Ontology highlight
ABSTRACT:
SUBMITTER: Ferriero K
PROVIDER: S-EPMC7530181 | biostudies-literature | 2020
REPOSITORIES: biostudies-literature
Ferriero Kristen K Shah Biraj B Yan Yun Y Khatri Surya S Caccamese John J Napoli Joseph A JA Bober Michael B MB Crane Janet L JL
Frontiers in pediatrics 20200918
Noonan syndrome is a genetic disorder caused by mutations in the RAS/MAPK pathway. Multiple giant cell lesions are a rare sequelae of disruptions in this pathway, termed Noonan-like multiple giant cell lesions (NL/MGCLs). Medical management of these tumors rather than surgical intervention is preferential as the lesions are benign but locally destructive and recurring. This case series describes four male pediatric patients with Noonan syndrome and multiple giant cell lesions of the jaw treated ...[more]