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Haemorrhagic soft-tissue sarcoma: Oncological outcomes and prognostic factors for survival
Affiliation:1. The Royal Orthopaedic Hospital NHS Foundation Trust, Bristol Road South, Northfield, Birmingham, B31 2AP, UK;2. Department of Orthopedic Surgery, College of Medicine, King Saud University, Riyadh, Saudi Arabia;3. College of Health and Life Sciences, Aston University, Birmingham, B4 7ET, UK;4. Aston Medical School, Aston University, Birmingham, B4 7ET, UK;1. Department of Medical Oncology, IRCCS Fondazione Istituto Nazionale Tumori (INT), 20133 Milan, Italy;2. Department of Medical Oncology, Sarcoma Center, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA 02215, USA;3. Department of Medicine, Centre Léon Bérard, Université Claude Bernard Lyon I, Unicancer, 69008 Lyon, France;4. Medical Oncology Department, Tata Memorial Centre, Homi Bhabha National Institute, 400012 Mumbai, India;5. Department of Medical Oncology, Ospedale Santo Stefano, 59100, Prato, Italy;6. Department of Radiation Oncology, Dana-Farber Cancer Institute/ Brigham and Women’s Hospital, Boston 02215, MA, USA;7. Department of Sarcoma Medical Oncology, The University of Texas M.D. Anderson Cancer Center, Houston 77030, TX, USA;8. Department of Surgical Oncology, Institut Curie, Université Paris Sciences et Lettres, 75005, France;9. Departmen of Pathology, Leiden University Medical Center, 2333 ZA, Leiden, The Netherlands;10. Department of Surgery, INT, 20133 Milan, Italy;11. Department of Medicine, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, 10065, New York, NY, USA;12. Division of Hematology-Oncology, Vanderbilt University Medical Center, Nashville, TN 37232, USA;13. Department of Pathology, Azienda Ospedaliera Università Padova, 35129, Padova, Italy;14. Department of Laboratory Medicine and Pathobiology, University of Toronto & Pathology and Laboratory Medicine Mount Sinai Hospital, ON M5G 1X5, Toronto, Canada;15. Department of Medical Oncology, Peter MacCallum Cancer Centre, Melbourne VIC 3000, Australia;p. Soft tissue and bone sarcoma service, University College Hospital, UCLH NHS Trust, NW1 2BU, London, United Kingdom;q. Department of Oncology, Skåne University Hospital, and Lund University, 222 42, Lund, Sweden;r. Department of Medical Oncology, Leiden University Medical Center, 2333 ZA, Leiden, The Netherlands;s. Mount Sinai Hospital, Princess Margaret Hospital, University of Toronto, ON M5G 1X5, Toronto, ON, Canada;t. The Hospital for Sick Children and Princess Margaret Cancer Center, University of Toronto, ON M5G 2C1, Toronto, Canada;u. Department of Radiotherapy, the Netherlands Cancer Institute, 1066 CX, Amsterdam and the Leiden University Medical Center, 2333 ZA, Leiden, The Netherlands;v. Department of Surgery, the Royal Marsden NHS Foundation Trust, SW3 6JJ, London, United Kingdom;w. Division of Surgical Oncology and Thoracic Surgery, Mannheim University Medical Center, Medical Faculty Mannheim, University of Heidelberg, 69117 Heidelberg, Germany;x. Departments of Orthopaedics and Oncological Sciences, Huntsman Cancer Institute, University of Utah, UT 84112, Salt Lake City, USA;y. Sarcoma Unit, the Royal Marsden NHS Foundation Trust and Institute of Cancer Research, SW3 6JJ, London, United Kingdom;z. Sarcoma Unit, Mannheim Cancer Center (MCC), Mannheim University Medical Center, University of Heidelberg, 68167 Mannheim, Germany;11. Department of Musculoskeletal Oncology, National Cancer Center Hospital, Tokyo, Japan;12. Department of Radiation Oncology, Duke University Medical Center, NC 27710 Durham, USA;13. Division of Pediatrics, University of Texas M.D. Anderson Cancer Center, 77030 Huston, TX, USA;14. Medical Oncology, Insitut Gustave Roussy, 94805 Villejuif, Ile-de-France, France;15. Unit of Oncogenetics and Functional Oncogenomics, 33081 Aviano, Italy;16. Medical Oncology Department, University Hospital Fundacion Jimenez Diaz, University Hospital General de Villalba and Instituto de Investigacion Sanitaria FJD, 28040 Madrid, Spain;17. Abramson Cancer Center, University of Pennsylvania, 19104 Philadelphia, PA, USA;18. Department of Radiation Therapy, the Royal Marsden NHS Foundation Trust, SW3 6JJ, London, United Kingdom;19. Osteoncology, Soft Tissue and Bone Sarcoma and Innovative Therapy Unit, IRCCS Istituto Ortopedico Rizzoli, 40136 Bologna, Italy;110. Department of Surgery, Brigham and Women’s Hospital, Center for Sarcoma and Bone Oncology, DFCC, Harvard Medical School, Boston 02215, MA, USA;111. Princess Margaret Cancer Center, ON M5G 2C1, Toronto, Canada;112. Department of Individualized Cancer Management, Moffitt Cancer Center, FL 33612, Tampa, FL, USA;113. Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie National Research Institute of Oncology, 00-001, Warsaw, Poland;114. Department of Pathology, Brigham and Women’s Hospital, Harvard Medical School, MA 02215, Boston, MA, USA;115. Department of Surgery, The Royal Marsden Hospital and The Institute of Cancer Research, SW3 6JJ, London, the United Kingdom of Great Britain and Northern Ireland;1p. Garvan Institute of Medical Research, NSW 2010, Sydney, Australia;1q. Department of Research, Evaluative Epidemiology Unit, INT, 20133 Milan, Italy;1r. Sylvester Comprehensive Cancer Center, University of Miami, 33136 Miami, FL, USA;1s. Department of Medical Oncology, Netherlands Cancer Institute, 1066 CX, Amsterdam, The Netherlands;1t. Department of Surgical Oncology, the Netherlands Cancer Institute, 1066 CX, Amsterdam, The Netherlands;1u. Department of Hematology and Oncology, Fox Chase Cancer Center, 19111 Philadelphia, PA, USA;1v. Department of Medical Oncology, University of Colorado Cancer Center, 80045 Aurora, CO, USA;1w. Unit of Clinical Epidemiology and Trial Organization, Department of Applied Research and Technological Development, Fondazione IRCCS Istituto Nazionale Tumori, 20133 Milan, Italy;1. Urology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy;2. Università degli Studi di Milano, Milan, Italy;3. Università degli Studi di Padova, Italy;4. Radiology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy;5. Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy;6. Pathology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy;7. Testis surgery Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy;1. Department of Surgery - ASST Sette Laghi, Varese, Italy;2. Department of Surgery, Patel Hospital, Karachi, Pakistan;3. European School of Soft Tissue Sarcoma Surgery, ESSO, Brussels, Belgium;4. Sarcoma Service, Department of Surgery, IRCCS Fondazione Istituto Nazionale dei Tumori di Milano, Milan, Italy;5. Midlands Abdominal and Retroperitoneal Sarcoma Unit (MARSU), Queen Elizabeth Hospital Birmingham, UK;6. Clinical Epidemiology and Trial Organization, Department of Applied Research and Technological Development, Fondazione IRCCS Istituto Nazionale dei Tumori di Milano, Milan, Italy;7. Department of Orthopedic Surgery, ASST Monza Ospedale San Gerardo, Italy;1. Department of Radiology, Holy Family Hospital, New Delhi, India;2. Department of Orthopedics, Southport and Ormskirk, Southport, UK;3. Department of Orthopedics, Indraprastha Apollo Hospital, New Delhi, India;4. Department of Spinal Surgery, Royal Orthopaedic Hospital, Birmingham, UK;5. Department of Musculoskeletal Radiology, McMaster University, Hamilton, Canada;6. Department of Musculoskeletal Radiology, Royal Orthopaedic Hospital, Birmingham, UK;1. Oncology Service, The Royal Orthopaedic Hospital NHS Foundation Trust, Birmingham, UK;2. Department of Orthopaedic Surgery, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama, Japan;1. Gustave Roussy, Sarcoma Group, Division of International Patients Care, Villejuif, France;2. Department of Hematology-Oncology, Faculty of Medicine, Saint Joseph University, Beirut, Lebanon;3. Department of Pathology, Hotel-Dieu de France, Faculty of Medicine, Saint Joseph University, Beirut, Lebanon;4. Gustave Roussy, Sarcoma Group, Division of Medical Oncology, Villejuif, France;5. Bicêtre Teaching Hospital, AP-HP, Université Paris Saclay, Department of Orthopaedic Surgery, Le Kremlin-Bicêtre, France;6. Gustave Roussy, Sarcoma Group, Department of Ambulatory Cancer Care, Villejuif, France
Abstract:IntroductionHaemorrhagic soft-tissue sarcomas (HSTS) are characterised by aggressive local growth and highly metastatic behaviour. We aimed to describe oncological outcomes and prognostic factors.Materials and methodsRetrospective review including 64 patients treated with palliation (n = 7), with limb salvage surgery (LSS) (n = 9), with neoadjuvant radiotherapy (RT) + LSS (n = 12), with LSS + adjuvant RT (n = 30) or amputation (n = 6). Kaplan-Meier survival analysis estimated overall survival (OS), metastasis-free survival (MFS) and local recurrence-free survival (LRFS). After uni- and multivariate analysis, prognostic factors affecting OS, MFS and LRFS were identified.ResultsMedian age was 67 years (IQR 23 years) with median follow-up of 11 months (IQR 28 months). All cases were high grade. Eight (13%) had pulmonary metastases at presentation and another 40 (63%) developed metastases after median 9 months (IQR 19 months). Median OS was 12 months (IQR 38 months), and estimated OS after two-years was 15.9% and 52.9% for patients with and without metastatic disease at presentation, respectively. Improved OS was associated with negative resection margins (p = 0.031), RT (p = 0.045), neoadjuvant RT (versus adjuvant RT, p = 0.044) and amputation (versus LSS, p < 0.001). MFS was 35.1% after two-years. LR occurred in 18 of 51 (35.3%) patients with surgically treated localised disease. LRFS was 63.4% after two-years and significantly affected by a negative margin (p = 0.042) and RT (p = 0.001).ConclusionHaemorrhagic soft-tissue sarcomas should be excised, either with amputation or LSS with a clear resection margin. If LSS is attempted, neoadjuvant RT reduces the risk of tumour spillage and early LR, enhances the feasibility of achieving clear resection margins, and offers superior overall survival compared to adjuvant RT.
Keywords:Soft tissue neoplasms  Haemorrhage  Radiotherapy  Surgical procedures  Survival rate  Prognosis
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