Primary intracranial rhabdomyosarcoma of the cerebellopontine angle mimicking a vestibular schwannoma in a child.

Autor: Nair, Prakash, Das, Kuntal Kanti, Srivastava, Arun K., Sahu, R. N., Kumar, Raj, Yadava, Kamlesh, Pandey, Rakesh
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Zdroj: Asian Journal of Neurosurgery; Jan-Mar2017, Vol. 12 Issue 1, p109-111, 3p
Abstrakt: 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. [ABSTRACT FROM AUTHOR]
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