Neurofibromatosis Type I and Neurofibromatosis Type II

Neurofibromatosis type 1 (NF1) and Neurofibromatosis type 2 (NF2) are inherited, autosomal dominant, multisystem diseases. These syndromes are distinct from one another; however, both are associated with intracranial tumors. NF1 is associated with multiple tumor types, most commonly optic pathway gliomas and plexiform neurofibromas in children. Although benign, these tumors can cause significant morbidity due to dysfunction of local structures. Treatment is reserved for symptomatic lesions in the form of chemotherapy or targeted therapy when not amenable to surgical resection due to location. Malignant transformation is rare in pediatric patients. Tumors associated with NF2 are histologically benign, producing few symptoms during childhood and the aim of any treatment should be to preserve neurological functions and optimize long-term quality of life. Stereotactic surgery and radiation therapy have been used to preserve hearing in adolescents with vestibular schwannomas with growing evidence though limited to date, for chemotherapy and targeted therapies.