Neurocutaneous melanosis presenting with hydrocephalus and malignant transformation: case-based update

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Neurocutaneous melanosis with hydrocephalus: report of one case.

Neurocutaneous melanosis (NCM) is a rare nonfamilial syndrome and characterized by large or numerous congenital melanocytic nevi and excessive proliferation of melanin-containing cells in the leptomeninges. It is believed to be an embryonic neuroectodermal dysplasia. Patients with NCM may develop severe hydrocephalus and other neurological symptoms with extremely poor prognosis. We report an in...

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Neurocutaneous melanosis presenting with hydrocephalus. Case report and review of the literature.

Neurocutaneous melanosis (NCM) is a rare congenital neurocutaneous syndrome characterized by large or multiple congenital melanocytic nevi and benign or malignant melanocytic tumors of the leptomeninges. The authors report the case of a 5-month-old girl with congenital giant melanocytic nevi who presented with symptomatic hydrocephalus. A right frontal ventriculostomy was performed in the patie...

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Neurocutaneous melanosis presenting with intracranial amelanotic melanoma.

We describe imaging findings in a 2-year-old girl with neurocutaneous melanosis and malignant cerebral melanoma. Because the cerebral melanoma in this child was of the amelanotic type, high-signal intensity on unenhanced T1-weighted images was not present. The cutaneous lesions played a crucial role in establishing a correct (presumed) histopathologic diagnosis on the basis of the imaging findi...

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Neurocutaneous Melanosis with Malignant Leptomeningeal Melanoma

Neurocutaneous melanosis (NCM) is a rare congenital syndrome characterized by the presence of large or multiple congenital melanocytic nevi with either benign or malignant pigmented cell tumors of the leptomeninges. We report a case of NCM in a patient with previously robust health. No neurological deficit was noted in the patient. At the age of 21 years old, blurred vision initially appeared. ...

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Neurocutaneous Melanosis Presenting as Chronic Partial Epilepsy

BACKGROUND Neurocutaneous melanosis (NCM) is a rare neurocutaneous syndrome characterized by the presence of multiple congenital melanocytic nevi (CMN) and the proliferation of melanocytes in the central nervous system, usually involving the leptomeninges. Chronic partial epilepsy as a sole manifestation is rare in NCM. CASE REPORT A 32-year-old man suffering from chronic partial epilepsy pre...

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ژورنال

عنوان ژورنال: Child's Nervous System

سال: 2018

ISSN: 0256-7040,1433-0350

DOI: 10.1007/s00381-018-3851-5