EES cases. The rate of no evidence of disease and 5-year overall survival was 69% for both outcomes. Mortality occurred in 29% of cases, while recurrence and secondary metastasis occurred in 35% and 16% of cases, respectively. Conclusions: Our findings provide insight into the clinical features and outcomes of EES among children and adolescents.
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Affiliation(s)
- Maher Ghandour
- Department of Orthopedics, Heidelberg University Hospital, 69129 Heidelberg, Germany
| | - Burkhard Lehner
- Department of Orthopedics, Heidelberg University Hospital, 69129 Heidelberg, Germany
| | - Matthias Klotz
- Orthopedics and Trauma Surgery, Marienkrankenhaus Soest, 59494 Soest, Germany
| | - Andreas Geisbüsch
- Department of Orthopedics, Heidelberg University Hospital, 69129 Heidelberg, Germany
| | - Jakob Bollmann
- Department of Orthopedics, Heidelberg University Hospital, 69129 Heidelberg, Germany
| | - Tobias Renkawitz
- Department of Orthopedics, Heidelberg University Hospital, 69129 Heidelberg, Germany
| | - Axel Horsch
- Department of Orthopedics, Heidelberg University Hospital, 69129 Heidelberg, Germany
- Correspondence:
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15
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C-Reactive Protein Pretreatment-Level Evaluation for Ewing's Sarcoma Prognosis Assessment-A 15-Year Retrospective Single-Centre Study. Cancers (Basel) 2022; 14:cancers14235898. [PMID: 36497377 PMCID: PMC9735882 DOI: 10.3390/cancers14235898] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/01/2022] [Revised: 11/26/2022] [Accepted: 11/27/2022] [Indexed: 12/03/2022] Open
Abstract
Background: A pathological/inflamed cellular microenvironment state is an additional risk factor for any cancer type. The importance of a chronic inflammation state in most diffuse types of tumour has already been analysed, except for in Ewing’s sarcoma. It is a highly malignant blue round cell tumour, with 90% of cases occurring in patients aged between 5 and 25 years. Worldwide, 2.9 out of 1,000,000 children per year are affected by this malignancy. The aim of this retrospective study was to analyse the role of C-reactive protein (CRP) as a prognostic factor for Ewing’s sarcomas. Methods: This retrospective study at Klinikum rechts der Isar included 82 patients with a confirmed Ewing’s sarcoma diagnosis treated between 2004 and 2019. Preoperative CRP determination was assessed in mg/dL with a normal value established as below 0.5 mg/dL. Disease-free survival time was calculated as the time between the initial diagnosis and an event such as local recurrence or metastasis. Follow-up status was described as death of disease (DOD), no evidence of disease (NED) or alive with disease (AWD). The exclusion criteria of this study included insufficient laboratory values and a lack of information regarding the follow-up status or non-oncological resection. Results: Serum CRP levels were significantly different in patients with a poorer prognosis (DOD) and in patients who presented distant metastasis (p = 0.0016 and p = 0.009, respectively), whereas CRP levels were not significantly different in patients with local recurrence (p = 0.02). The optimal breakpoint that predicted prognosis was 0.5 mg/dL, with a sensitivity of 0.76 and a specificity of 0.74 (AUC 0.81). Univariate CRP analysis level >0.5 mg/dL revealed a hazard ratio of 9.5 (95% CI 3.5−25.5). Conclusions: In Ewing’s sarcoma cases, we consider a CRP pretreatment value >0.5 mg/dL as a sensitive prognostic risk factor indication for distant metastasis and poor prognosis. Further research with more data is required to determine more sensitive cutoff levels.
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16
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Nasri S, Aqamour R, Haddar L, Guerrouj I, Haddar K, Aichouni N, Kamaoui I, Skiker I. Extraskeletal Ewing sarcoma: A case report. Radiol Case Rep 2022; 17:4809-4813. [PMID: 36238207 PMCID: PMC9550844 DOI: 10.1016/j.radcr.2022.09.048] [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: 08/17/2022] [Revised: 09/07/2022] [Accepted: 09/13/2022] [Indexed: 11/18/2022] Open
Abstract
Extraskeletal Ewing sarcoma is a rare tumor mainly affecting young people, of poor prognosis with very high mortality rates especially in metastatic forms. It can affect different locations, without specific clinical signs, which delays the diagnosis. Imaging plays an important role for diagnosis, staging, preoperative assessment and surveillance. The diagnosis should be set early to a better management. We report a case of a 30-year-old man with a large extraskeletal Ewing sarcoma of the left thigh. The patient was initially treated with chemotherapy. Unfortunately, the tumor has increased in size making surgery impossible. The patient ultimately died of pulmonary metastases.
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17
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The Prognostic Role of the C-Reactive Protein and Serum Lactate Dehydrogenase in a Pediatric Series of Bone Ewing Sarcoma. Cancers (Basel) 2022; 14:cancers14133064. [PMID: 35804835 PMCID: PMC9264769 DOI: 10.3390/cancers14133064] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/09/2022] [Revised: 06/16/2022] [Accepted: 06/20/2022] [Indexed: 12/22/2022] Open
Abstract
Simple Summary Ewing sarcoma is a rare and aggressive tumor of childhood and adolescence. Over the years, different prognostic factors have been explored to stratify high-risk patients. The roles of C-reactive protein (CRP) and serum lactate dehydrogenase (LDH) as potential new prognostic factors would be a useful and simple for risk stratification, but they have rarely been investigated. In our work, we analyze the role of LDH and CRP as prognostic factors in a population of pediatric and adolescent patients affected by Ewing sarcoma. Our study confirms the potential prognostic role of LDH at diagnosis as an independent prognostic factor. LDH evaluation is not expensive, and it can be beneficial for developing countries where diagnostic and staging resources in the pediatric oncology field are poor. Abstract Background: Ewing sarcoma (ES) is a rare and aggressive pediatric cancer. Numerous studies have attempted to identify new prognostic biomarkers. The predictive value of serum LDH and CRP has not been clearly described, to date. Methods: The objective of our retrospective study was to investigate the prognostic value of LDH and CRP levels and their association with overall survival in a series of ES patients. Results: Between 2004 and 2019, 89 ES patients were included. In a univariable analysis, high levels of LDH and CRP were associated with the worst prognosis. In a multivariable analysis, only higher LDH values remained associated with a lower survival. The high-LDH-level group experienced all 21 deaths registered in our population (24%) and about 90% of disease progressions. The 5-year overall survival was 66.4% in the high-LDH-level group, while no deaths were observed in the low-LDH-level group. The 5-year progression-free survival was 57.9% in the high-LDH-level group versus 80.4% in the low-LDH-level group. Conclusions: In our study, LDH levels at diagnosis were strongly correlated with the prognosis, and they might be considered a prognostic factor in Ewing sarcoma. The LDH value, along with its very low cost and its reproducibility in almost all centers, make it suitable as a potential prognostic biomarker in clinical practice.
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18
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Li W, Xu C, Hu Z, Dong S, Wang H, Liu Q, Tang ZR, Li W, Wang B, Lei Z, Yin C. A Visualized Dynamic Prediction Model for Lymphatic Metastasis in Ewing's Sarcoma for Smart Medical Services. Front Public Health 2022; 10:877736. [PMID: 35602163 PMCID: PMC9114797 DOI: 10.3389/fpubh.2022.877736] [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: 02/17/2022] [Accepted: 03/28/2022] [Indexed: 11/30/2022] Open
Abstract
Background This study aims to predict the lymphatic metastasis in Ewing's sarcoma (ES) patients by nomogram. The risk of lymphatic metastasis in patients with ES was predicted by the built model, which provided guidance for the clinical diagnosis and treatment planning. Methods A total of 929 patients diagnosed with ES were enrolled from the year of 2010 to 2016 in the Surveillance, Epidemiology, and End Results (SEER) database. The nomogram was established to determine predictive factors of lymphatic metastasis according to univariate and multivariate logistic regression analysis. The validation of the model performed using multicenter data (n = 51). Receiver operating characteristics (ROC) curves and calibration plots were used to evaluate the prediction accuracy of the nomogram. Decision curve analysis (DCA) was implemented to illustrate the practicability of the nomogram clinical application. Based on the nomogram, we established a web calculator to visualize the risk of lymphatic metastases. We further plotted Kaplan-Meier overall survival (OS) curves to compare the survival time of patients with and without lymphatic metastasis. Results In this study, the nomogram was established based on six significant factors (survival time, race, T stage, M stage, surgery, and lung metastasis), which were identified for lymphatic metastasis in ES patients. The model showed significant diagnostic accuracy with the value of the area under the curve (AUC) was 0.743 (95%CI: 0.714–0.771) for SEER internal validation and 0.763 (95%CI: 0.623–0.871) for multicenter data external validation. The calibration plot and DCA indicated that the model had vital clinical application value. Conclusion In this study, we constructed and developed a nomogram with risk factors to predict lymphatic metastasis in ES patients and validated accuracy of itself. We found T stage (Tx OR = 2.540, 95%CI = 1.433–4.503, P < 0.01), M stage (M1, OR = 2.061, 95%CI = 1.189–3.573, P < 0.05) and survival time (OR = 0.982, 95%CI = 0.972–0.992, P < 0.001) were important independent factors for lymphatic metastasis in ES patients. Furthermore, survival time in patients with lymphatic metastasis or unclear situation (P < 0.0001) was significantly lower. It can help clinicians make better decisions to provide more accurate prognosis and treatment for ES patients.
