{"title":"Therapeutic Strategies in Neurocutaneous Melanocytosis.","authors":"Dipanjan Basu","doi":"10.21873/anticanres.17341","DOIUrl":null,"url":null,"abstract":"<p><p>Neurocutaneous melanocytosis (NCM) is a rare, congenital condition primarily affecting children, characterized by large or giant congenital melanocytic nevi (L/GCMN) on the skin and pigmented lesions in the brain. The presence of pigmented lesions in the brain often leads to neurological symptoms like headaches, seizures, and hydrocephalus, typically manifesting before age two. Melanocytic lesions in the brain can range from benign melanocytosis to malignant melanoma. NCM is nearly always fatal if symptomatic, with a high risk of malignant transformation. Patients with larger skin nevi with neurological involvement tend to have a greater lifetime risk of malignancy. There is no specific treatment, and current therapies focus on palliative care, including surgery, radiation, and chemotherapy. Malignant transformation into melanoma requires aggressive treatments like surgery, radiation, and chemotherapy. Despite these approaches, outcomes remain poor, with no definitive cure for NCM. This study aims to review and critically evaluate the current therapeutic strategies for NCM while also exploring prospective avenues for developing specific and effective treatments. It aims to highlight recent advancements in the molecular understanding of NCM and examine how these insights may inform the development of novel therapeutic approaches. Additionally, the study will address the significant unmet medical needs associated with this rare and often fatal condition, emphasizing the importance of continued research and innovation in its management.</p>","PeriodicalId":8072,"journal":{"name":"Anticancer research","volume":"44 12","pages":"5157-5167"},"PeriodicalIF":1.6000,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Anticancer research","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.21873/anticanres.17341","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"ONCOLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
Neurocutaneous melanocytosis (NCM) is a rare, congenital condition primarily affecting children, characterized by large or giant congenital melanocytic nevi (L/GCMN) on the skin and pigmented lesions in the brain. The presence of pigmented lesions in the brain often leads to neurological symptoms like headaches, seizures, and hydrocephalus, typically manifesting before age two. Melanocytic lesions in the brain can range from benign melanocytosis to malignant melanoma. NCM is nearly always fatal if symptomatic, with a high risk of malignant transformation. Patients with larger skin nevi with neurological involvement tend to have a greater lifetime risk of malignancy. There is no specific treatment, and current therapies focus on palliative care, including surgery, radiation, and chemotherapy. Malignant transformation into melanoma requires aggressive treatments like surgery, radiation, and chemotherapy. Despite these approaches, outcomes remain poor, with no definitive cure for NCM. This study aims to review and critically evaluate the current therapeutic strategies for NCM while also exploring prospective avenues for developing specific and effective treatments. It aims to highlight recent advancements in the molecular understanding of NCM and examine how these insights may inform the development of novel therapeutic approaches. Additionally, the study will address the significant unmet medical needs associated with this rare and often fatal condition, emphasizing the importance of continued research and innovation in its management.
期刊介绍:
ANTICANCER RESEARCH is an independent international peer-reviewed journal devoted to the rapid publication of high quality original articles and reviews on all aspects of experimental and clinical oncology. Prompt evaluation of all submitted articles in confidence and rapid publication within 1-2 months of acceptance are guaranteed.
ANTICANCER RESEARCH was established in 1981 and is published monthly (bimonthly until the end of 2008). Each annual volume contains twelve issues and index. Each issue may be divided into three parts (A: Reviews, B: Experimental studies, and C: Clinical and Epidemiological studies).
Special issues, presenting the proceedings of meetings or groups of papers on topics of significant progress, will also be included in each volume. There is no limitation to the number of pages per issue.