Aggressive management of orbital meningeal melanocytoma

• Published in: Acta neurochirurgica Vol. 145; no. 12; pp. 1121 - 1126
• Main Authors: de Tella, Jr, O I, Agner, C, Aguiar, P H, Herculano, M A, Prandini, M N, Stavile, J N
• Format: Journal Article
• Language: English
• Published: Austria Springer Nature B.V 01.12.2003
• Subjects: Adult; Antigens, Neoplasm; Antineoplastic Combined Chemotherapy Protocols - therapeutic use; Biomarkers, Tumor - analysis; Chemotherapy, Adjuvant; Combined Modality Therapy; Craniotomy; Diagnosis, Differential; Follow-Up Studies; Humans; Male; Melanoma-Specific Antigens; Meningeal Neoplasms - drug therapy; Meningeal Neoplasms - pathology; Meningeal Neoplasms - radiotherapy; Meningeal Neoplasms - surgery; Microsurgery; Neoplasm Proteins - analysis; Neoplasm Recurrence, Local - diagnosis; Nervous system; Nevus - drug therapy; Nevus - pathology; Nevus - radiotherapy; Nevus - surgery; Orbit - pathology; Orbit - surgery; Orbital Neoplasms - drug therapy; Orbital Neoplasms - pathology; Orbital Neoplasms - radiotherapy; Orbital Neoplasms - surgery; Postoperative Complications - diagnosis; Radiotherapy, Adjuvant; S100 Proteins - analysis; Tomography, X-Ray Computed
• ISSN: 0001-6268; 0942-0940
• DOI: 10.1007/s00701-003-0121-3

Meningeal melanocytoma generally occurs in the posterior fossa. Orbital manifestation is rarely encountered. A thirty-five year-old man presented with progressive proptosis of his right eye. Computed tomography (CT) and Magnetic Resonance Imaging (MRI) of the brain showed an expansive intraconal mass lesion occupying the superior orbital compartment, the entire orbital apex, and the optic foramen. Histological analysis and Immunohistochemical staining for S-100 and HMB-45 monoclonal antibodies confirmed melanocytoma. Microsurgical removal was accomplished through a fronto-orbital craniotomy. Chemotherapy and irradiation followed the initial intervention. The patient returned for follow up two years after surgery, complaining of headache and right visual loss. A subfrontal tumor with massive edema was found on follow up CT scan. 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.