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Affiliation(s)
- Wenle Li
- Department of Orthopedics, Xianyang Central Hospital, Xianyang, China.,Clinical Medical Research Center, Xianyang Central Hospital, Xianyang, China
| | - Chan Xu
- Clinical Medical Research Center, Xianyang Central Hospital, Xianyang, China
| | - Zhaohui Hu
- Department of Spinal Surgery, Liuzhou People's Hospital, Liuzhou, China
| | - Shengtao Dong
- Department of Spine Surgery, Second Affiliated Hospital of Dalian Medical University, Dalian, China
| | - Haosheng Wang
- Department of Orthopaedics, The Second Hospital of Jilin University, Changchun, China
| | - Qiang Liu
- Department of Orthopedics, Xianyang Central Hospital, Xianyang, China
| | - Zhi-Ri Tang
- School of Physics and Technology, Wuhan University, Wuhan, China
| | - Wanying Li
- Clinical Medical Research Center, Xianyang Central Hospital, Xianyang, China
| | - Bing Wang
- Clinical Medical Research Center, Xianyang Central Hospital, Xianyang, China
| | - Zhi Lei
- Chronic Disease Division, Luzhou Center for Disease Control and Prevention, Luzhou, China.,Faculty of Medicine, Macau University of Science and Technology, Taipa, Macau SAR, China
| | - Chengliang Yin
- Faculty of Medicine, Macau University of Science and Technology, Taipa, Macau SAR, China
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19
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A case report of retroperitoneal Ewing sarcoma requiring adrenalectomy. Int J Surg Case Rep 2022; 95:106966. [PMID: 35597126 PMCID: PMC9127152 DOI: 10.1016/j.ijscr.2022.106966] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/06/2021] [Revised: 03/24/2022] [Accepted: 03/25/2022] [Indexed: 12/02/2022] Open
Abstract
Introduction and importance Ewing's sarcoma is a rare entity of malignant in both skeletal and extra-skeletal sites. There are few patients reported as Extra-Skeletal Sarcoma, and fewer reported cases for Extraskeletal Ewing sarcoma involving the retroperitoneal region. Reporting such a rare entity will add to literature in helping the diagnosis and management of such cases. Case presentation We present a case of 26 year old previously healthy female complaining of a vague abdominal pain and discovered to have retroperitoneal Ewing sarcoma adherent to left adrenal gland. Clinical discussion She was managed with a combination of neoadjuvant chemotherapy and resection through exploratory laparotomy. Conclusion In short, Ewing sarcoma should be considered in the differential diagnosis for young patient who is presenting with a retroperitoneal mass.
Ewing's sarcoma is a rare entity of malignant in both skeletal and extra-skeletal sites. There are few patients reported as Extra-Skeletal Sarcoma (ESS). EES involving retroperitoneal region, from previous studies. We present a case of EES involving the retroperitoneal region and its subsequent management.
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20
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MC SB, Choudhary A, A. S, Jacob LA, KN L, AH R, LK R, Saldanha S, G. C. Ewing Sarcoma of Phalanx—A Common Tumor with an Uncommon Presentation. Indian J Med Paediatr Oncol 2022. [DOI: 10.1055/s-0042-1742443] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/18/2022] Open
Affiliation(s)
- Suresh Babu MC
- Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
| | - Akansha Choudhary
- Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
| | - Sreevalli A.
- Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
| | - Linu Abraham Jacob
- Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
| | - Lokesh KN
- Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
| | - Rudresha AH
- Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
| | - Rajeev LK
- Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
| | - Smitha Saldanha
- Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
| | - Champaka G.
- Department of Pathology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
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21
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Wang JG, Wei QF, Tang QM. Regarding the Children's Oncology Group AEWS1031 Trial for Ewing Sarcoma. J Clin Oncol 2022; 40:1506. [PMID: 35235371 DOI: 10.1200/jco.21.02546] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022] Open
Affiliation(s)
- Ji-Gan Wang
- Ji-Gan Wang, MD, Qiu-Fen Wei, MD, and Qu-Mei Tang, MD, Pediatrics Department, Maternal and Child Health Hospital of Guangxi Zhuang Autonomous Region, Nanning, Guangxi, China; Guangxi Clinical Research Center for Pediatric Diseases, Guangxi, China
| | - Qiu-Fen Wei
- Ji-Gan Wang, MD, Qiu-Fen Wei, MD, and Qu-Mei Tang, MD, Pediatrics Department, Maternal and Child Health Hospital of Guangxi Zhuang Autonomous Region, Nanning, Guangxi, China; Guangxi Clinical Research Center for Pediatric Diseases, Guangxi, China
| | - Qu-Mei Tang
- Ji-Gan Wang, MD, Qiu-Fen Wei, MD, and Qu-Mei Tang, MD, Pediatrics Department, Maternal and Child Health Hospital of Guangxi Zhuang Autonomous Region, Nanning, Guangxi, China; Guangxi Clinical Research Center for Pediatric Diseases, Guangxi, China
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22
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Pankratjevaite L, Eskandarani HA, Lizdenis P, Saladzinskas Z. Challenges in diagnosing an extraosseous Ewing sarcoma: A case report. Int J Surg Case Rep 2022; 91:106708. [PMID: 35030406 PMCID: PMC8760346 DOI: 10.1016/j.ijscr.2021.106708] [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: 10/26/2021] [Revised: 12/09/2021] [Accepted: 12/14/2021] [Indexed: 10/30/2022] Open
Abstract
INTRODUCTION Ewing sarcoma is a highly malignant and rare tumour of bones and soft tissue. It may occur at any age, but it is more common in children and teenagers. CASE REPORT We report a case of a 56-year-old woman with EES involving the right iliac fossa. Previous abdominal trauma with retroperitoneal hematoma, nonspecific symptoms and unusual age for EES have caused diagnostics difficulties. The first histopathological examination misdiagnosed tumour to be a GIST, and just after the second surgery the accurate diagnosis of EES was made. CONCLUSIONS The diagnosis of ES sometimes is complicated and delayed. Prompt detailed examination and imaging studies should be performed to people with long lasting pain without trauma and other nonspecific symptoms, especially followed by a palpable mass. The treatment of EES is multimodal.
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Affiliation(s)
- Lina Pankratjevaite
- Department of Breast Surgery, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark; Department of Breast Surgery, Herlev and Gentofte Hospital, Copenhagen University Hospital, Denmark.
| | - Hassan Ali Eskandarani
- Department of Plastic Surgery, Herlev and Gentofte Hospital, Copenhagen University Hospital, Denmark
| | - Paulius Lizdenis
- Department of Surgery, Medical Academy, Hospital of Lithuanian University of Health Sciences, Kaunas, Lithuania
| | - Zilvinas Saladzinskas
- Department of Surgery, Medical Academy, Hospital of Lithuanian University of Health Sciences, Kaunas, Lithuania
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23
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Margol A, Abongwa C, Dhall G. Primary Primitive Neuroectodermal Tumor of the Spine With t(11;22): Report of 3 Cases and Review of Literature. J Pediatr Hematol Oncol 2021; 43:e983-e986. [PMID: 33480656 DOI: 10.1097/mph.0000000000002063] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/04/2020] [Accepted: 12/10/2020] [Indexed: 11/26/2022]
Abstract
Intradural extramedullary peripheral primitive neuroectodermal tumor (pPNET) with t(11;22) is a rare clinical finding in the pediatric population with few published cases in the literature. The authors report 3 cases of intradural primary pPNET and discuss the clinical presentation, treatment, and survival of the patients. Clinicians should be vigilant in considering pPNET in the differential diagnosis of extradural masses. The authors also compare the clinical course and outcome of therapy with primary PNET of the central nervous system and Ewing sarcoma family of tumors. In addition, this report highlights the risk for leptomeningeal dissemination at recurrence and discusses the importance of central nervous system-targeted therapy for durable disease control.
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MESH Headings
- Adolescent
- Adult
- Bone Neoplasms/diagnosis
- Bone Neoplasms/genetics
- Chromosomes, Human, Pair 11/genetics
- Chromosomes, Human, Pair 22/genetics
- Diagnosis, Differential
- Female
- Humans
- Male
- Neuroectodermal Tumors, Primitive/diagnosis
- Neuroectodermal Tumors, Primitive/genetics
- Prognosis
- Sarcoma, Ewing/diagnosis
- Sarcoma, Ewing/genetics
- Spinal Neoplasms/diagnosis
- Spinal Neoplasms/genetics
- Translocation, Genetic
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Affiliation(s)
- Ashley Margol
- Division of Pediatric Oncology, Children's Hospital of Orange County, Orange
| | - Chenue Abongwa
- Division of Pediatric Oncology, Children's Hospital of Orange County, Orange
- Division of Hematology/Oncology and Bone Marrow Transplantation, Children's Hospital of Los Angeles, Los Angeles, CA
| | - Girish Dhall
- Division of Pediatric Oncology, Children's Hospital of Orange County, Orange
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24
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Koscielniak E, Sparber-Sauer M, Scheer M, Vokuhl C, Kazanowska B, Ladenstein R, Niggli F, Ljungman G, Paulussen M, Bielack SS, Seitz G, Fuchs J, Hallmen E, Klingebiel T, On Behalf Of The Cws Study Group. Extraskeletal Ewing sarcoma in children, adolescents, and young adults. An analysis of three prospective studies of the Cooperative Weichteilsarkomstudiengruppe (CWS). Pediatr Blood Cancer 2021; 68:e29145. [PMID: 34089219 DOI: 10.1002/pbc.29145] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/02/2020] [Revised: 04/22/2021] [Accepted: 05/09/2021] [Indexed: 11/11/2022]
Abstract
BACKGROUND We have analyzed the outcome of patients with localized extraskeletal Ewing sarcoma (EES) treated in three consecutive Cooperative Weichteilsarkomstudiengruppe (CWS) soft tissue sarcoma (STS) studies: CWS-91, CWS-96, and CWS-2002P. METHODS Patients were treated in CWS-91 with four- (vincristine, dactinomycin, doxorubicin, and ifosfamide [VAIA] or cyclophosphamide [VACA II]) or five-drug (+etoposide [EVAIA]) cycles, in CWS-96 they were randomly assigned to receive VAIA or CEVAIE (+carboplatin and etoposide), and in CWS-2002P with VAIA III plus optional maintenance therapy (MT) with cyclophosphamide and vinblastine. Local therapy consisted of resection and/or radiotherapy (RT). RESULTS Two hundred forty-three patients fulfilled the eligibility criteria. The 5-year event-free survival (EFS) and overall survival (OS) were 63% (95% confidence interval [CI] 57-69) and 73% (95% CI 67-79), respectively. The 5-year EFS by study was 64% (95% CI 54-74) in CWS-91, 57% (95% CI 48-66) in CWS-96, and 79% (95% CI 67-91) in CWS-2002P (n.s.). The 5-year OS was 72% (95% CI 62-82) in CWS-91, 70% (95% CI 61-79) in CWS-96, and 86% (95% CI 76-96) in CWS-2002P (n.s.). In CWS-96, 5-year EFS and OS in the VAIA arm versus the CEVAIE were 65% (95% CI 52-81) versus 55% (95% CI 39-76) log-rank p = .13, and 85% (95% CI 75-96) versus 61% (95% CI 45-82), log-rank p = .09. CONCLUSION Our analysis provides interesting information on the treatment and specificities of EES, which can be useful for a better understanding of this rare entity and should be considered in the development of future clinical trials for Ewing sarcoma defined as FET-ETS fusion positive tumors.
