Neurocutaneous Melanocytosis-Associated Hydrocephalus: The Memorial Sloan Kettering Experience from 2001 to 2022.

Publisher: John Wiley Country of Publication: United States NLM ID: 101186624 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1545-5017 (Electronic) Linking ISSN: 15455009 NLM ISO Abbreviation: Pediatr Blood Cancer Subsets: MEDLINE

Original Publication: Hoboken, N.J. : John Wiley, c 2004-

Hydrocephalus*/etiology ; Hydrocephalus*/pathology ; Melanoma*/pathology ; Melanoma*/complications ; Melanosis*/pathology ; Melanosis*/etiology ; Neurocutaneous Syndromes*/pathology ; Neurocutaneous Syndromes*/complications ; Ventriculoperitoneal Shunt*; Humans ; GTP Phosphohydrolases/genetics ; Membrane Proteins ; Prognosis ; Skin Neoplasms/pathology ; Skin Neoplasms/complications

We report two neurocutaneous melanocytosis (NCM) patients who required ventriculoperitoneal shunt placement and subsequently developed intraperitoneal melanoma. These patients with NCM are at an increased risk for developing NRAS-associated melanomas in the central nervous system, which in turn may lead to symptomatic hydrocephalus requiring cerebrospinal fluid diversion. Due to the rarity of NCM, current knowledge on disease progression and appropriate management is limited. Ongoing studies aiming to better understand this condition and inform its clinical management may help to identify risk factors for developing more severe complications.
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P30 CA008748 United States CA NCI NIH HHS; P30 CA008748 United States CA NCI NIH HHS; United States CA NCI NIH HHS

Keywords: CNS melanoma; abdominal disease; hydrocephalus; neurocutaneous melanocytosis; ventriculoperitoneal shunt

EC 3.6.1.- (GTP Phosphohydrolases)
0 (Membrane Proteins)
EC 3.6.1.- (NRAS protein, human)

Neurocutaneous melanosis

Date Created: 20241106 Date Completed: 20241122 Latest Revision: 20241219

20241219

10.1002/pbc.31434

39502030