A case of neurocutaneous melanosis of onset in hydrocephalus with rapid malignant transformation

DOI
  • Saito Ryozo
    Department of Neurosurgery, Faculty of Medicine, Yamagata University
  • Ito Miiko
    Department of Neurosurgery, Faculty of Medicine, Yamagata University
  • Mitobe Yuta
    Department of Neurosurgery, Faculty of Medicine, Yamagata University
  • Sonoda Yukihiko
    Department of Neurosurgery, Faculty of Medicine, Yamagata University

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  • 水頭症で発症し急速な悪性転化を来した神経皮膚黒色症の1例

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Abstract

<p>In patients with large congenital melanocytic nevi, approximately 2.5-5% of them have been reported to have melanocyte-proliferative lesions in the central nervous system called neurocutaneous melanosis (NCM). It has been regarded as a condition with a poor prognosis that developed intractable epilepsy and rapidly progressed communicated hydrocephalus and caused with high probability conversion to malignant melanoma. On the other hand, there were reports of cases in which malignant transformation was rare and the prognosis was better, so the concept of NCM was chaotic. It has become clear that NCM was classified into two types such as “leptomeningeal melanosis” and “parenchymal melanosis” depending on the location of melanosis in CNS.</p><p>We report a case of extremely poor prognosis with rapid malignant transformation after the first presentation of hydrocephalus, with a review of the literature.</p>

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