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Affiliation(s)
- Ewa Koscielniak
- Klinikum Stuttgart-Olgahospital, Pediatrics 5 (Oncology, Hematology, Immunology), Stuttgart, Germany.,University of Tuebingen, Tuebingen, Germany
| | - Monika Sparber-Sauer
- Klinikum Stuttgart-Olgahospital, Pediatrics 5 (Oncology, Hematology, Immunology), Stuttgart, Germany
| | - Monika Scheer
- Department of Pediatric Oncology/Hematology, Charité-Universitätsmedizin, Berlin, Germany
| | - Christian Vokuhl
- Institute of Children's Pathology, University of Bonn, Bonn, Germany
| | - Bernarda Kazanowska
- Department of Pediatric Hematology/Oncology and BMT, University of Wroclaw, Wroclaw, Poland
| | | | - Felix Niggli
- Department of Pediatric Oncology, University of Zürich, Zurich, Switzerland
| | - Gustaf Ljungman
- Department of Women's and Children's Health, Uppsala University, Uppsala, Sweden
| | | | - Stefan S Bielack
- Klinikum Stuttgart-Olgahospital, Pediatrics 5 (Oncology, Hematology, Immunology), Stuttgart, Germany
| | - Guido Seitz
- Department of Pediatric Surgery, University Children's Hospital Marburg, Marburg, Germany
| | - Joerg Fuchs
- Department of Pediatric Hematology and Oncology, Hospital for Children and Adolescents, University of Tuebingen, Tuebingen, Germany
| | - Erika Hallmen
- Klinikum Stuttgart-Olgahospital, Pediatrics 5 (Oncology, Hematology, Immunology), Stuttgart, Germany
| | - Thomas Klingebiel
- Department for Children and Adolescents, University Hospital, Goethe University, Frankfurt, Germany
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Primary Extraosseous Ewing Sarcoma of the Upper Limb: Report of a Rare Case. INTERNATIONAL JOURNAL OF CANCER MANAGEMENT 2021. [DOI: 10.5812/ijcm.113387] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Abstract
Introduction: Extraosseous Ewing sarcomas (EESs) are rare tumors that originate from soft tissues. Upper extremity EESs account for about 3% of all cases. Here we reported a case of ESS of the upper limb whose management became complicated due to the COVID-19 pandemic. Case Presentation: A 27-year-old female with EES of the right deltoid region presented after 3 months delay when the tumor had reached a huge size. Neoadjuvant therapy was initiated for her with acceptable results, however, her surgical treatment was postponed 3 times due to the cancellation of elective operations in the hospital as well as her involvement with COVID-19 infection. She developed multiple pulmonary metastases shortly after the surgery and passed away within a fortnight due to respiratory complications. Conclusions: Although not “emergent” by definition, surgical treatment of patients with cancer, especially those who suffer from malignancies with high metastatic potential such as Ewing sarcoma (including EES), should not be considered as “elective” since the disease may progress in a short time and become incurable.
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26
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Abboud A, Masrouha K, Saliba M, Haidar R, Saab R, Khoury N, Tawil A, Saghieh S. Extraskeletal Ewing sarcoma: Diagnosis, management and prognosis. Oncol Lett 2021; 21:354. [PMID: 33747211 DOI: 10.3892/ol.2021.12615] [Citation(s) in RCA: 56] [Impact Index Per Article: 14.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/21/2020] [Accepted: 02/02/2021] [Indexed: 12/26/2022] Open
Abstract
Extraskeletal Ewing sarcoma (EES) is a relatively uncommon primary tumor of the soft tissues, which accounts for 20-30% of all reported cases of ES. Being uncommon, all members of the ES family tumors are treated following the same general protocol of sarcoma tumors. The present review summarizes the diagnosis, management and prognosis of EES, focusing on the differences between the subtypes of ESS. The clinical features and imaging of EES are also discussed. Magnetic resonance imaging is the modality of choice for diagnostic imaging and local staging, while core-needle biopsy with pathological testing is used to obtain a definitive diagnosis. Although several oncology groups endorse that ES family of tumors should be treated with similar algorithm and protocols, some studies have demonstrated that surgery and radiotherapy may be used as a form of local control. However, further studies are required to conclude the optimum treatment option for EES.
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Affiliation(s)
- Abdallah Abboud
- Division of Orthopedic Surgery, Department of Surgery, American University of Beirut Medical Center, Beirut 1013, Lebanon
| | - Karim Masrouha
- Division of Orthopedic Oncology, Department of Orthopedic Surgery, NYU Langone Health, New York, NY 10016, USA
| | - Maelle Saliba
- Department of Pathology and Laboratory Medicine, American University of Beirut Medical Center, Beirut 1013, Lebanon
| | - Rachid Haidar
- Division of Orthopedic Surgery, Department of Surgery, American University of Beirut Medical Center, Beirut 1013, Lebanon
| | - Raya Saab
- Department of Pediatrics and Adolescent Medicine, American University of Beirut Medical Center, Beirut 1013, Lebanon
| | - Nabil Khoury
- Department of Diagnostic Radiology, American University of Beirut Medical Center, Beirut 1013, Lebanon
| | - Ayman Tawil
- Department of Pathology and Laboratory Medicine, American University of Beirut Medical Center, Beirut 1013, Lebanon
| | - Said Saghieh
- Division of Orthopedic Surgery, Department of Surgery, American University of Beirut Medical Center, Beirut 1013, Lebanon
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27
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Affiliation(s)
- Nicolò Riggi
- From the Institute of Pathology, Faculty of Biology and Medicine, University of Lausanne and Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland (N.R., I.S.); and the Department of Pathology and Center for Cancer Research, Massachusetts General Hospital and Harvard Medical School, Boston, and the Broad Institute of Harvard University and the Massachusetts Institute of Technology, Cambridge - both in Massachusetts (M.L.S.)
| | - Mario L Suvà
- From the Institute of Pathology, Faculty of Biology and Medicine, University of Lausanne and Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland (N.R., I.S.); and the Department of Pathology and Center for Cancer Research, Massachusetts General Hospital and Harvard Medical School, Boston, and the Broad Institute of Harvard University and the Massachusetts Institute of Technology, Cambridge - both in Massachusetts (M.L.S.)
| | - Ivan Stamenkovic
- From the Institute of Pathology, Faculty of Biology and Medicine, University of Lausanne and Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland (N.R., I.S.); and the Department of Pathology and Center for Cancer Research, Massachusetts General Hospital and Harvard Medical School, Boston, and the Broad Institute of Harvard University and the Massachusetts Institute of Technology, Cambridge - both in Massachusetts (M.L.S.)
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28
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Muratori F, Mondanelli N, Pelagatti L, Frenos F, Matera D, Beltrami G, Innocenti M, Capanna R, Roselli G, Scoccianti G, Livi L, Greto D, Muntoni C, Baldi G, Tamburini A, Campanacci DA. Clinical features, prognostic factors and outcome in a series of 29 extra-skeletal Ewing Sarcoma. Adequate margins and surgery-radiotherapy association improve overall survival. J Orthop 2020; 21:236-239. [PMID: 32273664 DOI: 10.1016/j.jor.2020.03.018] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/10/2020] [Accepted: 03/23/2020] [Indexed: 01/04/2023] Open
Abstract
Objective Authors review a series of 29 extra-skeletal Ewing Sarcoma (EES). Methods They analyzed characteristics, prognostic factors and outcome of EES. Results Authors report 60% Overall Survival (OS) and 56% of Event Free Survival (EFS) at 5 years. Better 5 years EFS was found in patients with localized disease (68.8%) compared to metastatic EES (33.3%) (p = 0.042). Radiotherapy + surgery offered the best local treatment (p=0.017). Volume (p = 0.032), Surgical margins (p = 0.01), metastatic disease (p = 0.0013) were a significant prognostic factor for OS at 5-yrs. Conclusion Adequate margins and surgery+radiotherapy improve Overall Survival.
