Death from a malignant cerebellopontine angle triton tumor despite stereotactic radiosurgery. Case report.
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.
Department of Neurological Surgery, University of Pittsburgh Medical Center, Pennsylvania, USA.
C H
CH
Comey
M R
MR
McLaughlin
H D
HD
Jho
A J
AJ
Martinez
L D
LD
Lunsford
D000328:Adult; D002528:Cerebellar Neoplasms / Q000473:pathology / Q000601:surgery*; D002530:Cerebellopontine Angle / Q000473:pathology / Q000601:surgery*; D003390:Cranial Nerve Neoplasms / Q000473:pathology / Q000601:surgery; D018450:Disease Progression; D005158:Facial Paralysis / Q000209:etiology; D017809:Fatal Outcome; D005500:Follow-Up Studies; D006311:Hearing Disorders / Q000209:etiology; D006801:Humans; D008297:Male; D008866:Microsurgery; D009361:Neoplasm Invasiveness; D009364:Neoplasm Recurrence, Local / Q000473:pathology; D009442:Neurilemmoma / Q000473:pathology / Q000601:surgery*; D016634:Radiosurgery; D014012:Tinnitus / Q000209:etiology; D014725:Vestibular Nerve / Q000473:pathology / Q000601:surgery