Treatment strategies and outcomes of primary Myxofibrosarcomas in a large patients cohort |
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Affiliation: | 1. Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy;2. Department of Cancer Medicine, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy;3. Department Radiation Therapy, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy;4. Department of Radiology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy;5. Department of Pathology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy;6. Department of Clinical Epidemiology and Trial Organization, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy;1. Division of Gastrointestinal Surgery, Department of Surgery, Graduate School of Medicine, Kobe University, 7-5-2, Kusunoki-cho, Chuo-ku, Kobe, Hyogo, 650-0017, Japan;2. Division of Minimally Invasive Surgery, Department of Surgery, Graduate School of Medicine, Kobe University, 7-5-2, Kusunoki-cho, Chuo-ku, Kobe, Hyogo, 650-0017, Japan;3. Department of Gastroenterological Surgery, Hyogo Cancer Center, 13-70, Kitaoji-cho, Akashi, Hyogo, 673-8558, Japan;1. Department of Surgery, Isala Hospital, Dr. van Heesweg 2, Zwolle, the Netherlands;2. Department of Plastic Surgery, OLVG Hospital, Oosterpark 9, Amsterdam, the Netherlands;3. Department of Surgery, Gelre Hospital, Albert Schweitzerlaan 31, Apeldoorn, the Netherlands;4. Department of Plastic Surgery, Isala Hospital, Dr. van Heesweg 2, Zwolle, the Netherlands;1. Department of General Surgery (Hepatobiliary Surgery), The Affiliated Hospital of Southwest Medical University, Luzhou, 646000, China;2. Academician (Expert) Workstation of Sichuan Province, Luzhou, 646000, China;3. Nuclear Medicine and Molecular Imaging Key Laboratory of Sichuan Province, Luzhou, 646000, China;4. Department of General Surgery (Thyroid Surgery), The Affiliated Hospital of Southwest Medical University, Luzhou, 646000, China;5. Cardiovascular and Metabolic Diseases Key Laboratory of Luzhou, China;1. Department of Gastroenterology, The Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, 310009, Zhejiang, China;2. Department of Gastroenterology, Lishui City People''s Hospital, Lishui, 323000, Zhejiang, China |
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Abstract: | Backgroundthis study analysed primary myxofibrosarcoma (MFS) to investigate patient outcomes focusing on histopathologic margins and perioperative treatments.Patients and methodsdata from consecutive patients affected by primary and localized MFS of the extremities or trunk wall who underwent surgery (2002–2017) were analysed. Local recurrence (LR), amputation rate, incidence of distant metastasis (DM), and overall survival (OS) were studied.ResultsOf 293 included patients, 52 (17%) patients received perioperative treatments and 54 (18%) had positive microscopic histopathologic margins (R1). Median follow-up was 80 months (IQR, 49–109). 5-yr CCI of LR was 0.12 (SE: 0.02). Status of histopathologic margins (P < 0.001), tumour malignancy grade (P = 0.018) and size (P = 0023) were independent prognostic factor for LR. Nine amputations (amputation rate: 3%) were performed (N = 1 for primary tumour; N = 8 for LR). Larger tumour size (P = 0.015) and higher grade (P = 0.025) were independent prognostic factor for DM. 5-year OS was 0.84 (95%CI 0.79–0.88). Patient age (P = 0.008), tumour size (P = 0.013) and malignancy grade (P = 0.018) were independently associated to OS. In the subgroup of patients who had a re-excision for a primary MFS (N = 116, 40%), the presence of residual disease was not associated with LR, DM, or OS.Conclusionin this study 5-year LR, DM and OS were 12%, 17%, and 84%, respectively. One in six patients had a positive surgical margin, which was a prognostic factor for LR, while DM and OS were predicted by tumour grade and size. Findings from this large patient cohort may set benchmarks for investigating new treatment options for MFS. |
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Keywords: | Sarcoma Myxofibrosarcoma Local recurrence Survival Chemotherapy Radiotherapy |
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