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-zDOI Listing

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