Death from a malignant cerebellopontine angle triton tumor despite stereotactic radiosurgery. Case report

J Neurosurg. 1998 Oct;89(4):653-8. doi: 10.3171/jns.1998.89.4.0653.

Abstract

Malignant vestibular nerve tumors are rare: to date, only three cases have been reported in the literature. The authors report a case of an eighth cranial nerve tumor that progressed 5 years after stereotactic radiosurgery. The patient was a 44-year-old man who underwent stereotactic radiosurgery for a 27-mm cerebellopontine angle tumor that was discovered on investigation of tinnitus and hearing loss. He developed facial weakness after 5 years, and repeated imaging revealed tumor enlargement. Despite complete microsurgical excision, the tumor rapidly recurred locally and subsequently disseminated within the neuraxis. The patient died 1 year after tumor progression was detected. Histopathological analysis revealed a malignant spindle cell neoplasm with frequent mitotic figures. The presence of positive rhabdoid elements on immunohistochemical studies confirmed that it was a triton tumor. The authors review the relevant literature concerning the classification and management of malignant vestibular nerve tumors and discuss the implications of tumor progression after stereotactic radiosurgery.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Cerebellar Neoplasms / pathology
  • Cerebellar Neoplasms / surgery*
  • Cerebellopontine Angle / pathology
  • Cerebellopontine Angle / surgery*
  • Cranial Nerve Neoplasms / pathology
  • Cranial Nerve Neoplasms / surgery
  • Disease Progression
  • Facial Paralysis / etiology
  • Fatal Outcome
  • Follow-Up Studies
  • Hearing Disorders / etiology
  • Humans
  • Male
  • Microsurgery
  • Neoplasm Invasiveness
  • Neoplasm Recurrence, Local / pathology
  • Neurilemmoma / pathology
  • Neurilemmoma / surgery*
  • Radiosurgery*
  • Tinnitus / etiology
  • Vestibular Nerve / pathology
  • Vestibular Nerve / surgery