Orbital lipofibromatosis in a child: a case report.

PURPOSE: Lipofibromatosis is a rare, benign, soft tissue tumor that typically involves extremities particularly in children. We report a young girl with lipofibromatosis involving the superotemporal quadrant of the left orbit. CASE REPORT: An 8-year-old girl presented with a history of a gradually expanding mass in the superotemporal quadrant of the left orbit over several years. Orbital imaging showed a soft tissue mass superolateral to the globe with possible infiltration into the lacrimal gland and lateral rectus muscle. The lesion was excised preserving adjacent structures. Histopathological examination revealed that the lesion consisted of adipose tissue with fibroblastic elements, being consistent with a diagnosis of lipofibromatosis. No recurrence of the lesion was seen in 18 months follow-up. COMMENT: Orbital involvement of lipofibromatosis as reported here is indeed a very rare entity. Diagnosis can be confirmed by histopathological analysis. As the lesion has a tendency to infiltrate into surrounding structures, surgical excision without hampering important structures is a treatment of choice.