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Affiliation(s)
- Francesco Muratori
- Divisione di Ortopedia Oncologica e Ricostruttiva Azienda Ospedaliero Universitaria Careggi Firenze, Italy
| | - Nicola Mondanelli
- Divisione Ortopedia e Traumatologia Azienda Ospedaliera Universitaria Siena, Italy
| | - Lorenzo Pelagatti
- Divisione di Ortopedia Oncologica e Ricostruttiva Azienda Ospedaliero Universitaria Careggi Firenze, Italy
| | - Filippo Frenos
- Divisione di Ortopedia Oncologica e Ricostruttiva Azienda Ospedaliero Universitaria Careggi Firenze, Italy
| | - Davide Matera
- Divisione di Ortopedia Oncologica e Ricostruttiva Azienda Ospedaliero Universitaria Careggi Firenze, Italy
| | - Giovanni Beltrami
- Divisione di Ortopedia Oncologica e Ricostruttiva Azienda Ospedaliero Universitaria Careggi Firenze, Italy
| | - Matteo Innocenti
- Divisione di Ortopedia Oncologica e Ricostruttiva Azienda Ospedaliero Universitaria Careggi Firenze, Italy
| | | | - Giuliana Roselli
- Istituto di Radiologia Azienda Ospedaliera Universitaria Careggi Firenze, Italy
| | - Guido Scoccianti
- Divisione di Ortopedia Oncologica e Ricostruttiva Azienda Ospedaliero Universitaria Careggi Firenze, Italy
| | - Lorenzo Livi
- Istituto di Radioterapia, Azienda Ospedaliero Universitaria Careggi Firenze, Italy
| | - Daniela Greto
- Istituto di Radioterapia, Azienda Ospedaliero Universitaria Careggi Firenze, Italy
| | - Cristina Muntoni
- Istituto di Radioterapia, Azienda Ospedaliero Universitaria Careggi Firenze, Italy
| | - Giacomo Baldi
- Divisione di Oncologia Medica, Ospedale Prato, Italy
| | | | - Domenico Andrea Campanacci
- Divisione di Ortopedia Oncologica e Ricostruttiva Azienda Ospedaliero Universitaria Careggi Firenze, Italy
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Fujibuchi T, Miyawaki J, Kidani T, Imai H, Miura H. Prediction of Soft Tissue Sarcoma from Clinical Characteristics and Laboratory Data. Cancers (Basel) 2020; 12:E679. [PMID: 32183216 PMCID: PMC7140089 DOI: 10.3390/cancers12030679] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/17/2020] [Revised: 03/10/2020] [Accepted: 03/12/2020] [Indexed: 12/15/2022] Open
Abstract
The accurate diagnosis of soft tissue tumors may be difficult. Simple clinical characteristics or laboratory data that can predict tumor malignancy can be useful tools for diagnosing soft tissue tumors. Between 2003 and 2018, 588 patients with primary soft tissue tumors were retrospectively reviewed. Their clinical characteristics and laboratory data were evaluated to determine their association with the diagnosis of benign, intermediate, or malignant tumor. Multivariable analysis revealed that tumor size ≥ 5.6 cm (odds ratio (OR), 6.15; p < 0.001), white blood cell (WBC) count ≥ 5700/µL (OR, 2.49; p = 0.002), hemoglobin (Hb) count ≤ 12.4 g/dL (OR, 2.56; p = 0.004), C-reactive protein (CRP) level ≥ 0.17 mg/dL (OR, 2.64; p < 0.001), and lactate dehydrogenase (LDH) level ≥ 240 IU/L (OR, 4.94; p < 0.001) were significant predictive factors for sarcoma. The sensitivity and specificity in the presence of three or more predictive factors for detecting malignant tumors were 0.58 and 0.90 respectively, and it was an appropriate threshold with the maximum Youden's index of 0.49. Simple clinical and laboratory data were useful tools for predicting whether the tumor is malignant. Patients with soft tissue tumors that meet any three or more predictive factors should be referred to a specialist.
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Affiliation(s)
- Taketsugu Fujibuchi
- Department of Bone and Joint Surgery, Ehime University Graduate School of Medicine, Shitsukawa, Toon, Ehime 791-0295, Japan; (J.M.); (T.K.); (H.I.); (H.M.)
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30
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Role of Radiation Therapy in Adult Extraskeletal Ewing's Sarcoma Patients Treated with Chemotherapy and Surgery. Sarcoma 2019; 2019:5413527. [PMID: 31178655 PMCID: PMC6507246 DOI: 10.1155/2019/5413527] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/26/2018] [Accepted: 03/13/2019] [Indexed: 12/23/2022] Open
Abstract
Radiation therapy (RT) is advocated in the multimodal treatment of high-grade soft tissue sarcoma (STS), but its role may be less clear in chemotherapy-sensitive STS such as extraskeletal Ewing sarcoma (EES). The purpose of this study was to determine the role of RT on overall survival (OS) in localized EES adult patients treated with chemotherapy and surgery. Adult patients diagnosed with EES and reported to the National Cancer Database from 2004 to 2014 were evaluated. All patients were treated with surgical resection. Patient demographics, tumor characteristics, treatments received, resection margins, and survival were examined for the 232 patients identified. Using multivariate analysis and Cox proportional hazard analysis, predictors of OS were determined. In the overall cohort, 40 percent of patients received RT and 78 percent received chemotherapy, with 31 percent receiving both. The addition of RT to the patients receiving surgery + chemotherapy did not improve OS (p < 0.05). Twenty-four percent of patients who achieved R0 resection after surgery still received RT without any improvement in OS. Patients treated at community cancer centers were more likely to receive additional RT compared with Comprehensive Cancer Centers (p < 0.05). In adult EES patients with localized disease treated with chemotherapy and surgery, the addition of RT does not improve overall survival.
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31
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Tan GJS, Gerrand CH, Rankin KS. Blood-borne biomarkers of osteosarcoma: A systematic review. Pediatr Blood Cancer 2019; 66:e27462. [PMID: 30251311 DOI: 10.1002/pbc.27462] [Citation(s) in RCA: 12] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/09/2018] [Revised: 08/29/2018] [Accepted: 08/30/2018] [Indexed: 12/27/2022]
Abstract
Osteosarcoma is the most common type of primary malignant bone tumor in children and young adults. Development of clinically useful biomarkers has the potential to improve treatments. The aim of this review was to investigate the recent literature assessing the utility of biomarkers for osteosarcoma. A detailed literature search was performed, with hand searches for related research publications. The search was limited to publications in English between January 2007 and February 2017. Of 286 studies identified, 24 met the inclusion criteria. There is a wide range of osteosarcoma biomarkers identified which act as clinical prognostic factors in patient outcome.
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Affiliation(s)
- Gerald J S Tan
- The Ipswich Hospital, East Suffolk and North Essex NHS Foundation Trust, Ipswich, United Kingdom
| | - C H Gerrand
- The London Sarcoma Service, Royal National Orthopaedic Hospital, Stanmore, United Kingdom
| | - K S Rankin
- North of England Bone and Soft Tissue Tumour Service, Freeman Hospital, Newcastle Upon Tyne, United Kingdom
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32
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Jiang S, Wang G, Chen J, Dong Y. Comparison of clinical features and outcomes in patients with extraskeletal vs skeletal Ewing sarcoma: an SEER database analysis of 3,178 cases. Cancer Manag Res 2018; 10:6227-6236. [PMID: 30538569 PMCID: PMC6260126 DOI: 10.2147/cmar.s178979] [Citation(s) in RCA: 22] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/20/2022] Open
Abstract
Background The clinicopathological characteristics, outcomes and prognostic factors of primary extraskeletal Ewing sarcoma (EES) remained insufficiently explored. We aimed to examine these aspects and compared the same with skeletal Ewing sarcoma (SES). Patients and methods We identified Ewing sarcoma, peripheral primitive neuroectodermal tumors or Askin tumor patients who were registered in the Surveillance, Epidemiology, and End Results database from 1973 to 2014. Clinicopathological features were assessed by using Fisher’s exact tests. Cancer-specific survival (CSS) and overall survival (OS) were estimated by using the Kaplan–Meier method and the Cox proportional hazards model. Prognostic factors were identified by multivariate Cox regression analysis. Results The age of patients with EES was diagnosed to be higher and they were more likely to be female (46.1% vs 36.2%; P<0.001), have tumor <10 cm (49.8% vs 35.4%; P<0.001), have regional node involvement (5.4% vs 1.0%; P<0.001) and receive surgery (69.1% vs 53.8%; P<0.001) compared to patients with skeletal tumors. Metastatic status did not differ by origin. Kaplan–Meier analysis showed that the origin had significant difference in CSS and OS among patients aged 0–19 years and with metastatic stage at presentation, but not in patients aged 20–39, ≥40 years and with no-metastatic stage. A Cox multivariable model controlling for differences between groups confirmed inferior survival for patients with EES. Age, tumor size, tumor stage and surgery were the most important factors significantly influencing both CSS and OS in the EES and SES patients. Race, year of diagnosis and tumor site were associated with CSS and OS among patients with SES, but failed in EES. Conclusion The clinicopathological characteristics, outcomes and prognostic factors differed among patients with EES compared to patients with SES. Extraskeletal origin was an unfavorable prognostic factor.
