A rare case of solitary interhemispheric myofibroma with excellent outcome in a 20 month old boy is described. The clinicopathological features of this unusual condition are reviewed with emphasis on the CNS manifestations.

A case of congenital fibrosarcoma was first diagnosed by William and Schrum1 and was subsequently renamed congenital generalised fibromatosis by Stout in 19542 as a distinct form of juvenile fibromatosis characterised by tumour-like nodules involving the skin, soft tissues, bones, and viscera. Based on the ultrastructural and immunohistochemical features of the cell of origin and the occurrence of this condition in infants, as well as congenitally, it was renamed infantile myofibromatosis by Chung and Enzinger in 1981.3 This disorder is considered to represent a hamartomatous myofibroblastic proliferation, although laboratory evidence suggests that it may arise secondary to oestrogen stimulation in utero. Infantile myofibromatosis represents the most common fibrous tumour of infancy and may present with solitary or multicentric lesions. When visceral involvement is present, the multilesional form is termed “generalised”. Cases with familial incidence,3spontaneous regression,4 5 and fatal outcome3 6 have all been described. Poor outcome has generally been associated with extensive visceral involvement and …