Trial of Selumetinib in Patients With Neurofibromatosis Type II Related Tumors
Conditions: Neurofibromatosis 2; Vestibular Schwannoma; Meningioma; Ependymoma; Glioma Intervention: Drug: Selumetinib Sponsors: Children's Hospital Medical Center, Cincinnati; AstraZeneca Not yet recruiting - verified March 2017
AbstractNeurofibromatosis type II (NF2) is a tumor predisposition syndrome characterized by the development of distinctive nervous system lesions. NF2 results from loss-of-function alterations in theNF2 gene on chromosome 22, with resultant dysfunction of its protein product merlin. NF2 is most commonly associated with the development of bilateral vestibular schwannomas; however, patients also have a predisposition to development of other tumors including meningiomas, ependymomas, and peripheral, spinal, and cranial nerve schwannomas. Patients may also develop other characteristic manifestations such as ocular lesions, neu...
CONCLUSION: This is the first case of medulloblastoma occurring in a patient with NF2 and raises the possibility of an association between medulloblastoma and NF2. PMID: 26539318 [PubMed]