Neurocutaneous melanosis (NCM) is one of the rare, congenital, noninheritable phakomatoses characterized by the presence of large and/or multiple congenital melanocytic cutaneous nevi associated with intracranial leptomeningeal melanocytosis. NCM usually presents before 2 years of age. So far 302 cases have been reported in literature. We report a case of NCM presenting with obstructive hydrocephalus and Dandy-Walker Variant in a young adult.
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Therapeutic 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.
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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.
View Article and Find Full Text PDFIsolated melanoma metastasis in a patient with large congenital nevus without detectable primary melanoma: a case report and review of literature.
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Chemotherapy Unit and One-Day Chemotherapy Unit, Specialist Oncology Hospital NU-MED, Tomaszów Mazowiecki, Poland.
Giant congenital pigmented nevi constitute an extremely diverse group of skin lesions with varying morphologies. These nevi are often associated with many clinical implications, such as increased risk of melanoma and the presence of neurocutaneous melanosis, with melanoma being the primary concern. We present a rare case of a 62-year-old patient with a giant congenital birthmark who reported to the oncology department due to a tumor in the lower abdomen detected during an ultrasound examination.
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Complexo Hospital de Clínicas, Universidade Federal do Parana, Curitiba, State of Paraná, Brazil; Department of Pediatrics, Complexo Hospital Pequeno Principe, Curitiba, State of Paraná, Brazil.
Neurocutaneous melanosis is characterized by melanin-producing cells within the skin, leptomeninges, or brain parenchyma. It is a severe manifestation of congenital melanocytic nevus syndrome and can develop malignant melanomas. The disorder is commonly present in pediatric patients within the first two years of life and has a poor prognosis.
View Article and Find Full Text PDFCongenital melanocytic nevus syndrome: An association between congenital melanocytic nevi and neurological abnormalities.
Semin Pediatr Neurol
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Department of Pediatrics, Division of Pediatric Dermatology, Dell Medical School, University of Texas, 1301 Barbara Jordan Blvd, Suite 200A, Austin, TX 78723, United States. Electronic address:
Article Synopsis
- Congenital melanocytic nevus syndrome is characterized by the presence of congenital melanocytic nevi (CMN) along with various neurosystem abnormalities, primarily due to mutations in the NRAS gene.
- CMN appears as collections of melanocytes on the skin and can be classified by size and various clinical features like location and color, while neurological issues might include seizures and developmental delays.
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