Pleomorphic sarcomas or spindle cell sarcomas of the bone are rare malignant tumors that affect the adult. Fracture in the affected bone is the most frequent first symptom. Multimodal treatment is similar to the one for osteosarcoma, but the benefit is less evidence-based, due to the rarity of this sarcoma subtype. The present paper is a case report of a 73-year-old patient, who presented with a comminuted fracture of the distal third of the femur after a fall on ice. Differential diagnosis of bone metastasis was made. After the histopathological confirmation, the surgical team decided to amputate, with prior patient consent. The tumor was staged pT1NxMx, R0. Immunohistochemical studies confirmed the histopathological diagnosis. A lytic lesion in the stump bone appeared on post-operative MRI and was interpreted as skip metastasis. No other metastatic sites were detected. The multidisciplinary team decided for adjuvant chemotherapy (3 courses) and then radiotherapy. He was unable to receive the total planned dose of radiotherapy due to local toxicity. Even so, he is free of recurrence on long-term follow-up.
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