المؤلفون: Romo, Carlos G, Piotrowski, Anna F, Campian, Jian L, Diarte, Jose, Rodriguez, Fausto J, Bale, Tejus A, Dahiya, Sonika, Gutmann, David H, Lucas, Calixto-Hope G, Prichett, Laura, Mellinghoff, Ingo, Blakeley, Jaishri O

المصدر: Neuro Oncol ; ISSN:1523-5866 ; Volume:25 ; Issue:8

مصطلحات موضوعية: Neurofibromatosis type 1, Non-optic pathway glioma, glioma, overall survival, treatment outcome

الوصف: People with NF1 have an increased prevalence of central nervous system malignancy. However, little is known about the clinical course or pathologic features of NF1-associated gliomas in adults, limiting clinical care and research.

العلاقة: https://doi.org/10.1093/neuonc/noad033Test; https://pubmed.ncbi.nlm.nih.gov/36840626Test; https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10398805Test/

الإتاحة: https://doi.org/10.1093/neuonc/noad033Test
https://pubmed.ncbi.nlm.nih.gov/36840626Test
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10398805Test/

المؤلفون: Jiang, Chunhui, McKay, Renée M, Lee, Sang Y, Romo, Carlos G, Blakeley, Jaishri O, Haniffa, Muzlifah, Serra, Eduard, Steensma, Matthew R, Largaespada, David, Le, Lu Q

المصدر: J Invest Dermatol ; ISSN:1523-1747 ; Volume:143 ; Issue:8

مصطلحات موضوعية: ECM, GEM, KO, LOH, MAPK/extracellular signal–regulated kinase kinase, MEK, MPNST, NF1, cNF, cutaneous neurofibroma, extracellular matrix, genetically engineered mouse, knockout, loss of heterozygosity, malignant peripheral nerve sheath tumor, neurofibromatosis type 1, pNF, plexiform neurofibroma

الوصف: Neurofibromatosis type 1 is one of the most common genetic disorders of the nervous system and predisposes patients to develop benign and malignant tumors. Cutaneous neurofibromas (cNFs) are NF1-associated benign tumors that affect nearly 100% of patients with NF1. cNFs dramatically reduce patients' QOL owing to their unaesthetic appearance, physical discomfort, and corresponding psychological burden. There is currently no effective drug therapy option, and treatment is restricted to surgical removal. One of the greatest hurdles for cNF management is the variability of clinical expressivity in NF1, resulting in intrapatient and interpatient cNF tumor burden heterogeneity, that is, the variability in the presentation and evolution of these tumors. There is growing evidence that a wide array of factors are involved in the regulation of cNF heterogeneity. Understanding the mechanisms underlying this heterogeneity of cNF at the molecular, cellular, and environmental levels can facilitate the development of innovative and personalized treatment regimens.

العلاقة: https://doi.org/10.1016/j.jid.2022.12.027Test; https://pubmed.ncbi.nlm.nih.gov/37318402Test; https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11173230Test/

الإتاحة: https://doi.org/10.1016/j.jid.2022.12.027Test
https://pubmed.ncbi.nlm.nih.gov/37318402Test
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11173230Test/