64例胰腺神经内分泌肿瘤的临床病理特征  

Clinicopathological features of pancreatic neuroendocrine neoplasms： a retrospective analysis of 64 cases

作　　者：要文青[1] 王威亚[1] 李甘地[1] 

机构地区：[1]四川大学华西医院病理科,成都610041

出　　处：《中华肿瘤杂志》2014年第4期287-293,共7页Chinese Journal of Oncology

摘　　要：目的探讨胰腺神经内分泌肿瘤（P-NENs）患者的临床病理特点、治疗和预后情况。方法收集四川大学华西医院病理科2006年1月到2010年12月诊断的P-NENs患者的临床病理资料，通过对神经内分泌标记物突触素（Syn）和嗜铬粒蛋白A（CsA）的免疫组织化学染色确定肿瘤是否具有神经内分泌性质，按世界卫生组织2010年第4版P—NENs的诊断标准，以组织学核分裂象计数和Ki67增殖活性确定肿瘤的分级。按照是否引起临床综合征分为功能性P—NENs和非功能性P-NENs，探讨其临床病理特征和诊疗情况，分析P-NENs患者的预后影响因素。结果64例P—NENs患者中，神经内分泌瘤（NET）60例，神经内分泌癌（NEC）4例。肿瘤位于胰头33例（51．6％），胰尾21例（32．8％）。大体标本主要表现为单发、实性、圆形或类圆形肿块，肿瘤最大径为0．2—12cm，平均3．32cm；免疫组化染色结果显示，CgA和Syn的阳性率分别为96．9％和95．3％。临床分期为Ⅰ、Ⅱ、Ⅲ和Ⅳ期的患者分别为44、11、1和8例。64例P-NENs中，男性23例，女性41例。患者的发病年龄为20～69岁，平均年龄45．56岁。功能性P—NENs 38例，主要表现为激素过度分泌症状；非功能性26例，临床症状不典型。行手术治疗58例，非手术治疗6例。与胰腺其他肿瘤不同，P-NENs预后好，5年无进展生存率为91．4％。结论P—NENs可发生于胰腺各个部位，临床表现多样，易被误诊，确诊依靠病理诊断，手术是其主要治疗手段，预后相对较好。肿瘤分级和远处转移是影响P-NENs患者预后的独立因素。Objective To analyze the clinicopathological features of pancreatic neuroendocrine neoplasms （P-NENs）. Methods From January 2006 to December 2010, 64 patients with P-NENs were diagnosed in the Department of Pathology, West China Hospital, Siehuan University staining of neuroendocrine markers, synaptophysin （Syn） and chromogranin A （CgA）, were first made to determine whether the tumor had neuroendocrine properties, then the P-NENs were classified as neuroendocrine tumor （NET）, neuroendocrine carcinoma （NEC） and mixed adenoneuroendocrine carcinoma （MANEC, （;3） according to the morphological changes and proliferative activity （Ki 67 expression）. Results Of all the 64 cases detected, 60 were NETs and four were NEC. Most of the tumors were single solitary masses, and more than half of the tumors arose in the head of the pancreas, while about one third in the tail. The positive rates of CgA and Syn immunostaining were 96.9% and 95.3%, respectively. The tumor stages of the 64 patients were as follows ： stage Ⅰ , 44 cases; stage Ⅱ, 11 cases; stage Ⅲ, one case ; and stage Ⅳ, 8 cases. The median age of patients in the study was 45.56 years. Of all the P-NENs, 38 cases were functional ones, presenting with characteristic clinical syndrome owing to hormone hypersecretion, while 26 cases were nonfunctional ones with no distinct clinical syndrome. 58 patients underwent surgical operation. The 5-year progression-free survival rate was 91- 4%. Conclusions P-NENs may occur anywhere in the pancreas, and the clinical manifestations may not be easy to distinguish from other diseases. Diagnosis depends on pathological examination. Surgery is the major approach option, and the clinical prognosis is rather good. The tumor histological grade and distant metastasis are independent prognostic factors in P-NENs.

关 键 词：胰腺 神经内分泌瘤 病理学 临床 

分 类 号：R735.9[医药卫生—肿瘤]