Effects of Hormonal Changes on the Growth of Pediatric Plexiform Neurofibromas

Neurofibromatosis (NF) is a genetic disorder that affects almost every organ system. The condition causes tumors to grow on nerves in the brain and throughout the body.

Children with neurofibromatosis often develop tumors called plexiform neurofibromas. These tumors are usually large and irregularly shaped and can grow from nerves anywhere in the body. 

While plexiform neurofibromas are generally benign they can sometimes interfere with normal organ development and result in learning disabilities, poor school performance and attention deficit disorders.

Clinicians have suggested that an increase in the size and number of plexiform neurofibromas (as well as an increased potential for malignant transformation) in patients with NF1 occurs during periods of hormonal change, specifically puberty and pregnancy.

Amish Shah, MD, PhD, of the Center for Childhood Cancer Research is analyzing these tumors to determine whether or not hormonal changes in pediatric patients cause plexiform neurofibromas to grow faster.

The results of these studies may lead to the identification of novel targets that can be used to slow the growth of plexiform neuroblastomas, which frequently cannot be surgically removed and often lead to developmental and cognitive impairments.