Malignant fibrous histiocytoma and pleomorphic sarcoma in association with medullary bone infarcts

Abstract

A malignant fibrous histiocytoma arose in the proimal tibia of a 40-year-old man who had multiple, symmetrically distributed, medullary bone infarcts of unknown etiology, involving the distal femora and the tibiae. Despite amputation and chemotherapy, widespread metastases developed and death occurred 19 months after surgery. A polemorphic sarcoma, probably representing an anaplastic malignant fibrous histiocytoma, arose in association with a single medullary infarct in the proximal humerus of a 33-year-old woman. She remains well without evidence of disease five years after treatment by radical radiotherapy followed by shoulder disarticulation. Sarcoma arising in association with bone infarction is a rare entity. Sixteen cases reported in the medical literature, including our own, are reviewed. The sarcomas arose in the tibia in nine cases, the femur in six cases and the humerus in one case. The patients were usually older individuals and 13 of the 16 were male. All but two had multiple bone infarctions. Four of the patients had caisson disease, three had what is probably an hereditary bone dysplasia, one had sickle cell disease and eight had infarcts of unknown etiology. Most patients have had a rapidly fatal outcome. Thirteen of the sarcomas have been fibrosarcomas or malignant fibrous histiocytomas, both of which are rare primary bone tumors. Analysis of the published cases of bone infarction-related sarcomas suggests that the risk of developing a sarcoma is greatest in infarcts with large medullary components.