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Affiliation(s)
- Sujing Jiang
- Department of Medical Oncology, The Second Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, Zhejiang, China,
| | - Guannan Wang
- Department of Medical Oncology, The Second Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, Zhejiang, China,
| | - Jieyu Chen
- Department of Medical Radiology, The Second Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, Zhejiang, China
| | - Ying Dong
- Department of Medical Oncology, The Second Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, Zhejiang, China,
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Bouaoud J, Temam S, Cozic N, Galmiche-Rolland L, Belhous K, Kolb F, Bidault F, Bolle S, Dumont S, Laurence V, Plantaz D, Tabone MD, Marec-Berard P, Quassemyar Q, Couloigner V, Picard A, Gomez-Brouchet A, Le Deley MC, Mahier-Ait Oukhatar C, Kadlub N, Gaspar N. Ewing's Sarcoma of the Head and Neck: Margins are not just for surgeons. Cancer Med 2018; 7:5879-5888. [PMID: 30449071 PMCID: PMC6308064 DOI: 10.1002/cam4.1801] [Citation(s) in RCA: 15] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/13/2018] [Revised: 09/05/2018] [Accepted: 09/06/2018] [Indexed: 12/18/2022] Open
Abstract
Background, Methods To describe the characteristics, treatments (systemic/local), and outcome (oncological/functional) of French patients with head and neck Ewing's sarcomas (HNES) registered in the Euro‐Ewing 99 (EE99) database. Specific patient‐level data were reviewed retrospective. Results Forty‐seven HNES patients in the EE99 database had a median age of 11 years, 89% had bone tumors (skull 55%, mandible 21%, maxilla 11%), 89% had small tumors (<200 mL), and they were rarely metastatic (9%). Local treatment was surgery radiotherapy (55%), exclusively surgery (28%), or radiotherapy (17%). Metastatic relapses occurred in five patients with high relapse risk factors (metastasis at diagnosis, poor histological response, large tumors). Local progression/relapses (LR) after exclusive radiotherapy occurred in three patients with persistent extra‐osseous residue and in four patients considered R0 margins (postchemotherapy surgery, without postoperative radiotherapy [PORT]), reclassified by pathological review as R1a. Pathological review reclassified 72% of R0 margins: 11/18 to R1a and 2/18 to R2. Five patients had confirmed R0 margins after postchemotherapy surgery without PORT and had no LR Eight patients had R2 margins (initial surgery without previous chemotherapy, with PORT) and had no LR With a median follow‐up of 9.3 years, the 3‐year LR rate, EFS, and OS were 84.8%, 78.6%, and 89.3%, respectively. Among the 5‐year survivors, 88% had long‐term sequelae. Conclusion To optimize HNES management, patients should be treated from diagnosis in expert centers with multidisciplinary committees to discuss treatment strategy (type of surgery, need for PORT) and validate surgical margins.
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Affiliation(s)
- Jebrane Bouaoud
- Unit of maxillofacial and Plastic Surgery, Necker-Enfants Malades, Paris, France
| | - Stephane Temam
- Unit of Head and Neck Surgery, Gustave Roussy, Villejuif, France
| | - Nathalie Cozic
- Department of Biostatistics, Gustave Roussy, Villejuif, France
| | - Louise Galmiche-Rolland
- Department of Pathology, Necker-Enfants Malades, Paris, France.,University Paris Descartes, Paris, France
| | - Kahina Belhous
- University Paris Descartes, Paris, France.,Department of Pediatric Radiology, Necker-Enfants Malades, Paris, France
| | - Frederic Kolb
- Plastic and Reconstructive Surgery Department, Gustave Roussy, Villejuif, France
| | | | - Stephanie Bolle
- Radiation Oncology Department, Gustave Roussy, Villejuif, France
| | - Sarah Dumont
- Department of medical oncology, Gustave Roussy, Villejuif, France
| | | | - Dominique Plantaz
- Department of Pediatric Hematology-Oncology, University Hospital Centre of Grenoble, Grenoble, France
| | | | - Perrine Marec-Berard
- Department of Pediatric Oncology, Léon Bérard Cancer Center, Institute for Paediatric Haematology and Oncology, Lyon, France
| | - Quentin Quassemyar
- Plastic and Reconstructive Surgery Department, Gustave Roussy, Villejuif, France
| | - Vincent Couloigner
- University Paris Descartes, Paris, France.,Unit of Otolaryngology and Head and Neck Surgery, Necker-Enfants Malades, Paris, France
| | - Arnaud Picard
- Unit of maxillofacial and Plastic Surgery, Necker-Enfants Malades, Paris, France.,University Paris Descartes, Paris, France
| | | | - Marie-Cécile Le Deley
- Centre Oscar Lambret, Lille, France.,CESP, INSERM, Fac. de médecine - Univ. Paris-Sud, Université Paris-Saclay, Villejuif, France
| | | | - Natacha Kadlub
- Unit of maxillofacial and Plastic Surgery, Necker-Enfants Malades, Paris, France.,University Paris Descartes, Paris, France
| | - Nathalie Gaspar
- Department of Oncology for Child and Adolescents, Gustave Roussy, Villejuif, France
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Smolle MA, van Praag VM, Posch F, Bergovec M, Leitner L, Friesenbichler J, Heregger R, Riedl JM, Pichler M, Gerger A, Szkandera J, Stöger H, Smolle-Jüttner FM, Liegl-Atzwanger B, Fiocco M, van de Sande MA, Leithner A. Surgery for metachronous metastasis of soft tissue sarcoma - A magnitude of benefit analysis using propensity score methods. Eur J Surg Oncol 2018; 45:242-248. [PMID: 30031674 DOI: 10.1016/j.ejso.2018.06.019] [Citation(s) in RCA: 18] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/24/2018] [Revised: 05/14/2018] [Accepted: 06/20/2018] [Indexed: 12/14/2022] Open
Abstract
INTRODUCTION Metastasectomy is hypothesised to improve OS in metastatic STS, but evidence in favour of this approach derives from non-controlled single-arm cohorts affected by selection bias. The objective was to quantify the effect of metastasectomy vs. non-surgical management on overall survival (OS) in patients with metachronous metastases from extremity- and trunk soft tissue sarcoma (STS). MATERIALS AND METHODS From a population of 1578 STS patients, 135 patients who underwent surgery for localised STS at two European centres between 1998 and 2015 and developed metachronous STS metastases were included. Propensity score analyses with inverse-probability-of-treatment-weights (IPTW) and landmark analyses were performed to control for selection and immortal time bias, respectively. RESULTS OS was significantly longer in the 68 patients undergoing metastasectomy than in the 67 patients who were treated non-invasively for their metastasis (10-year OS: 23% vs. 4%; hazard ratio (HR) = 0.34, 95% CI: 0.22-0.53, p < 0.0001). This association prevailed after IPTW-weighting of the data to control for the higher prevalence of favourable prognostic factors in the surgery group (adjusted 10-year OS: 17% vs. 3%, log-rank p < 0.0001; HR = 0.33, 95% CI: 0.20-0.52, p < 0.0001). Five-year OS estimates were 27.8% in patients who had and 14.5% in patients who had not undergone metastasectomy within the first 3 months after diagnosis of a metastasis (p < 0.0001). CONCLUSION In this observational bi-centre study, metastasectomy was associated with prolonged survival in patients with metachronous STS metastases. In the absence of randomized studies, our results indicate that metastasectomy should be considered as an important treatment option for metachronous STS metastases.
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Affiliation(s)
- Maria A Smolle
- Department of Orthopaedics and Trauma, Medical University of Graz, Auenbruggerplatz 5, 8036, Graz, Austria; Comprehensive Cancer Centre Graz, Graz, Austria.
| | - Veroniek M van Praag
- Department of Orthopaedic Surgery, Leiden University Medical Centre, Albinusdreef 2, 2333 ZA, Leiden, The Netherlands.
| | - Florian Posch
- Comprehensive Cancer Centre Graz, Graz, Austria; Division of Oncology, Department of Internal Medicine, Medical University of Graz, Auenbruggerplatz 15, 8036, Graz, Austria.
| | - Marko Bergovec
- Department of Orthopaedics and Trauma, Medical University of Graz, Auenbruggerplatz 5, 8036, Graz, Austria; Comprehensive Cancer Centre Graz, Graz, Austria.
| | - Lukas Leitner
- Department of Orthopaedics and Trauma, Medical University of Graz, Auenbruggerplatz 5, 8036, Graz, Austria; Comprehensive Cancer Centre Graz, Graz, Austria.
| | - Jörg Friesenbichler
- Department of Orthopaedics and Trauma, Medical University of Graz, Auenbruggerplatz 5, 8036, Graz, Austria; Comprehensive Cancer Centre Graz, Graz, Austria.
| | - Ronald Heregger
- Comprehensive Cancer Centre Graz, Graz, Austria; Division of Oncology, Department of Internal Medicine, Medical University of Graz, Auenbruggerplatz 15, 8036, Graz, Austria.
| | - Jakob M Riedl
- Comprehensive Cancer Centre Graz, Graz, Austria; Division of Oncology, Department of Internal Medicine, Medical University of Graz, Auenbruggerplatz 15, 8036, Graz, Austria.
| | - Martin Pichler
- Comprehensive Cancer Centre Graz, Graz, Austria; Division of Oncology, Department of Internal Medicine, Medical University of Graz, Auenbruggerplatz 15, 8036, Graz, Austria; Department of Experimental Therapeutics, The UT MD Anderson Cancer Center, Sout Campus Research Building 4, 1901 East Road, Houston, TX, USA.
| | - Armin Gerger
- Comprehensive Cancer Centre Graz, Graz, Austria; Division of Oncology, Department of Internal Medicine, Medical University of Graz, Auenbruggerplatz 15, 8036, Graz, Austria.
| | - Joanna Szkandera
- Comprehensive Cancer Centre Graz, Graz, Austria; Division of Oncology, Department of Internal Medicine, Medical University of Graz, Auenbruggerplatz 15, 8036, Graz, Austria.
| | - Herbert Stöger
- Comprehensive Cancer Centre Graz, Graz, Austria; Division of Oncology, Department of Internal Medicine, Medical University of Graz, Auenbruggerplatz 15, 8036, Graz, Austria.
| | - Freyja-Maria Smolle-Jüttner
- Comprehensive Cancer Centre Graz, Graz, Austria; Division of Thoracic and Hyperbaric Surgery, Medical University of Graz, Auenbruggerplatz 29, 8036, Austria.
| | - Bernadette Liegl-Atzwanger
- Comprehensive Cancer Centre Graz, Graz, Austria; Institute of Pathology, Medical University of Graz, Auenbruggerplatz 25, 8036, Graz, Austria.
| | - Marta Fiocco
- Department of Orthopaedic Surgery, Leiden University Medical Centre, Albinusdreef 2, 2333 ZA, Leiden, The Netherlands; Department of Medical Statistics and Bioinformatics, Leiden University Medical Centre, Albinusdreef 2, 2333 ZA, Leiden, The Netherlands; Mathematical Institute Leiden University, Albinusdreef 2, 2333 ZA, Leiden, The Netherlands.
| | - Michiel Aj van de Sande
- Department of Orthopaedic Surgery, Leiden University Medical Centre, Albinusdreef 2, 2333 ZA, Leiden, The Netherlands.
| | - Andreas Leithner
- Department of Orthopaedics and Trauma, Medical University of Graz, Auenbruggerplatz 5, 8036, Graz, Austria; Comprehensive Cancer Centre Graz, Graz, Austria.
