【摘要】 目的提高对胰腺原始神经外胚叶肿瘤(PNET)诊断和治疗的认识。方法分析我院胰腺PNET病例报道,阐述胰腺PNET的诊断和治疗。结果胰腺PNET极罕见,本例患者行保留十二指肠的胰腺钩突肿瘤切除术后行放疗及6疗程化疗,随访8个月无复发。胰腺PNET临床症状无特异性,以梗阻性黄疸及腹痛为主,诊断上除光镜下为小圆细胞恶性肿瘤及神经分化性结构外,免疫组织化学结果显示,针对特异性MIC2基因表达蛋白P30/32的胰头钩突CD99(+),细胞遗传学显示,t(11;22)(q24;q12)染色体异常,符合原始神经外胚叶肿瘤/尤文肉瘤(PNET/Ewing’s sarcoma)诊断,其恶性程度高,手术治疗、早期化疗、密切随访可能是提高生存率的主要手段,远期预后一般较差。结论临床及病理科医师应了解胰腺PNET的存在,提高临床和病理诊断水平,早期积极治疗,以期改善预后。更多还原

【Abstract】 Objective To improve the diagnosis and treatment of primitive neuroectodermal tumors (PNET) of the pancreas. Methods One patient with PNET of the pancreas was reported in this article. The corresponding literatures on the diagnosis and treatment was reviewed. Results 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. Conclusion PNET can occur in pancreas,and diagnosis and treatment should be made as early as possible to improve the outcome.更多还原