Clinical presentation

These tumors occur mainly in the second decade of life. In a series of ten patients, the median age was 10 years, and the tumor was most commonly located in the extremities. In this series, all tumors were localized, and five of nine patients (for whom follow-up was available) were alive and in first remission.[151]

In another series of 17 pediatric patients with malignant fibrous histiocytoma (now classified as undifferentiated pleomorphic sarcoma), the median age at diagnosis was 5 years and the extremities were involved in eight cases.[152] All patients with metastatic disease died, and two patients experienced a clinical response to a doxorubicin-based regimen.

For more information about the treatment of malignant fibrous histiocytoma of bone, see Osteosarcoma and Undifferentiated Pleomorphic Sarcoma of Bone Treatment.