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Background: Granular cell tumors (GCTs) are rare neoplasms that may also involve the musculoskeletal system and peripheral nerves of the extremities. In these locations, their clinical presentation, management, and outcomes remain poorly characterized. Methods: A systematic review was conducted according to PRISMA guidelines. PubMed, MEDLINE, EMBASE, and Scopus were searched for articles published between 1975 and 2025 reporting GCTs arising from the musculoskeletal system or peripheral nerves, with available data on clinical presentation and treatment. Tumor location and size, symptoms, treatment modality, and oncological outcomes (recurrence or metastasis) at the latest follow-up were extracted. Results: Forty articles describing 67 cases were included (50 females, 17 males). Tumors showed benign (47) or atypical (2) behavior in 49 cases and malignant features in 18 cases. The mean largest tumor diameter was 44 mm, and malignant lesions were significantly larger than benign ones. Thirty-one lesions were located in the lower limbs, 25 in the upper limbs, and 11 had central musculoskeletal localizations. Swelling was the most common presenting symptom (92%), followed by pain (40%). Surgical excision was performed in all patients except one, who underwent primary amputation. Adjuvant chemotherapy or radiotherapy was sporadically used in malignant cases (two cases each). Among the malignant cases with reported oncological follow-up, 44% developed distant metastases, and one (5.6%) also experienced local recurrence. Only one benign GCT recurred (2%), whereas all other non-malignant lesions remained CDF (98%). Conclusions: Although rare, GCTs should be considered in the differential diagnosis of musculoskeletal soft-tissue tumors, given their potential for malignant behavior and metastatic spread.