Pediatric meningeal melanomatosis with the presence of atypical melanocytic cells in the cerebrospinal fluid

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  • NAMBU Junichi
    Department of Diagnostic Pathology, Okinawa Prefectural Nanbu Medical Center & Children’s Medical Center
  • NAKAZATO Iwao
    Department of Diagnostic Pathology, Okinawa Prefectural Nanbu Medical Center & Children’s Medical Center
  • HIGA Yoshihiro
    Department of Clinical Laboratory, Okinawa Prefectural Nanbu Medical Center & Children’s Medical Center

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Other Title
  • 髄液中にメラニン色素を含有する異型細胞を認めた小児の髄膜黒色腫症の 1 例
  • ―A case report―

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Abstract

<p>Background : Meningeal melanomatosis is an extremely rarely encountered condition in pediatric practice. Herein, we report a case of pediatric meningeal melanomatosis in which atypical melanocytic cells were detected in the cerebrospinal fluid.</p><p>Case : A 4-year-old boy was admitted to our hospital with a history of dysstasia, vomiting and altered consciousness, and was suspected as having encephalitis. After admission, the findings of MRI suggested the possibility of leptomeningeal melanosis, and cerebrospinal fluid (CSF) cytology and brain biopsy were performed. CSF cytology showed atypical cells with intracytoplasmic melanin, and brain biopsy revealed atypical cells containing melanin proliferating under the arachnoid membrane and invading the cerebral parenchyma. Finally, the diagnosis of meningeal melanomatosis was established.</p><p>Conclusion : Meningeal melanomatosis is extremely rare. Neither the symptoms nor the imaging findings are specific, and it is often difficult to distinguish this condition from other diseases of the nervous system, like meningitis. CSF cytology has an important role in the diagnosis.</p>

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