Malignant fibrous histiocytoma developing in irradiated sacral chordoma

Abstract

Malignant fibrous histiocytoma (MFH), arising at the site of a sacral chordoma 8 years after massive radiotherapy, is described. Initially, the patient received 7000 rad to the sacral area and, on recurrence, 5 years later, an additional 4000 rad. Two years later, a sacral mass was noted again. Biopsy then revealed MFH; chest x-ray showed multiple lung metastases. A combination chemotherapy, consisting of cyclophosphamide, vincristine, adriamycin (doxorubicin), and DTIC, resulted in a 6 month partial response. Subsequently, the patient died because of progressive metastatic disease. At autopsy, 8 years after diagnosis, both the sacral lesion and the lung metastases proved to be MFH, and no residual chordoma was found.