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Peripheral Primitive Neuroectodermal Tumor: Report of a Case

週邊胚葉神經外胚層瘤:一病例報告

摘要


週邊胚葉神經外胚層瘤(p PNET)是一罕見之腫瘤。我們報告這一罕見病例。一位11歲男孩主訴左小腿腫痛1年。放射線檢驗發現一噬骨性病灶於遠端脛骨。經組織病理及特殊染色確立診斷治療。以化學治療合併局部放射治療後達到完全之緩解(患者及家屬拒絕手術治療)。根據回顧文獻得知,週邊胚葉神經外胚層瘤是罕見高度惡性度之腫瘤,一般預後不好。極待發展新的治療方式以改善預後。

並列摘要


Peripheral primitive neuroectodermal tumor (pPNET) is a rare tumor. We present a patient who was diagnosed as pPNET. An 11-year-old boy had complained of pain and swelling in his left leg for one year. Imaging studies revealed an osteolytic lesion in his left distal tibia. Histopathology and immunohistochemistry results led to the diagnosis of pPNET. The treatment of the tumor consisted of a combination of chemotherapy with hematopoietic growth factor support and local radiotherapy. Complete remission was achieved after completion of treatment. After a brief review of the literature, we found that PNETs are unusual and highly aggressive neoplasms. In general, the prognosis of patients with PNETs is poor and new treatment strategies are necessary for patients with these rare tumors.

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