Meningeal melanocytic tumor of the malar bone

Abstract

Meningeal melanocytoma is a central nervous system tumor. It is a rare and benign lesion originating from leptomeningeal melanocytes. Although usually a unifocal type, there are in the literature reports of multifocal cases and malignant transformations, with leptomeningeal dissemination. We present the clinical case of a male child, 7 years old, diagnosed with meningeal melanocytoma, which surrounded the right malar bone and clearly exceeded the bone limits to the temporal fossa. There was complete bone destruction and exophthalmus with eyeball compression. We opted to perform an extended excision of the tumor, after craniotomy, sacrificing the malar bone, orbital floor, and lateral orbital wall, using coronal, subciliar, and orbital roof approaches. The reconstruction used bone graft from calvaria duplication. During the immediate post-operatory period, the patient had a small ectropion, which disappeared within 8 days. The medium-term result was satisfactory. Meningeal melanocytoma is a rare and benign lesion; however, it can feature severe destruction, and has the potential of becoming malignant, in which case there is no therapeutic protocol. In the present case, we achieved satisfactory functional and aesthetic results, but the repercussions in growth and development of the child are uncertain. Level of Evidence: Level V, diagnostic study