Neurocutaneous melanosis is a rare congenital syndrome characterized by large or multiple congenital melanocytic nevi and benign or malignant pigment cell tumors of the leptomeninges. The prognosis is extremely poor for symptomatic patients, even in the absence of malignant melanoma. We present serial MR imaging findings in the brain and spine of a child with congenital giant hairy nevi who developed progressive leptomeningeal melanomatosis and whose neurologic condition rapidly deteriorated.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7974137 | PMC |
Publication Analysis
Top Keywords
Similar Publications
Missense mutation of NRAS is associated with malignant progression in neurocutaneous melanosis.
Acta Neuropathol Commun
January 2024
Department of Neurosurgery, Brain Research Institute, Niigata University, 1-757 Asahimachi, Chuo-ku, 951-8585, Niigata, Japan.
Neurocutaneous melanosis (NCM) is a rare congenital neurocutaneous syndrome characterized by congenital melanocytic nevus of skin and abnormal proliferation of leptomeningeal melanocytes. Early acquisition of post-zygotic somatic mutations has been postulated to underlie the pathogenesis of NCM. The pathogenesis of NCM remains to be fully elucidated, and treatment options have not been established.
View Article and Find Full Text PDFPrimary meningeal melanoma masquerading as neurofibromatosis type 2: illustrative case.
J Neurosurg Case Lessons
November 2021
Departments of Pathology and Laboratory Medicine.
Background: Primary meningeal melanocytic neoplasms are exceedingly rare tumors, representing only 0.06% to 0.1% of all primary brain tumors and ranging in spectrum from benign localized tumors to highly aggressive malignant lesions.
View Article and Find Full Text PDFNonsyndromic Primary Diffuse Leptomeningeal Melanomatosis in a Child.
Iran J Child Neurol
January 2021
Department of Neurological Surgery, Ghaem Hospital, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran.
In this study, we present a case of primary diffuse leptomeningeal melanomatosis (PDLM), without neurocutaneous melanosis syndrome. A female patient (age: 14 years) presented with headache, nausea, vomiting, vertigo, diplopia, and lower limb weakness. The magnetic resonance imaging (MRI) showed leptomeningeal isointensity on T1- and T2-weighted images and hyperintensity on fluid attenuation inversion recovery (FLAIR) sequences.
View Article and Find Full Text PDFImaging and Clinical Features of Neurocutaneous Melanosis in the Pediatric Population.
Curr Med Imaging
January 2022
Department of Biomedical and Dental Sciences and of Morphofunctional Imaging, University of Messina, Messina, Italy.
Background: Neurocutaneous Melanosis (NCM) is a rare nonfamilial phakomatosis characterized by the presence of congenital melanocytic nevi and abnormal melanocyte infiltration of the leptomeninges.
Objective And Methods: This paper shows the importance of early diagnosis and the most important imaging features of the disease on CT and MR scans. PubMed database was searched from January 1972 to September 2020.
Novel Insights into Diagnosis, Biology and Treatment of Primary Diffuse Leptomeningeal Melanomatosis.
J Pers Med
April 2021
Department of Pediatrics and Adolescent Medicine and Comprehensive Center for Pediatrics, Medical University of Vienna, 1090 Vienna, Austria.
Primary diffuse leptomeningeal melanomatosis (PDLMM) is an extremely rare and aggressive cancer type for which best treatment strategies remain to be elucidated. Herein, we present current and prospective diagnostic strategies and treatment management of PDLMM. Against the background of an extensive literature review of published PDLMM cases and currently employed therapeutic strategies, we present an illustrative case of a pediatric patient suffering from PDLMM.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!