Neurocutaneous melanosis

Harper, C. G.; Thomas, D. G. T.

doi:10.1136/jnnp.37.6.760

Cited by 12 publications

(3 citation statements)

References 1 publication

(1 reference statement)

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“…NCM was first described by Rokitansky in 1861 (8), and at least 82 additional cases associated with CMN have been reported in the English language literature (1,5,9–63). The criteria for the diagnosis of NCM were redefined by Kadonga and Frieden in 1991 (5), and include large (greater than 20 cm in an adult; greater than 9 cm on the head or greater than 6 cm on the body in an infant) and/or multiple (more than two) CMN in association with the proliferation of melanocytes within the central nervous system (CNS).…”

Section: Discussionmentioning

confidence: 99%

Cerebral Mass Due to Neurocutaneous Melanosis: Eight Years Later

Schaffer

McNiff

Bolognia

2001

Pediatric Dermatology

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Neurocutaneous melanosis (NCM) is associated most commonly with giant congenital melanocytic nevi (CMN), in particular those on the scalp or in a posterior axial location that are accompanied by satellite congenital nevi. It also can occur in patients with multiple medium-sized CMN. In general, the prognosis of those with symptomatic NCM is poor, even in the absence of malignancy, while the prognosis of those with asymptomatic NCM detected via screening varies and is more difficult to predict. Herein we report an asymptomatic patient with a giant CMN and multiple satellite nevi who had a screening magnetic resonance imaging (MRI) study at age 5 months that showed a rounded area of increased signal in the right temporal lobe on T1-weighted images, suggestive of parenchymal melanosis. This melanotic mass was resected at age 10 months, and histologic examination of the surgical specimen showed prominent perivascular collections of benign, pigment-containing melanocytes within cerebral tissue. The patient remains healthy 8 years later. His excellent long-term outcome and other reports of NCM with localized central nervous system (CNS) involvement apparent on MRI may have implications for management, including early imaging of patients with high-risk CMN and potential surgical intervention for NCM.

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Section: Discussionmentioning

confidence: 99%

Cerebral Mass Due to Neurocutaneous Melanosis: Eight Years Later

Schaffer

McNiff

Bolognia

2001

Pediatric Dermatology

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“…Neurocutaneous melanoblastosis has been reported to present with a variety of neurological complications in-cluding seizures in almost all patients, psychiatric disturbances, increased intracranial pressure with signs of space-occupying lesions, spinal cord involvement and hydrocephalus [1,[4][5][6][7][8][9][10]. The dense perivascular cellular infiltration in the leptomeninges acts as a barrier to complete CSF circulation and absorption [7].…”

Section: Discussionmentioning

confidence: 99%

“…Affected cerebral areas are the brain stem and cerebellum [1,3,4]. Clinically, hydrocephalus and seizures are the most common forms of presentation [5][6][7][8]. We report an unusual case of neurocutaneous melanoblastosis presenting with cerebral venous sinus thrombosis, in which the diagnosis was suspected on the basis of clinical, magnetic resonance imaging (MRI) and cerebospinal fluid (CSF) findings and finally confirmed by histopathology.…”

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confidence: 97%

Neurocutaneous melanoblastosis associated with cerebral sinus venous thrombosis

Schnittger

Kolbe

Fedorowski

et al. 1999

Journal of Neurology

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Meningeal melanocytoma of Meckel's cave associated with ipsilateral Ota's nevus case report

Botticelli¹,

Villani²,

Angiari³

et al. 1983

Cancer

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A case of meningeal melanocytoma of the left Meckel's cave associated with ipsilateral Ota's nevus in a 43‐year‐old woman, was studied by light and electron microscopy. The cells of the tumor were characterized by the presence of dendritic cytoplasmic processes, melanosomes and premelanosomes; hence, they were deemed as neoplastic melanocytes. Moreover, the tumor was lacking in histologic and ultrastructural features of pigmented meningioma, melanotic Schwannoma and primary meningeal melanoma. The prolonged clinical course was different from primary and metastatic malignant melanomas of the meninges. The best treatment appears to be radical excision, when possible; otherwise, the local or partial enucleation followed by radiation therapy has been found to be the best curative to date. On the whole, meningeal melanocytoma cannot be considered as entirely benign, given its morphologic patterns that resemble those of uveal melanoma, and its potential for recurrence. The association of this tumor with Ota's nevus is referred to as having a common origin from an arrested migration of melanoblasts at different stages.

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Neurocutaneous melanosis

Cited by 12 publications

References 1 publication

Cerebral Mass Due to Neurocutaneous Melanosis: Eight Years Later

Cerebral Mass Due to Neurocutaneous Melanosis: Eight Years Later

Neurocutaneous melanoblastosis associated with cerebral sinus venous thrombosis

Meningeal melanocytoma of Meckel's cave associated with ipsilateral Ota's nevus case report

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