ArticleCancer discovery2013
Primary melanoma of the CNS in children is driven by congenital expression of oncogenic NRAS in melanocytes.
Article in Cancer discovery, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 43 papers.
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Who cites it
43 citing papers in PubMed, 77 citations in OpenAlex.
- Pediatric Melanoma: Emerging Therapies and Ongoing Clinical Trials.Dermatology and therapy · 2026Review
- Pigmented Peril: A fatal case of Primary intracranial melanoma in a paediatric patient.Radiology case reports · 2025Article
- Primary Diffuse Leptomeningeal Melanomatosis Leading to Raised Intracranial Pressure in a Pediatric Patient.Clinical case reports · 2025Article
- Congenital melanocytic neoplasms: clinical, histopathological and recent molecular developments.Virchows Archiv : an international journal of pathology · 2025Review
- Mical1 deletion in tyrosinase expressing cells affects mouse running gaits.Genes, brain, and behavior · 2024Article
- Review
- Missense mutation of NRAS is associated with malignant progression in neurocutaneous melanosis.Acta neuropathologica communications · 2024Article
- Primary Diffuse Leptomeningeal Melanomatosis in a Child with Extracranial Metastasis: Case Report.Current oncology (Toronto, Ont.) · 2024Article
- Primary intracranial malignant melanoma in an adolescent girl with NRAS and TP53 mutations: case report and literature review.Frontiers in oncology · 2024Article
- The journey from melanocytes to melanoma.Nature reviews. Cancer · 2023Review
- Characterization of proteome-size scaling by integrative omics reveals mechanisms of proliferation control in cancer.Science advances · 2023Article
- How Should We Treat Meningeal Melanocytoma? A Retrospective Analysis of Potential Treatment Strategies.Cancers · 2022Article
- Longest survival with primary intracranial malignant melanoma: A case report and literature review.World journal of clinical cases · 2022Article
- Liquid Biopsy with Detection of NRASDiagnostics (Basel, Switzerland) · 2022Article
- Article
- Primary meningeal melanoma masquerading as neurofibromatosis type 2: illustrative case.Journal of neurosurgery. Case lessons · 2021Article
- The retrospective molecular analysis of large or giant congenital melanocytic nevi in a group of Polish children.Journal of mother and child · 2021Article
- Cyclooxygenase-2 Inhibitor: A Potential Combination Strategy With Immunotherapy in Cancer.Frontiers in oncology · 2021Review
- Pericyte Ontogeny: The Use of Chimeras to Track a Cell Lineage of Diverse Germ Line Origins.Methods in molecular biology (Clifton, N.J.) · 2021Article
- The Evolution of Melanoma - Moving beyond Binary Models of Genetic Progression.The Journal of investigative dermatology · 2020Review
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Authors and funding
15 authors at 6 institutions in 3 countries.
Funding
Abstract
unlabelledNRAS mutations are common in human melanoma. To produce a mouse model of NRAS-driven melanoma, we expressed oncogenic NRAS (NRAS(G12D)) in mouse melanocytes. When NRAS(G12D) was expressed in the melanocytes of developing embryos, it induced melanocyte proliferation and congenital melanocytic lesions reminiscent of human blue nevi but did not induce cutaneous melanoma. Unexpectedly, however, it did induce early-onset primary melanoma of the central nervous system (CNS). The tumors were rapidly proliferating and caused neurologic symptoms, rapid health deterioration, and death. NRAS is not a common driver oncogene of primary melanoma of the CNS in adults, but we report two cases of primary melanoma of the CNS in children, both of which carried oncogenic mutations in NRAS. We conclude that acquisition of somatic mutations in NRAS in CNS melanocytes is a predisposing risk factor for primary melanoma of the CNS in children, and we present a mouse model of this disease. SIGNIFICANCE: We show that the acquisition of NRAS mutations in melanocytes during embryogenesis is a risk factor for early-onset melanoma of the CNS. We have developed a powerful mouse model to study this rare but devastating childhood disease, and to develop therapeutic approaches for its treatment.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.