• Journal of Korean Neurosurgical Society
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• v.50 no.5
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• pp.475-477
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• 2011

Neurocutaneous melanosis associated with Dandy-Walker malformation is a rare dysmorphogenesis that is associated with single or multiple giant pigmented cutaneous nevi and diffuse involvement of the central nervous system. In this article, we present a 2-month-old patient with neurocutaneous melanosis associated with Dandy-Walker malformation. In addition, we reviewed the literature and discussed the pathogenesis based on the preferred hypotheses.

• Journal of Korean Neurosurgical Society
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• v.29 no.3
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• pp.389-395
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• 2000

Arare case of primary malignant melanoma of central nervous system in 12-month-old infant with neuro-cutaneous melanosis is presented. Primary malignant melanomas in central nervous system are very rare in children, however, it is known that leptomeningeal melanosis is malignant in 40-50% with neurocutaneous melanosis. Spinal MRI, brain CT and MRI showed diffuse intradural extramedullary mass of entire spinal cord and enhancing mass in brain stem. CSF cytology revealed abnormal cells. Biopsy from leptomeninges of posterior fossa was compatible with malignant melanoma.

• Clinical and Experimental Pediatrics
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• v.49 no.2
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• pp.212-216
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• 2006

Neurocutaneous melanosis is a rare congenital syndrome characterized by the presence of large or multiple congenital melanocytic nevi and benign pigment cell tumors of the leptomeninges. Neurocutaneous melanosis is thought to represent an error in the morphogenesis of embryonal neuroectoderm. We experienced a neonate who presented with giant, dark colored pigmented nevi covering chest, abdomen, neck and arms, with satellite lesions. Magnetic resonance image showed a nodular hyperintense lesion in the amygdala of the right temporal lobe, and T1-weighted images showed hyperintensities in the adjacent leptomeninges. We report a rare case of neurocutaneous melanosis with a brief review of related literature.

• Journal of Korean Neurosurgical Society
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• v.56 no.1
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• pp.61-65
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• 2014

Neurocutaneous melanosis (NCM) is a rare congenital syndrome consisting of benign or malignant melanotic tumors of the central nervous system with large or numerous cutaneous melanocytic nevi. The Dandy-Walker complex (DWC) is characterized by an enlarged posterior fossa with high insertion of the tentorium, hypoplasia or aplasia of the cerebellar vermis, and cystic dilatation of the fourth ventricle. These each two conditions are rare, but NCM associated with DWC is even more rare. Most patients of NCM with DWC present neurological symptoms early in life such as intracranial hemorrhage, hydrocephalus, and malignant transformation of the melanocytes. We report a 14-year-old male patient who was finally diagnosed as NCM in association with DWC with extensive intracerebral and spinal cord involvement.