Meningeal melanocytoma of the brain and oculodermal melanocytosis (nevus of Ota): case report and literature review.

BACKGROUND

Meningeal melanocytomas are rare benign pigmented tumors of meningeal melanocytes. Nevus of Ota is a melanocytic pigmentation of the sclera and the skin adjacent to the eye. We report a rare case of parietal convexity meningeal melanocytoma associated with ipsilateral mucocutaneous melanosis and review the literature.

METHODS

A 17-year-old man presented with headache and blindness. There was left scalp and facial mucocutaneous melanosis. During surgery, speckled hyperpigmentation was seen in all layers of the scalp, bone, dura, and even ependyma. Pathologic examination of the tumor and scalp consisted of light microscopy and immunohistochemistry. In a literature review, the demographics (age, sex), clinical characteristics (site of lesion, duration of symptoms), therapeutic options (surgical excision, radiotherapy), and prognosis (recurrence, death, death related to the disease, and follow-up) were analyzed. For statistical analysis we used the independent sample t-test and chi(2) tests.

RESULTS

Ninety-five cases (45 intracranial and 50 spinal) of meningeal melanocytoma have been reported. The median age of patients with intracranial lesions (40 years) was significantly lower than that of patients with spinal lesions (49 years) (p = 0.016). Of the 95 patients, 57.9% were female. In the nervous system, melanocytes are more common in the infratentorial and cervical areas, but more than half of the tumors have been located in the supratentorial region and the thoracic spine. In 46 months median follow-up, tumor recurrence rate and tumor related death rate were 26.3% and 10.5%, respectively. These were not statistically significant for different therapeutic modalities. There was a trend toward shorter survival for patients who underwent subtotal tumor resection without radiotherapy compared with other groups.

CONCLUSIONS

The prognostic criteria, differential diagnosis, and the embryological aspects of meningeal melanocytoma are discussed with a review of the related literature.