Evidence of polyclonality in neurofibromatosis type 2-associated multilobulated vestibular schwannomas.

ABSTRACT:

Background

Neurofibromatosis type 2 (NF2) is a tumor syndrome that results from mutation of the NF2 tumor suppressor gene. The hallmark of NF2 is the presence of bilateral vestibular schwannoma (VS). Though NF2-associated and sporadic VS share identical histopathologic findings and cytogenetic alterations, NF2-associated VS often appears multilobulated, is less responsive to radiosurgery, and has worse surgical outcomes. Temporal bone autopsy specimens and MRI of the inner ear performed on NF2 patients suggest that multiple discrete tumors may be present within the labyrinth and cerebellopontine angle.

Methods

Treatment-naïve ears in patients enrolled in a prospective NF2 natural history study (NIH#08-N-0044) were included for MRI analysis. T2-weighted and postcontrast T1-we

SUBMITTER: Dewan R 

PROVIDER: S-EPMC4483079 | biostudies-literature | 2015 Apr

REPOSITORIES: biostudies-literature