Neurocutaneous melanosis

A disorder characterized by the presence of melanin-producing cells within the brain parenchyma or leptomeninges, which may cause neurologic signs and symptoms, such as SEIZURES; HYDROCEPHALUS; ARACHNOID CYSTS; BRAIN NEOPLASMS, and SYRINGOMYELIA. Occurs as a rare but severe manifestation of congenital MELANOCYTIC NEVUS syndrome (CMNS) (OMIM: 137550). Some patients with neurocutaneous melanosis or CMNS may develop MELANOMA. Somatic mutations in the NRAS gene have been identified. OMIM: 249400

Also Known As:

Networked: 28 relevant articles (0 outcomes, 1 trials/studies)

Disease Context: Research Results

Related Diseases

1.	Melanoma (Melanoma, Malignant)
2.	Nevus (Nevi)
3.	Pigmented Nevus (Melanocytic Nevus)
4.	Neurocutaneous Syndromes (Phakomatoses)
5.	Hydrocephalus (Hydrocephaly)

Experts

1.	Arshad, Saleha: 1 article (04/2021)
2.	Azizi, Amedeo A: 1 article (04/2021)
3.	Baumgartner, Alicia: 1 article (04/2021)
4.	Berger, Walter: 1 article (04/2021)
5.	Bernkopf, Marie: 1 article (04/2021)
6.	Boztug, Heidrun: 1 article (04/2021)
7.	Dorfer, Christian: 1 article (04/2021)
8.	Engstler, Gernot: 1 article (04/2021)
9.	Gojo, Johannes: 1 article (04/2021)
10.	Haberler, Christine: 1 article (04/2021)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Neurocutaneous melanosis:

1.	Melanins (Melanin)IBA 12/01/2008 - "The vascular endothelial cell is a new origin of melanin pigment on cynomolgus macaque with neurocutaneous melanosis." 08/01/2005 - "Neurocutaneous melanosis is a rare congenital syndrome characterized by proliferation of melanin-producing cells in the skin and leptomeninges. " 10/01/1994 - "Neurocutaneous melanosis presenting as an intradural mass of the cervical canal: magnetic resonance features and the presence of melanin as a clue to diagnosis: case report." 09/01/1994 - "Magnetic resonance (MR) imaging is sensitive in detecting melanin; several recent reports have described the MR findings in neurocutaneous melanosis. " 06/01/1994 - "Neurocutaneous melanosis is a rare congenital syndrome characterised by large or numerous congenital pigmented naevi and excessive proliferation of melanin-containing cells in the leptomeninges. "
2.	Codon (Codons)IBA 09/01/2013 - "Multiple congenital melanocytic nevi and neurocutaneous melanosis are caused by postzygotic mutations in codon 61 of NRAS." 10/21/2014 - "Oncogenic codon 13 NRAS mutation in a primary mesenchymal brain neoplasm and nevus of a child with neurocutaneous melanosis."
3.	Fluorodeoxyglucose F18 (Fludeoxyglucose F 18)FDA Link 04/01/2020 - "Serial 18F-FDG PET/CT findings in a patient with neurocutaneous melanosis."
4.	Hepatocyte Growth Factor (Growth Factor, Hepatocyte)IBA 03/01/2001 - "Transgenic mice overexpressing hepatocyte growth factor/scatter factor (HGF/SF) demonstrate extensive pigmented nevi in both skin and leptomeninges of the central nervous system resembling human neurocutaneous melanosis. "
5.	Vanilmandelic Acid (Vanillylmandelic Acid)IBA 01/11/2015 - "Brain MRI revealed neurocutaneous melanosis (NCM) in 7/24 children (29.2%), symptomatic in 1. Among urine catecholamines levels from 20 patients (33 samples), dopamine concentration was elevated in 28/33, noradrenaline in 15, adrenaline in 11, and vanillylmandelic acid in 4. In 6 NCM children, all catecholamines concentrations were higher than in patients without NCM (statistically insignificant). "
6.	PlasticsIBA 01/01/1995 - "Plastic surgeons who treat congenital giant nevi should be aware of the neurocutaneous melanosis syndrome and its variants. "
7.	Norepinephrine (Noradrenaline)FDA LinkGeneric 01/11/2015 - "Brain MRI revealed neurocutaneous melanosis (NCM) in 7/24 children (29.2%), symptomatic in 1. Among urine catecholamines levels from 20 patients (33 samples), dopamine concentration was elevated in 28/33, noradrenaline in 15, adrenaline in 11, and vanillylmandelic acid in 4. In 6 NCM children, all catecholamines concentrations were higher than in patients without NCM (statistically insignificant). "
8.	S100 Proteins (S 100 Protein)IBA 03/01/1987 - "Cells from neurocutaneous melanosis contained extremely large amounts of S-100 protein. "
9.	Methionine (L-Methionine)FDA Link 10/01/2011 - "Neurocutaneous melanosis: assessment on F-18 FDG and [11C]-methionine PET/CT and MRI."
10.	Epinephrine (Adrenaline)FDA LinkGeneric 01/11/2015 - "Brain MRI revealed neurocutaneous melanosis (NCM) in 7/24 children (29.2%), symptomatic in 1. Among urine catecholamines levels from 20 patients (33 samples), dopamine concentration was elevated in 28/33, noradrenaline in 15, adrenaline in 11, and vanillylmandelic acid in 4. In 6 NCM children, all catecholamines concentrations were higher than in patients without NCM (statistically insignificant). "

Therapies and Procedures

1.	Dermabrasion 07/01/2006 - "Future studies on CMN should report: (i) diameter, percentage of body surface, and localization of the CMN; (ii) percentage of naevus area removed by excision or subject to dermabrasion or other superficial treatments; (iii) mean and median age at entry into the study; (iv) mean and median follow-up time; (v) details on each melanoma case; (vi) standardized morbidity ratio of melanoma; and (vii) percentage of neurocutaneous melanosis."
2.	Ventriculoperitoneal Shunt 12/01/2007 - "Progressive brainstem compression in an infant with neurocutaneous melanosis and Dandy-Walker complex following ventriculoperitoneal shunt placement for hydrocephalus. "
3.	Therapeutics 01/01/2010 - "Rapid response to therapy of neurocutaneous melanosis with leptomeningeal melanoma."
4.	Lenses 08/01/1987 - "The authors reported the clinical course and the postmortem examination of a unique case of neurocutaneous melanosis with numerous anomalies and complications, which included congenital dislocation of lenses, hypogonadism, ectopia of prostatic duct, genuine phimose, retentio testis, psina bifida and neurogenic bladder. "
5.	Decompression 08/01/2016 - "A 5-year-old child with PLM without neurocutaneous melanosis presented with cystic enlargement of the brainstem surrounding cisterns and hydrocephalus requiring occipitoaxial decompression and endoscopic cystostomy. "