Neurocutaneous melanosis

Common Name(s)

Neurocutaneous melanosis

Neurocutaneous melanosis (NCM) is a rare, non-inherited condition of the central nervous system. It is characterized by melanocytic nevi in both the skin and the brain. Two-thirds of people with NCM have giant congenital melanocytic nevi, and the remaining one-third have numerous lesions but no giant lesions. Most patients present with neurological features early in life, which can be secondary to intracranial hemorrhages (bleeding in the brain), impairment of cerebrospinal fluid circulation (fluid around the brain and spinal cord), and/or malignant transformation of the melanocytes.

The prognosis of patients with symptomatic neurocutaneous melanosis is extremely poor, even in the absence of malignancy. Chemotherapy has been ineffective in the few patients in whom it has been tried.

 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Neurocutaneous melanosis" for support, advocacy or research.

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Nevus Network

Nevus Network Goals are to provide a network of support for those with congenital nevi and/or NCM.

Last Updated: 24 Nov 2009

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General Support Organizations

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Neurocutaneous melanosis" for support, advocacy or research.

Logo
Nevus Network

Nevus Network Goals are to provide a network of support for those with congenital nevi and/or NCM.

http://www.nevusnetwork.org

Last Updated: 24 Nov 2009

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General Support Organizations

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Neurocutaneous melanosis" returned 16 free, full-text research articles on human participants. First 3 results:

A case of neurocutaneous melanosis and neuroimaging findings.
 

Author(s): Rahsan Gocmen, Ezgi Guler, Elif Acar Arslan

Journal:

 

Neurocutaneous melanosis is a rare congenital disorder which presents with congenital cutaneous nevi and involvement of the central nervous system. We herein present a rare case of a 2-year-old girl who had central nervous system melanosis and giant congenital melanocytic nevi. Magnetic ...

Last Updated: 30 Apr 2015

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Oncogenic codon 13 NRAS mutation in a primary mesenchymal brain neoplasm and nevus of a child with neurocutaneous melanosis.
 

Author(s): Francis Shih, Stephen Yip, Patrick J McDonald, Albert E Chudley, Marc R Del Bigio

Journal:

 

A 28-month female with a clinical diagnosis of neurocutaneous melanosis and numerous intracranial abnormalities (including a right choroid plexus tumor and left hemimegalencephaly) presented with a rapidly expanding tumor in the left occipital cerebrum. Microscopic examination of ...

Last Updated: 25 Feb 2015

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Intramedullary and retroperitoneal melanocytic tumor associated with congenital blue nevus and nevus flammeus: an uncommon combination of neurocutaneous melanosis and phacomatosis pigmentovascularis--case report.
 

Author(s): Ryu Kurokawa, Phyo Kim, Toshiki Kawamoto, Hadzki Matsuda, Shujiro Hayashi, Soji Yamazaki, Atsushi Hatamochi, Shozo Mori, Mitsugi Shimoda, Keiichi Kubota

Journal: Neurol. Med. Chir. (Tokyo). 2013 ;53(10):730-4.

 

Neurocutaneous melanosis (NCM) is a rare condition characterized by central nervous system melanocytic tumors associated with congenital melanocytic nevi. Phacomatosis pigmentovascularis (PPV) is an association of vascular nevus with pigmentary nevus. Aberrant maturation of neural ...

Last Updated: 28 Oct 2013

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Neurocutaneous melanosis" returned 2 free, full-text review articles on human participants. First 3 results:

[Neurocutaneous melanosis. Case report and literature review].
 

Author(s): María Marta Buján, Andrea Bettina Cervini, Susana Pérsico, Adrián Martín Pierini

Journal: Arch Argent Pediatr. 2011 Dec;109(6):109-12.

 

Neurocutaneous melanosis is characterized by an increased number of melanocytes and melanin deposit in central nervous system associated with giant melanocytic congenital nevi. Patients with multiple satellite nevi or giant cutaneous melanocytic nevus in a midline location (overlying ...

Last Updated: 10 Jan 2012

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Neurocutaneous melanosis: follow-up and literature review.
 

Author(s): M A A Scattolin, J Lin, M M Peruchi, A J Rocha, M R Masruha, L C P Vilanova

Journal: J Neuroradiol. 2011 Dec;38(5):313-8.

 

Neurocutaneous melanosis is a rare, congenital, non-inherited syndrome characterized by numerous and/or large congenital nevi with intracranial leptomeningeal melanocytosis. This report describes two patients, presenting with a giant congenital nevus involving a major portion of the ...

Last Updated: 2 Dec 2011

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Symptoms, Diagnosis, and Treatment

There are currently no related results available in GeneReviews.

 
 
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Clinical Trial Information This information is provided by ClinicalTrials.gov

Pancreatic Cancer Screening of High-Risk Individuals in Arkansas
 

Status: Recruiting

Condition Summary: Pancreatic Neoplasms; Peutz-Jegher's Syndrome; BRCA1 Gene Mutation; BRCA2 Gene Mutation; Ataxia Telangiectasia; Familial Atypical Mole-Malignant Melanoma Syndrome; Colorectal Neoplasms, Hereditary Nonpolyposis; Hereditary Pancreatitis

 

Last Updated: 12 Jan 2016

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