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KMID : 0361020010440080876
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Korean Journal of Otolaryngology - Head and Neck Surgery
2001 Volume.44 No. 8 p.876 ~ p.880
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A Case of Extracranial Primitive Neuroectodermal Tumor that occured in the temporal fossa
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박시영/See Young Park
김상철/정범조/이혜경/Sang Chul Kim/Bum Jo JungHye Kyung Lee
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Abstract
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Primitive Neuroectodermal tumor(PNET) is a rare disease sharing
similar histology, immunohistochemistry, and cytogenetics with other
small round-cell tumor such as Ewing's sarcoma, rhabdomyosarcoma and
neuroblastoma. PNET is usually located in the cerebellar vermis in
children and constitutes up to 25% of central nervous system tumors in
children. PNET occasionally occurred in the periphery. Peripheral PNET
is usually located in the thoracopulmonary region and rare in the head
and neck without intracranial metastasis. Ultrastructural,immunohisto
-chemistrical and electronic microscopic data were necessary to
confirm the diagnosis of PNET. Due to frequent recurrence and early
metastasis, prognosis was bad and the treatment was concomittant
surgery & chemotherapy. The authors experienced a case of PNET located
in the extracranial temporal fossa, which were managed with surgery,
chemotherapy and radiotherapy.
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