A periosteal osteosarcoma arose in the first phalanx of the fourth finger of a 32-year-old woman's right hand. She had no predisposing factors for this cancer. The tumor was initially locally resected, but as recurrences developed, the finger and later the hand were amputated. Despite therapy with doxorubicin and cisplatin, distant metastases occurred and ultimately caused the patient's death. Primary conventional osteosarcomas of the short tubular bones of the hands and feet are rare, but surface lesions are rarer. This case constitutes the first reported periosteal osteosarcoma to arise in a phalanx.
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