Peripheral Primitive Neuroectodermal Tumor of the Head-A Case Report

周邊性原始神經外胚層腫瘤是一推測源自於中樞神經及交感神經系統外的神經脊的軟組織腫瘤，其發生於頭部的機率很少見。我們報告一20歲男性發現於左顳部有一逐漸變大的腫塊約已有六周。核磁共振攝影檢查顯示左顳部軟組織腫瘤合併顴骨弓及顳骨侵犯腐蝕，組織病理切片檢查使用顯微鏡及免疫組織化學染色確立診斷為周邊性原始外胚層腫瘤，經一系列檢查，並無全身侵犯，之後病患接受廣泛性切除手術，切除包括腫瘤、顳肌、顴骨弓、部份顳骨及下頷骨的喙突，術後並接受化學治療及放射治療，目前術後追綜一年狀況良好。本文將回顧及討論此腫瘤的臨床特性、病理及治療。

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並列摘要

Peripheral primitive neuroectodermal tumors (PNETs) are a group of soft-tissue tumors of presumed neural crest origin arising outside the central and sympathetic nervous system. The occurrence of the tumors in the head is rare. A 20-year-old male presented with a progressively enlarged tumor over left temporal region for about six weeks. Magnetic resonance imaging study showed a heterogenous enhancing soft-tissue tumor over left temporal region with zygomatic arch and temporal bone erosion. Histological study of intra-lesional biopsy confirmed the diagnosis of PNET by using light microscopy and immunohistochemical stains. There was no systemic involvement after series of evaluations. The patient was then treated with extensive wide excision and combined postoperative chemotherapy and radiotherapy. The clinical features, pathology and treatment of these tumors are reviewed and discussed.

並列關鍵字

Primitive neuroectodermal tumor Introduction

國際替代計量

Peripheral Primitive Neuroectodermal Tumor of the Head-A Case Report

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