Open AccessPrimary intracranial rhabdomyosarcoma of the cerebellopontine angle mimicking a vestibular schwannoma in a child
Author(s) -
Prakash Nair,
Kuntal Kanti Das,
Arun Kumar Srivastava,
RN Sahu,
Raj Kumar,
Kamlesh Yadava,
Rakesh Pandey
Publication year - 2017
Publication title -
asian journal of neurosurgery
Language(s) - English
Resource type - Journals
ISSN - 2248-9614
DOI - 10.4103/1793-5482.150000
Subject(s) - medicine , cerebellopontine angle , schwannoma , rhabdomyosarcoma , radiation therapy , embryonal rhabdomyosarcoma , vestibular system , radiology , surgery , sarcoma , magnetic resonance imaging , pathology
Primary intracranial rhabdomyosarcoma (PIRMS) is a rare neoplasm, which affects infants and young children. We report a rare case of a primary embryonal rhabdomyosarcoma of the cerebellopontine angle in a 7-year-old boy with clinical and radiological features mimicking a vestibular schwannoma. The patient underwent definitive surgery and radiotherapy and is recurrence free at 6 months. PIRMS commonly occur in parameningeal locations or metastasize to the brain from an extracranial primary. Gross total tumor resection followed by adjuvant radiotherapy remains the current standard of treatment; however, chemotherapy has also been tried with favorable results. The overall prognosis continues to remain poor.