- 期刊
Peripheral Primitive Neuroectodermal Tumor: Report of Two Cases in Abdomen and Pelvis and Clinical Review of 23 Cases
原發性週邊神經外胚層腫瘤：原發於腹骨盆腔之二病例報告及二十三例病例回顧
摘要
原發性週邊神經外胚層腫瘤是一種好發於小孩及年輕人的惡性腫瘤。主要是位於腹骨盆腔，胸腔，以及四肢。最常見的症狀為疼痛及可觸摸到的腫塊。診斷則是依靠免疫泞組織化學染色，電子顯微鏡。治療方式為手術及化學治療，整體預後差。一般腸胃科醫師對此少見之惡性腫瘤不甚瞭解。我們報告二例原發於腹骨盆腔之原發性週邊神經外胚層腫瘤以及追蹤台大醫院二十年來罹患此病病例作進一步探討。
關鍵字
原發性週邊神經外胚層腫瘤並列摘要
Peripheral primitive neuroectodermal tumors (pPNETs) are a group of unusual small round cell malignancies arising in children and young adults. These tumors occur primarily in bone and soft tissue of the chest, abdomen/pelvis and extremities. The most common presentations are pain and palpable mass. Features of immunohistochemisty and electromicroscopy establish the diagnosis. The treatment of choice of pPNETs is combined surgical resection and chemotherapy; however, the overall prognosis is usually poor. We present two cases of pPNETs of abdominal and pelvic origin and review the previous cases of pPNETs in National Taiwan University Hospital and discuss the characteristics of abdominal and pelvic pPNETs.
延伸閱讀
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