Background: Low-grade fibromyxoid sarcoma is a rare soft tissue tumor characterized by a benign histological appearance but with a high potential for recurrence and metastasis. First described by Evans in 1987, recurrence and metastasis can occur decades after the initial diagnosis, complicating long-term management.
Case Presentation: We report the case of an 83-year-old Jewish female patient diagnosed with low-grade fibromyxoid sarcoma in her right shoulder. The patient underwent wide resection with positive margins, followed by regular follow-ups. At 9 months postoperatively, she developed recurrence involving the right trapezius, thoracic vertebrae, ribs, and pleural cavity, a second local resection was performed. At 2 years post-surgery, the patient remains recurrence free.
Conclusion: Low-grade fibromyxoid sarcoma, though rare, presents a high risk for recurrence and metastasis. Long-term follow-up and vigilant monitoring are crucial, particularly in cases where positive surgical margins are involved. Surgery remains the cornerstone of treatment, but ongoing research into systemic therapies may offer new options for managing metastatic or recurrent low-grade fibromyxoid sarcoma in the future.
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http://dx.doi.org/10.1186/s13256-025-05038-z | DOI Listing |
J Med Case Rep
January 2025
Division of Orthopedic Surgery, Hillel Yaffe Medical Center, Ha-Shalom St, 3820302, Hadera, Israel.
Background: Low-grade fibromyxoid sarcoma is a rare soft tissue tumor characterized by a benign histological appearance but with a high potential for recurrence and metastasis. First described by Evans in 1987, recurrence and metastasis can occur decades after the initial diagnosis, complicating long-term management.
Case Presentation: We report the case of an 83-year-old Jewish female patient diagnosed with low-grade fibromyxoid sarcoma in her right shoulder.
Virchows Arch
December 2024
Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, 350 W. 11thStreet, Room 4086, Indianapolis, IN, 46202, USA.
Sclerosing epithelioid fibrosarcoma (SEF) was originally described as a peculiar variant of fibrosarcoma in 1995. Subsequent studies showed that conventional SEF was associated with both immunohistochemical expression of MUC4 and EWSR1/FUS gene rearrangements with CREB3L1 as the predominant fusion partner. Since then, a distinct group of fibrous tumors characterized by YAP1::KMT2A and KMT2A::YAP1 gene rearrangements and SEF-like morphology has been described.
View Article and Find Full Text PDFAm J Surg Pathol
January 2025
Department of Medical Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.
The aim of the study was to report the outcome of primary localized low-grade fibromyxoid sarcoma (LGFMS), sclerosing epithelioid fibrosarcoma (SEF), and hybrid LGFMS/SEF (H-LGFMS/SEF). Patients with primary localized LGFMS, SEF, or H-LGFMS/SEF, surgically treated with curative intent from January 2000 to September 2022, were enrolled from 14 countries and 27 institutions. Pathologic inclusion criteria were predefined by expert pathologists.
View Article and Find Full Text PDFArch Pathol Lab Med
October 2024
From the Department of Pathology, Instituto de Biomedicina de Sevilla, Hospital Universitario Virgen del Rocío, CSIC-Universidad de Sevilla, Seville, Spain (Salguero-Aranda, Perez, Vargas-Padilla, Beltrán-Povea, Delgado-Bellido, Marcilla, Civantos, de Álava, Díaz-Martín).
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