Neurofibromatosis: Molecular Pathogenesis and Natural Compounds as Potential Treatments.

ABSTRACT: The neurofibromatosis syndromes, including NF1, NF2, and schwannomatosis, are tumor suppressor syndromes characterized by multiple nervous system tumors, particularly Schwann cell neoplasms. NF-related tumors are mainly treated by surgery, and some of them have been treated by but are refractory to conventional chemotherapy. Recent advances in molecular genetics and genomics alongside the development of multiple animal models have provided a better understanding of NF tumor biology and facilitated target identification and therapeutic evaluation. Many targeted therapies have been evaluated in preclinical models and patients with limited success. One major advance is the FDA approval of the MEK inhibitor selumetinib for the treatment of NF1-associated plexiform neurofibroma. Due to their an

SUBMITTER: Amaravathi A 

PROVIDER: S-EPMC8485038 | biostudies-literature | 2021

REPOSITORIES: biostudies-literature