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Gao J, Chow E, Aloma A, Gupta P. Peripheral primitive neuroendocrine tumor of the chest wall-A case report with pathological correlation. Radiol Case Rep 2018; 13:392-396. [PMID: 29904480 PMCID: PMC6000041 DOI: 10.1016/j.radcr.2018.01.003] [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] [Accepted: 01/04/2018] [Indexed: 12/20/2022] Open
Abstract
Primitive neuroectodermal tumor is a high-grade malignant tumor originating from the neural crest and neuroectoderm, which can be subdivided into central and peripheral categories. Peripheral primitive neuroectodermal tumor is thought to be identical to Ewing's sarcoma, and falls under a broader category of Ewing's sarcoma family of tumors. Very rarely, it may present without osseous involvement, known as extraosseous Ewing's sarcoma. Here we present a case of a 38-year-old woman, who presented with several-month history of a slow-growing chest wall mass, initially thought to be a breast mass. The mass was diagnosed as extraosseous Ewing's sarcoma upon tissue biopsy. The patient was started on a dose-intensified neoadjuvant therapy, based on protocol from pediatric population given rarity of this tumor in the adult population. While the patient was initially planned for surgical resection, the tumor showed excellent response to chemotherapy on follow-up imaging, and radiation therapy was elected in lieu of resection.
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Affiliation(s)
- Jidi Gao
- Tufts Medical Center, 800 Washington St, Boston, MA 02111, USA
- Corresponding author.
| | - Erika Chow
- Tufts University School of Medicine, 145 Harrison Ave, Boston, MA 02111, USA
| | - Aishatu Aloma
- Tufts Medical Center, 800 Washington St, Boston, MA 02111, USA
| | - Payal Gupta
- Tufts Medical Center, 800 Washington St, Boston, MA 02111, USA
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Iatrou I, Theologie-Lygidakis N, Schoinohoriti O, Tzermpos F, Mylonas AI. Ewing's sarcoma of the maxillofacial region in Greek children: Report of 6 cases and literature review. J Craniomaxillofac Surg 2018; 46:213-221. [DOI: 10.1016/j.jcms.2017.12.009] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/14/2017] [Revised: 11/02/2017] [Accepted: 12/08/2017] [Indexed: 11/28/2022] Open
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Li S, Yang Q, Wang H, Wang Z, Zuo D, Cai Z, Hua Y. Prognostic significance of serum lactate dehydrogenase levels in Ewing's sarcoma: A meta-analysis. Mol Clin Oncol 2016; 5:832-838. [PMID: 28105365 DOI: 10.3892/mco.2016.1066] [Citation(s) in RCA: 26] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/16/2016] [Accepted: 08/12/2016] [Indexed: 11/05/2022] Open
Abstract
A number of studies have investigated the role of serum lactate dehydrogenase (LDH) levels in patients with Ewing's sarcoma, although these have yielded inconsistent and inconclusive results. Therefore, the present study aimed to systematically review the published studies and conduct a meta-analysis to assess its prognostic value more precisely. Cohort studies assessing the prognostic role of LDH levels in patients with Ewing's sarcoma were included. A pooled hazard ratio (HR) with 95% confidence intervals (CIs) of overall survival (OS) or 5-year disease-free survival (DFS) was used to assess the prognostic role of the levels of serum LDH. Nine studies published between 1980 and 2014, with a total of 1,412 patients with Ewing's sarcoma, were included. Six studies, with a total of 644 patients, used OS as the primary endpoint and four studies, with 795 patients, used 5-year DFS. Overall, the pooled HR evaluating high LDH levels was 2.90 (95% CI: 2.09-4.04) for OS and 2.40 (95% CI: 1.93-2.98) for 5-year DFS. This meta-analysis demonstrates that high levels of serum LDH are associated with lower OS and 5-year DFS rates in patients with Ewing's sarcoma. Therefore, serum LDH levels are an effective biomarker of Ewing's sarcoma prognosis.
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Affiliation(s)
- Suoyuan Li
- Department of Orthopedics, Shanghai General Hospital, Nanjing Medical University, Shanghai, P.R. China
| | - Qing Yang
- Department of Orthopedics, Fudan University-Affiliated Zhongshan Hospital, Qingpu Branch, Shanghai, P.R. China
| | - Hongsheng Wang
- Department of Orthopedics, Shanghai 10th People's Hospital, School of Medicine Tongji University, Shanghai, P.R. China
| | - Zhuoying Wang
- Department of Orthopedics, Shanghai General Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, P.R. China
| | - Dongqing Zuo
- Department of Orthopedics, Shanghai 10th People's Hospital, School of Medicine Tongji University, Shanghai, P.R. China
| | - Zhengdong Cai
- Department of Orthopedics, Shanghai General Hospital, Nanjing Medical University, Shanghai, P.R. China
| | - Yingqi Hua
- Department of Orthopedics, Shanghai General Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, P.R. China
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Cash T, McIlvaine E, Krailo MD, Lessnick SL, Lawlor ER, Laack N, Sorger J, Marina N, Grier HE, Granowetter L, Womer RB, DuBois SG. Comparison of clinical features and outcomes in patients with extraskeletal versus skeletal localized Ewing sarcoma: A report from the Children's Oncology Group. Pediatr Blood Cancer 2016; 63:1771-9. [PMID: 27297500 PMCID: PMC4995129 DOI: 10.1002/pbc.26096] [Citation(s) in RCA: 76] [Impact Index Per Article: 8.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/14/2016] [Revised: 05/18/2016] [Accepted: 05/18/2016] [Indexed: 12/27/2022]
Abstract
BACKGROUND The prognostic significance of having extraskeletal (EES) versus skeletal Ewing sarcoma (ES) in the setting of modern chemotherapy protocols is unknown. The purpose of this study was to compare the clinical characteristics, biologic features, and outcomes for patients with EES and skeletal ES. METHODS Patients had localized ES and were treated on two consecutive protocols using five-drug chemotherapy (INT-0154 and AEWS0031). Patients were analyzed based on having an extraskeletal (n = 213) or skeletal (n = 826) site of tumor origin. Event-free survival (EFS) was estimated using the Kaplan-Meier method, compared using the log-rank test, and modeled using Cox multivariate regression. RESULTS Patients with extraskeletal ES (EES) were more likely to have axial tumors (72% vs. 55%; P < 0.001), less likely to have tumors >8 cm (9% vs. 17%; P < 0.01), and less likely to be white (81% vs. 87%; P < 0.001) compared to patients with skeletal ES. There was no difference in key genomic features (type of EWSR1 translocation, TP53 mutation, CDKN2A mutation/loss) between groups. After controlling for age, race, and primary site, EES was associated with superior EFS (hazard ratio = 0.69; 95% confidence interval: 0.50-0.95; P = 0.02). Among patients with EES, age ≥18, nonwhite race, and elevated baseline erythrocyte sedimentation rate were independently associated with inferior EFS. CONCLUSION Clinical characteristics, but not key tumor genomic features, differ between EES and skeletal ES. Extraskeletal origin is a favorable prognostic factor, independent of age, race, and primary site.
