Aggressive management of orbital meningeal melanocytoma

Abstract

Meningeal melanocytomas are rare benign pigmented tumors of the central nervous system. They are predominant in the posterior fossa and spinal cord and frequently mistaken for melanomas, especially on frozen sections. Orbital presentation is rare. The natural history is poorly defined.

Orbital melanocytoma completely resected with conservative surgery in association with ipsilateral nevus of Ota: Report of a case and review of the literature

“…Intraocular melanocytomas can also occur, involving the iris, choroid, and ciliary body . Approximately 110 cases of meningeal melanocytomas have been reported in the literature, whereas orbital melanocytomas have been rarely reported …”

Orbital Melanocytoma

“…Limas and Tio in 1972 described the melanocytic origin of these tumors based on the ultrastructural findings of melanosomes and premelanosomes in the neoplastic cells in a heavily pigmented foramen magnum tumor and used the term meningeal melanocytoma. Some authors consider melanocytoma as a borderline tumor between cellular blue nevus and spindle melanoma of the uveal tract (de Tella et al, 2003;Ibanez et al, 1997). They are more common in females in the 5th decade and present as intracranial or intraspinal meningeal-based masses where the melanocytes are in highest concentration.…”

“…The intermediate grade tumors can show aggressive behavior with local invasion, which was present in this case. Such tumors are more likely to recur and need to be managed aggressively with a combination of surgery, radiation therapy, and chemotherapy (de Tella et al, 2003). Tumors that recur can transform to melanoma.…”

“…Intraocular melanocytomas have been described in iris, choroid, sclera, conjunctiva, and ciliary body. Orbital presentation is rarely reported in literature (de Tella et al, 2003).…”

“…The MIB-1 labeling index usually range from 1% to 4%. These tumors are capable of recurrence and clinical behavior is unpredictable (de Tella et al, 2003). Malignant transformation to melanoma with subsequent leptomeningeal spread are also likely to occur in melanocytomas at some point in their evolution (Rades et al, 2004;Roser et al, 2004), especially when the tumors are allowed to recur.…”