Elsevier

World Neurosurgery

Volume 136, April 2020, Pages 311-314
World Neurosurgery

Case Report
Brain Atrophy Associated with Primary Diffuse Meningeal Melanomatosis

https://doi.org/10.1016/j.wneu.2020.01.164Get rights and content

Background

Primary meningeal melanomatosis is a rare leptomeningeal tumor, and the diagnosis is challenging due to nonspecific clinical symptoms and radiologic findings.

Case Description

A 21-year-old man presented with recurrent seizure and impaired memory. Cranial magnetic resonance imaging showed obvious brain atrophy with bilateral extensive meningeal enhancement in the supratentorial region. Diffusion-weighted imaging and fluid-attenuated inversion recovery showed slightly hyperintensive signals in the cortex. Microscopic examination revealed invasion of pigment into the Virchow-Robin space and cortex. Immunohistochemical examination of biopsy samples showed that cells were immunopositive for HMB45 and S-100 and immunonegative for melan-A with a Ki-67–positive percentage of 3%. No obvious atypia or nuclear mitosis was observed. Pathohistologic results of biopsied meninges confirmed the diagnosis of diffuse meningeal melanomatosis. The disease was aggravated with the occurrence of brain atrophy, recurrent seizure, and declined higher cortical function.

Conclusions

This case report illustrates that brain atrophy in meningeal melanomatosis is associated with a progressive decline of higher cortical function.

Introduction

Meningeal melanomatosis is a rare leptomeningeal tumor of melanotic cells that is characterized by slow and diffuse growth.1 The diagnosis of meningeal melanomatosis is challenging because the clinical symptoms and signs, as well as radiologic findings, are nonspecific. Therefore additional information gained via microscopy and immunohistochemical analysis is important for the correct diagnosis of meningeal melanomatosis. Here we report a case of diffuse meningeal melanocytosis diagnosed based on the pathohistologic findings biopsy.

Section snippets

Case Report

A 21-year-old college student was admitted to our hospital after experiencing seizure and impaired memory for 5 months. In March 2018, he suddenly lost consciousness with tetanic seizure and was diagnosed with viral encephalitis according to normal results on cranial magnetic resonance imaging (MRI; Figure 1A and B) and lumbar puncture. Cranial MRI results showed that bilateral cerebral fissures were widened (see Figure 1C and D). His memory, learning ability, and academic performance continued

Discussion

Primary melanoma is rare, accounting for ≈0.06–0.1% of all central nervous system tumors.2 The diagnosis of primary melanoma is commonly made on the basis of cerebrospinal fluid cytology and/or pathologic examination of biopsied samples.3 Primary melanoma is commonly immunopositive for HMB-45 and S-1004 and has a Ki-67 percentage of ≤5%–10%, indicating low proliferation.5 In this case, black tissues with uniform distribution were observed after exposure of the dura mater during surgery. A rich

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Conflict of interest statement: The authors declare that the article content was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

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