MALIGNANT FIBROUS HISTIOCYTOMA - AN UNUSUAL PRESENTATION

Malignant fibrous histiocytoma (MFH) is the commonest type of adult soft tissue sarcoma (Weiss and Enzinger, 1978). Intracranial MFHs, however, are rare and unreported in children. We report a case of MFH presenting as a posterior fossa mass in a 9-year-old boy with homocystinuria (HCU). This is an unusual presentation and the computed tomography (CT) finding of localized skull vault destruction proved an important differentiating feature from primary posterior fossa tumours. Early diagnosis of MFH is important as primary wide excision of involved bone margins improves prognosis, irrespective of subsequent radiotherapy or adjuvant chemotherapy (Tracy et al, 1984).