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Congenital melanocytic nevi initiated by BRAF fusion oncogene with firmness, pruritus, and desmoplastic stroma.
Br J Dermatol
February 2025
Department of Dermatology, University of Texas Southwestern, Dallas, TX, USA.
Background: Large and giant congenital melanocytic nevi present a risk for developing melanoma or neurocutaneous melanosis. Most are caused by NRAS or, less commonly, BRAF mutations.
Objectives: We present a series of patients with large to giant congenital melanocytic nevi with BRAF fusion genes as driver alterations and describe their unique clinical presentation.
Congenital Melanocytic Nevi.
Clin Plast Surg
April 2025
Shriners Hospital for Children, Chicago, IL, USA.
Congenital melanocytic nevi are benign neoplasms, which present early in life at various anatomic locations with a variety of clinical presentations. When evaluating large or giant nevi, it's important to use a multidisciplinary team and rule out comorbid conditions, such as neurocutaneous melanosis. Surgical interventions include direct excision, serial excision, and tissue expansion.
View Article and Find Full Text PDFClinical Features and Risks of Congenital Melanocytic Naevi: A Retrospective Analysis of Patients at the Queensland Children's Hospital.
Australas J Dermatol
March 2025
QCIF Bioinformatics, Institute for Molecular Bioscience, The University of Queensland, Brisbane, Queensland, Australia.
Background/objectives: Congenital melanocytic naevi (CMN) are a risk factor for melanoma. Melanoma risk is dependent on the congenital phenotype. Our primary aims were to assess the clinical characteristics of CMN that indicate a high risk of neurocutaneous melanosis (NCM) and melanoma in an Australian paediatric population group; to identify patient characteristics and clinical features of CMN that trigger further investigations; and to determine the rate of malignancy and other complications for CMN.
View Article and Find Full Text PDFTherapeutic Strategies in Neurocutaneous Melanocytosis.
Anticancer Res
December 2024
Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, PA, U.S.A.
Article Synopsis
- * The condition is usually fatal if symptoms appear, with a high chance of the brain lesions becoming malignant, especially in those with larger skin nevi.
- * Current treatments focus on palliative care and aim to manage symptoms, as there is no cure, highlighting the need for ongoing research to develop effective therapies and address this critical medical gap.
Neurocutaneous Melanocytosis-Associated Hydrocephalus: The Memorial Sloan Kettering Experience from 2001 to 2022.
Pediatr Blood Cancer
January 2025
Deaprtment of Pediatrics, Memorial Sloan Kettering Cancer Center, New York, New York, USA.
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.
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