Orbital apex leiomyoma with intracranial extension

BACKGROUND Leiomyoma is a rare, benign smooth muscle tumor of the orbit. It occasional ly shows some histologic resemblance to other common tumors of the orbit li ke neurofibroma and schwannoma. Its location at the orbital apex is uncommo n and only one case with intracranial extension has been reported so far. CASE DESCRIPTION A nine-year-old boy presented to us with left orbital pain. Four years earl ier he had undergone partial excision, elsewhere, of a tumor at the orbital apex, which was reported as a schwannoma. The computed tomography (CT) sca n showed regrowth of the tumor with intracranial extension. The tumor was t otally resected by an intracranial route. One year postoperatively there wa s no recurrence of the tumor. CONCLUSIONS Leiomyoma of the orbit, though a benign tumor, does show regrowth after par tial excision. Total excision, including any intracranial component, is adv ised. The cases reported so far are reviewed and the histopathology and pos sible etiopathogenesis of this tumor are discussed. (C) 2000 by Elsevier Sc ience Inc.