Orbital apex leiomyoma with intracranial extension

Abstract

Background: Leiomyoma is a rare, benign smooth muscle tumor of the orbit. It occasionally shows some histologic resemblance to other common tumors of the orbit like neurofibroma and schwannoma. Its location at the orbital apex is uncommon 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 earlier he had undergone partial excision, elsewhere, of a tumor at the orbital apex, which was reported as a schwannoma. The computed tomography (CT) scan showed regrowth of the tumor with intracranial extension. The tumor was totally resected by an intracranial route. One year postoperatively there was no recurrence of the tumor. Conclusions: Leiomyoma of the orbit, though a benign tumor, does show regrowth after partial excision. Total excision, including any intracranial component, is advised. The cases reported so far are reviewed and the histopathology and possible etiopathogenesis of this tumor are discussed.