Abstract

We present a case of a 40-year-old man with secondary fibrosarcoma of bone, arising from a non-ossifying fibroma. He subsequently developed metastatic disease that responded to four successive chemotherapy courses, the last three using the same dose/schedule of single agent ifosfamide. Eventual rapid progression of a huge intra-abdominal mass was associated with the syndrome of extrapancreatic tumour hypoglycemia (EPTH). The clinicopathological behaviour of fibrosarcoma of bone, and the mechanism of EPTH are discussed.