Elsevier

Surgical Neurology

Volume 59, Issue 3, March 2003, Pages 200-210
Surgical Neurology

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

https://doi.org/10.1016/S0090-3019(02)01052-2Get rights and content

Abstract

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 χ2 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.

Conclusion

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

Section snippets

Selection of cases

We have included only those cases described in sufficient detail to allow us to form independent conclusions. We agree with Winston et al [73] in excluding the following cases: the melanoblastic meningioma of Bailey and Bucy, which was probably a malignant melanoma as evidenced by the large number of pigmented lesions over the surface of the cerebral hemispheres and within the cerebellum, and by the presence of many mitotic figures. Several series include the cases mentioned by Russell and

History

A 17-year-old male Caucasian presented with headache and blindness in both eyes of 4 months’ duration. Visual loss was gradual and progressive, but of unknown duration. He had been incarcerated for 38 months. The patient’s medical history was significant only for left scalp and facial hyperpigmentation at least since early childhood.

Examination

The patient’s general medical examination was normal except for left scalp and facial cutaneous melanosis including left conjunctiva and sclera melanosis. He had

Results

To our knowledge, including the present case, 95 cases (45 intracranial and 50 spinal) of meningeal melanocytoma, have been reported to date (Table 1). The median age was 45 years (ranging 5 months–74 years). The median age of patients with intracranial lesions was 40 and for those with intraspinal lesions it was 49. It was significantly lower in patients with intracranial lesions (p = 0.016). Fifty-five of 95 patients (57.9%) were female. The median duration of symptoms before diagnosis was 29

Anatomic location

Pigmented cells are normally found in the leptomeninges, and according to Virchow, were first described by Valenti [10]. The electron microscopic studies of Limas and Tio demonstrated that the cell of origin of the so-called pigmented meningiomas is the melanocyte and not the meningothelial cell [38]; they proposed the term “meningeal melanocytoma” to describe these tumors.

Melanocytes are widely distributed throughout the leptomeninges and they occur in the highest concentration ventrolateral

Acknowledgements

The authors thank Drs Seyyed Mohammad Naser Hoseini and Mahnaz Banihashemi, dermatologists of the Khatam Hospital and Drs PV Kumar and Ahmad Monabbati, pathologists of Shiraz University and Elizabeth Tyler-Kabra, senior resident of neurosurgery at University of Pittsburgh for her careful review of manuscript.

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