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Affiliation(s)
- Thomas Cash
- Department of Pediatrics, Emory University, Children’s Healthcare of Atlanta, Health Sciences Research Building, Brumley Bridge, 3 Floor, W-350, 1760 Haygood Drive, Atlanta, GA 30322
| | - Elizabeth McIlvaine
- Department of Preventive Medicine, Keck School of Medicine, University of Southern California, 2001 N Soto Street, SSB 210C, MC 9234, Los Angeles, CA 90032
| | - Mark D. Krailo
- Department of Preventive Medicine, Keck School of Medicine, University of Southern California, 2001 N Soto Street, SSB 210C, MC 9234, Los Angeles, CA 90032
| | - Stephen L. Lessnick
- Center for Childhood Cancer and Blood Disorders at Nationwide Children’s Hospital and the Division of Hematology, Oncology and BMT at The Ohio State University, 700 Children’s Drive, WA5011, Columbus, OH 43205
| | - Elizabeth R. Lawlor
- Department of Pediatrics, University of Michigan, D4204 Med Prof Building Box 5718, 1500 East Med Center Drive, Ann Arbor, MI 48109-5718
| | - Nadia Laack
- Department of Radiation Oncology, Mayo Clinic, 200 First St SW, Rochester, MN 55905
| | - Joel Sorger
- Department of Orthopedics, Cincinnati Children’s Hospital Medical Center, 3333 Burnet Ave, ML 2017, Cincinnati, OH 45229
| | - Neyssa Marina
- Department of Pediatrics, Stanford University School of Medicine and Lucille Packard Children’s Hospital at Stanford, 1000 Welch Rd., Suite 300, Mail Code 5798, Palo Alto, CA 94304-1812
| | - Holcombe E. Grier
- Department of Pediatrics, Children’s Hospital Boston/Dana-Farber Cancer Institute and Harvard Medical School, 44 Binney Street, Boston, MA 02115
| | - Linda Granowetter
- Department of Pediatrics, NYU School of Medicine and NYU Langone Medical Center, 160 East 32nd Street, 2nd Floor, New York, NY 10016
| | - Richard B. Womer
- Department of Pediatrics, University of Pennsylvania Perelman School of Medicine and Children’s Hospital of Philadelphia, 324 South 34th St, Philadelphia, PA 19104
| | - Steven G. DuBois
- Department of Pediatrics, UCSF School of Medicine and UCSF Benioff Children’s Hospital, 550 16th Street, 4th Floor, San Francisco, CA 94158
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Biswas B, Bakhshi S. Management of Ewing sarcoma family of tumors: Current scenario and unmet need. World J Orthop 2016; 7:527-538. [PMID: 27672565 PMCID: PMC5027007 DOI: 10.5312/wjo.v7.i9.527] [Citation(s) in RCA: 36] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/27/2016] [Revised: 06/21/2016] [Accepted: 07/13/2016] [Indexed: 02/06/2023] Open
Abstract
Ewing sarcoma family tumors (ESFT) are heterogeneous, aggressive group of disease with peak incidence in adolescent and young adults. The outcome has been improved dramatically from 10% with surgery and radiotherapy alone to 65%-70% now, in localized disease, with the introduction of chemotherapy. Chemotherapy regimen evolved from single agent to multiagent with effort of many cooperative clinical trials over decades. The usual treatment protocol include introduction of multi-agent chemotherapy in neoadjuvant setting to eradicate systemic disease with timely incorporation of surgery and/or radiotherapy as local treatment modality and further adjuvant chemotherapy to prevent recurrence. Risk adapted chemotherapy in neoadjuvant and adjuvant setting along with radiotherapy has been used in many international collaborative trials and has resulted in improved outcome, more so in patients with localized disease. The role of high dose chemotherapy with stem cell rescue is still debatable. The outcome of patients with metastatic disease is dismal with long term outcome ranges from 20%-40% depending on the sites of metastasis and intensity of treatment. There is a huge unmet need to improve outcome further, more so in metastatic setting. Novel therapy targeting the molecular pathways and pathogenesis of ESFT is very much required. Here we have discussed the current standard of management in patients with ESFT, investigational targeted or novel therapies along with future promises.
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Ramaswamy A, Rekhi B, Bakhshi S, Hingmire S, Agarwal M. Indian data on bone and soft tissue sarcomas: A summary of published study results. South Asian J Cancer 2016; 5:138-45. [PMID: 27606300 PMCID: PMC4991135 DOI: 10.4103/2278-330x.187587] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
Abstract
Bone sarcomas are rare tumors, approximating 0.2% of all cancers, with osteosarcoma (OGS), chondrosarcoma, and Ewing sarcoma being the most common cancers in this subset. The formation of disease management groups/clinics focused on sarcomas has resulted in better understanding and management of these uncommon tumors. Multiple large-scale retrospective data from Tata Memorial Hospital (TMH) and All India Institute of Medical Sciences have reported outcomes comparable to Western data in the field of OGS and Ewing sarcoma, with interesting prognostic factors identified for further evaluation. Soft tissue sarcomas are a rare heterogeneous group of tumors, more than 50 different tumor entities. The common subtypes identified in India include Ewing sarcoma and synovial sarcoma. Valuable work regarding brachytherapy has been done by radiation oncologists from the TMH, especially in pediatric patients.
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Affiliation(s)
- Anant Ramaswamy
- Department of Medical Oncology, Tata Memorial Hospital, Mumbai, Maharashtra, India
| | - Bharat Rekhi
- Department of Pathology, Tata Memorial Hospital, Parel, Mumbai, Maharashtra, India
| | - Sameer Bakhshi
- All India Institute of Medical Sciences, New Delhi, India
| | - Sachin Hingmire
- Department of Oncology, Deenanath Mangeshkar Hospital, Pune, Maharashtra, India
| | - Manish Agarwal
- Department of Orthopedic Oncology, P. D. Hinduja Hospital and Medical Research Centre, Mumbai, Maharashtra, India
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Bailey KM, Airik M, Krook MA, Pedersen EA, Lawlor ER. Micro-Environmental Stress Induces Src-Dependent Activation of Invadopodia and Cell Migration in Ewing Sarcoma. Neoplasia 2016; 18:480-8. [PMID: 27566104 PMCID: PMC5018098 DOI: 10.1016/j.neo.2016.06.008] [Citation(s) in RCA: 21] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/31/2016] [Revised: 06/04/2016] [Accepted: 06/29/2016] [Indexed: 01/05/2023]
Abstract
Metastatic Ewing sarcoma has a very poor prognosis and therefore new investigations into the biologic drivers of metastatic progression are key to finding new therapeutic approaches. The tumor microenvironment is highly dynamic, leading to exposure of different regions of a growing solid tumor to changes in oxygen and nutrient availability. Tumor cells must adapt to such stress in order to survive and propagate. In the current study, we investigate how Ewing sarcoma cells respond to the stress of growth factor deprivation and hypoxia. Our findings reveal that serum deprivation leads to a reversible change in Ewing cell cytoskeletal phenotypes. Using an array of migration and invasion techniques, including gelatin matrix degradation invadopodia assays, we show that exposure of Ewing sarcoma cells to serum deprivation and hypoxia triggers enhanced migration, invadopodia formation, matrix degradation and invasion. Further, these functional changes are accompanied by and dependent on activation of Src kinase. Activation of Src, and the associated invasive cell phenotype, were blocked by exposing hypoxia and serum-deprived cells to the Src inhibitor dasatinib. These results indicate that Ewing sarcoma cells demonstrate significant plasticity in response to rapidly changing micro-environmental stresses that can result from rapid tumor growth and from necrosis-causing therapies. In response to these stresses, Ewing cells transition to a more migratory and invasive state and our data show that Src is an important mediator of this stress response. Our data support exploration of clinically available Src inhibitors as adjuvant agents for metastasis prevention in Ewing sarcoma.
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Affiliation(s)
- Kelly M Bailey
- University of Michigan, Department of Pediatrics, Ann Arbor, MI, 48109, USA; University of Michigan, Department of Translational Oncology Program, Ann Arbor, MI, 48109, USA
| | - Merlin Airik
- University of Michigan, Department of Pediatrics, Ann Arbor, MI, 48109, USA; University of Michigan, Department of Translational Oncology Program, Ann Arbor, MI, 48109, USA
| | - Melanie A Krook
- University of Michigan, Department of Pediatrics, Ann Arbor, MI, 48109, USA; University of Michigan, Department of Translational Oncology Program, Ann Arbor, MI, 48109, USA
| | - Elisabeth A Pedersen
- University of Michigan, Department of Pathology, Ann Arbor, MI, 48109, USA; University of Michigan, Department of Translational Oncology Program, Ann Arbor, MI, 48109, USA
| | - Elizabeth R Lawlor
- University of Michigan, Department of Pediatrics, Ann Arbor, MI, 48109, USA; University of Michigan, Department of Pathology, Ann Arbor, MI, 48109, USA; University of Michigan, Department of Translational Oncology Program, Ann Arbor, MI, 48109, USA.
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Grevener K, Haveman LM, Ranft A, van den Berg H, Jung S, Ladenstein R, Klco-Brosius S, Juergens H, Merks JHM, Dirksen U. Management and Outcome of Ewing Sarcoma of the Head and Neck. Pediatr Blood Cancer 2016; 63:604-10. [PMID: 26702872 DOI: 10.1002/pbc.25830] [Citation(s) in RCA: 47] [Impact Index Per Article: 5.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/13/2015] [Revised: 10/09/2015] [Accepted: 10/09/2015] [Indexed: 12/26/2022]
Abstract
BACKGROUND Ewing sarcoma (EWS) of the head and neck is rare. Multimodal treatment consists of chemotherapy and local treatment; however, local treatment for EWS of the head and neck is challenging. The first objective was to describe local treatment administered to the patients with localized EWS of the head and neck according to the EURO-E.W.I.N.G.99-trial, and to assess the impact on survival. The second objective was to systematically review the scientific literature available for this topic. PROCEDURE Fifty-one patients were included. Local control consisted of surgery and/or radiotherapy (RT). Event-free survival (EFS) and overall survival (OS) were determined. Outcome was analyzed by comparing local treatment approaches. A Medline search was performed for EWS of the head and neck. RESULTS Eighty-six percent of patients had localized disease. Most common primary sites included the skull (45%), maxilla (14%), and mandible (12%). Three-year EFS was 74% and 3-year OS was 87% for patients with localized disease. EFS was 40% for patients >15 years compared to 81% for patients <15 years. Local control consisted of surgery (S; 33%), RT (18%), or S + RT (45%). Related 3-year EFS was 81% (S), 80% (RT), and 72% (S + RT); 3-year OS was 80%, 76%, and 81%, respectively. CONCLUSIONS In patients with EWS of the head and neck, age, and stage are important prognostic factors. Although not statistically significant, large tumor volume seems to be a negative prognostic factor. No difference in EFS and OS could be found when comparing patients treated with surgery, RT, or combined surgery and RT.
