Sang Xin-ting, Liang Nai-xin, Mao Yi-lei, Lu Xin, Yang Zhi-ying, Zhong Shou-xian, Huang Jie-fu
Department of Surgery, PUMC Hospital, CAMS and PUMC, Beijing 100730, China.
Zhongguo Yi Xue Ke Xue Yuan Xue Bao. 2006 Apr;28(2):191-5.
To improve the diagnosis and treatment of primitive neuroectodermal tumors (PNET) of the pancreas.
One patient with PNET of the pancreas was reported in this article. The corresponding literatures on the diagnosis and treatment was reviewed.
The patient was diagnosed as pancreatic PNET by her clinical, microscopic, and immunohistochemical features as well as cytogenetic analysis after the resection of the tumor located in the uncinate process in PUMC Hospital. Radiochemotherapy was given after the operation for 8 months and no recurrence was observed. Since PNET of pancreas have no specific clinical symptoms and most patients have jaundice and/or abdominal pain, the diagnosis depended on the immunohistochemical features of positive P30/32(MIC2) and at least two of the neural markers. The cytogenetic analysis showed translocation mainly harbored the characteristic t (11; 22) (q24; q12). Since pancreatic PNET were highly aggressive, early chemotherapy, close follow-up, and immediate surgical interventions were required as early as possible.
PNET can occur in pancreas, and diagnosis and treatment should be made as early as possible to improve the outcome.
提高胰腺原始神经外胚层肿瘤（PNET）的诊断与治疗水平。
本文报道1例胰腺PNET患者，并复习其诊断与治疗的相关文献。
该患者在中国医学科学院北京协和医院接受肿瘤位于钩突部的切除术后，经临床、显微镜检查、免疫组化特征及细胞遗传学分析确诊为胰腺PNET。术后给予8个月的放化疗，未观察到复发。由于胰腺PNET无特异性临床症状，大多数患者有黄疸和/或腹痛，诊断依赖于P30/32（MIC2）阳性及至少两种神经标志物的免疫组化特征。细胞遗传学分析显示易位主要具有特征性的t（11；22）（q24；q12）。由于胰腺PNET具有高度侵袭性，需要尽早进行早期化疗、密切随访及立即手术干预。
PNET可发生于胰腺，应尽早进行诊断和治疗以改善预后。