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Affiliation(s)
- Knut Grevener
- Department of Pediatric Hematology and Oncology, University Hospital Muenster, Muenster, Germany
| | - Lianne M Haveman
- Department of Pediatric Oncology, Emma Children's Hospital, Academic Medical Centre, Amsterdam, The Netherlands
| | - Andreas Ranft
- Department of Pediatric Hematology and Oncology, University Hospital Muenster, Muenster, Germany
| | - Henk van den Berg
- Department of Pediatric Oncology, Emma Children's Hospital, Academic Medical Centre, Amsterdam, The Netherlands
| | - Susanne Jung
- Department of Maxillofacial Surgery, University Hospital Muenster, Muenster, Germany
| | - Ruth Ladenstein
- St Anna Children's Hospital, Vienna, Austria.,Children's Cancer Research Institute, Vienna, Austria
| | - Stephanie Klco-Brosius
- Department of Pediatric Hematology and Oncology, University Hospital Muenster, Muenster, Germany
| | - Heribert Juergens
- Department of Pediatric Hematology and Oncology, University Hospital Muenster, Muenster, Germany
| | - J Hans M Merks
- Department of Maxillofacial Surgery, University Hospital Muenster, Muenster, Germany
| | - Uta Dirksen
- Department of Pediatric Hematology and Oncology, University Hospital Muenster, Muenster, Germany
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Sangkhathat S. Current management of pediatric soft tissue sarcomas. World J Clin Pediatr 2015; 4:94-105. [PMID: 26566481 PMCID: PMC4637813 DOI: 10.5409/wjcp.v4.i4.94] [Citation(s) in RCA: 36] [Impact Index Per Article: 3.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/29/2015] [Revised: 07/21/2015] [Accepted: 09/29/2015] [Indexed: 02/06/2023] Open
Abstract
Pediatric soft tissue sarcomas are a group of malignant neoplasms arising within embryonic mesenchymal tissues during the process of differentiation into muscle, fascia and fat. The tumors have a biphasic peak for age of incidence. Rhabdomyosarcoma (RMS) is diagnosed more frequently in younger children, whereas adult-type non-RMS soft tissue sarcoma is predominately observed in adolescents. The latter group comprises a variety of rare tumors for which diagnosis can be difficult and typically requires special studies, including immunohistochemistry and molecular genetic analysis. Current management for the majority of pediatric sarcomas is based on the data from large multi-institutional trials, which has led to great improvements in outcomes over recent decades. Although surgery remains the mainstay of treatment, the curative aim cannot be achieved without adjuvant treatment. Pre-treatment staging and risk classification are of prime importance in selecting an effective treatment protocol. Tumor resectability, the response to induction chemotherapy, and radiation generally determine the risk-group, and these factors are functions of tumor site, size and biology. Surgery provides the best choice of local control of small resectable tumors in a favorable site. Radiation therapy is added when surgery leaves residual disease or there is evidence of regional spread. Chemotherapy aims to reduce the risk of relapse and improve overall survival. In addition, upfront chemotherapy reduces the aggressiveness of the required surgery and helps preserve organ function in a number of cases. Long-term survival in low-risk sarcomas is feasible, and the intensity of treatment can be reduced. In high-risk sarcoma, current research is allowing more effective disease control.
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Wong T, Goldsby RE, Wustrack R, Cash T, Isakoff MS, DuBois SG. Clinical features and outcomes of infants with Ewing sarcoma under 12 months of age. Pediatr Blood Cancer 2015; 62:1947-51. [PMID: 26173989 DOI: 10.1002/pbc.25635] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/09/2015] [Accepted: 05/18/2015] [Indexed: 11/07/2022]
Abstract
BACKGROUND Ewing sarcoma peaks in incidence in adolescence. Infants <12 months old have rarely been reported. We aimed to compare clinical features, treatment, and survival of infants <12 months to those of older pediatric patients with Ewing sarcoma. PROCEDURE We utilized the SEER database to identify patients <12 months of age diagnosed with Ewing sarcoma between 1973 and 2011. We used Fisher exact tests to compare clinical features and treatment modalities between these patients and patients aged 1-19 years. We used Kaplan-Meier methods to describe overall survival in these two groups. RESULTS Of 1,957 patients in the cohort, 39 (2.0%) were diagnosed at <12 months of age. Infants had a different distribution of primary tumor sites, with lower extremity tumors under represented. Compared to older patients, infants were more likely to have soft tissue tumors (81.6% vs. 27.1%; P < 0.001); have primitive neuroectodermal tumor/Askin tumor (61.5% vs. 19.9%; P < 0.001); and have tumors <8 cm (81.0% vs. 53.2%; P < 0.014). Infants were less likely to receive radiation therapy (13.2% vs. 53.3%; P < 0.001). Infants were at increased risk for early death (P < 0.013 by Wilcoxon), though long-term overall survival was not different between age groups (P < 0.25 by log rank). CONCLUSIONS Ewing sarcoma is rare in infants, with different clinical presentations and treatment approaches. These patients appear to be at higher risk for early death, but long-term survival is similar to older pediatric patients.
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Affiliation(s)
- Thalia Wong
- Department of Pediatrics, San Francisco School of Medicine, UCSF Benioff Children's Hospital, University of California, San Francisco, California
| | - Robert E Goldsby
- Department of Pediatrics, San Francisco School of Medicine, UCSF Benioff Children's Hospital, University of California, San Francisco, California
| | - Rosanna Wustrack
- Department of Orthopedics, San Francisco School of Medicine, UCSF Benioff Children's Hospital, University of California, California
| | - Thomas Cash
- Department of Pediatrics, Emory University, Atlanta, Georgia
| | - Michael S Isakoff
- Center for Cancer and Blood Disorders, Connecticut Children's Medical Center, University of Connecticut, Hartford, Connecticut
| | - Steven G DuBois
- Department of Pediatrics, San Francisco School of Medicine, UCSF Benioff Children's Hospital, University of California, San Francisco, California
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Pochon C, Martelli H, Mussini C, Pariente D, Heymann MF, Corradini N. Primary Hepatic Ewing Sarcoma With EWS-FLI1 RNA Transcript in a Child. J Pediatr Hematol Oncol 2015; 37:411-3. [PMID: 25411865 DOI: 10.1097/mph.0000000000000289] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
Affiliation(s)
- Cécile Pochon
- *Pediatric and Adolescent Oncology Department, Nantes University Hospital ∥Physiopathology of Bone Resorption and Therapy of Primitive Bone Tumors Laboratory, Nantes University UMR 957 ¶Pathology Department, Centre Hospitalier Universitaire, Nantes Departments of †Pediatric Surgery ‡Pediatric Pathology §Pediatric Radiology, Bicêtre Hospital Le Kremlin-Bicêtre, France
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Celecoxib inhibits Ewing sarcoma cell migration via actin modulation. J Surg Res 2015; 198:424-33. [PMID: 25934222 DOI: 10.1016/j.jss.2015.03.085] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/02/2015] [Revised: 03/16/2015] [Accepted: 03/27/2015] [Indexed: 01/07/2023]
Abstract
BACKGROUND Ewing sarcoma (ES) is an aggressive childhood solid tumor in which 30% of cases are metastatic at presentation, and subsequently carry a poor prognosis. We have previously shown that treatment with celecoxib significantly reduces invasion and metastasis of ES cells in a cyclooxygenase-2-independent fashion. Celecoxib is known to downregulate β-catenin independently of cyclooxygenase-2. Additionally, the actin cytoskeleton is known to play an important role in tumor micrometastasis. We hypothesized that celecoxib's antimetastatic effect in ES acts via modulation of one of these two targets. METHODS ES cells were treated with celecoxib, and the levels of β-catenin and total actin were examined by Western blot and quantitative polymerase chain reaction. Cells were transfected with small interfering RNA targeting β-catenin, and invasion assays were performed. Immunofluorescence staining for β-catenin and F-actin was performed on treated and untreated cells. Additionally, cells were subjected to a wound healing assay to assess migration. RESULTS Celecoxib had no effect on the messenger RNA or protein levels of β-catenin but did significantly decrease the amount of total actin within ES cells. Reduction of β-catenin by small interfering RNA had no effect on invasion, and celecoxib treatment of the β-catenin depleted cells continued to inhibit invasion. Immunofluorescence staining demonstrated no change in β-catenin with treatment but did show a significant reduction in the amount of F-actin, as well as morphologic changes of the cells. Wound healing assays demonstrated that celecoxib significantly inhibited migration. CONCLUSIONS Celecoxib does not exert its antimetastatic effects in ES through alteration of β-catenin but does significantly modulate the actin cytoskeleton.
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Sugitani A, Asai K, Kojima K, Eguchi Y, Kawaguchi T, Ohsawa M, Hirata K. Primary Pleural Synovial Sarcoma Treated with Pazopanib. Intern Med 2015; 54:2051-5. [PMID: 26278301 DOI: 10.2169/internalmedicine.54.3570] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/06/2022] Open
Abstract
A 42-year-old woman presented with chest pain and breathlessness with a nodule measuring 2×2 cm in size at the base of the right lung. A bronchoscopic examination did not reveal any malignancy. However, the patient developed difficulty in breathing, enlargement of the nodule, and right pleural effusion 14 days later. A video-assisted thoracic surgical biopsy specimen revealed the presence of pleural synovial sarcoma. The patient was treated with doxorubicin-ifosfamide combination chemotherapy because of metastasis to the pelvis. However, after a transient partial clinical response, there was a relapse of refractory disease. Although treated with pazopanib as second-line chemotherapy, the patient died eight months after the initial presentation.
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Affiliation(s)
- Arata Sugitani
- Department of Respiratory Medicine, Graduate School of Medicine, Osaka City University, Japan
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