Objective To study the clinical pathological features of primary pancreatic neuroendocrine neoplasms (PNEN). Method The clinical data of 57 patients with pathologically confirmed primary PNEN were enrolled in the study, and the clinical pathological features of those patients were analyzed on the basis of related literature. Result Ⅰn the 57 cases, there were 45 cases of neuroendocrine tumors (NET), 9 cases of neuroendocrine carcinoma (NEC), 3 cases of mixed gonadal neuroendocrine carcinoma (MANEC); Pathological examination showed that NET was small, while NEC was usually a large single solid mass, and MANEC was the largest, and may be associated with bleeding or necro-sis;Ⅰmmunohistochemical assay demonstrated the positive expression of SYN and CGA (89.47%and 94.74%, respective-ly), with a higher expression of CD56 and NSE (85.96%and 84.21%, respectively), while negative expression was ob-served in broad-spectrum CK, CD10, nuclearβ-catenin and other markers;30 cases of functional PNEN were with obvi-ous symptoms of excessive secretion of hormones, but 24 cases of non-functional PNEN showed no obvious symptoms;46 cases were followed up, and 8 cases died (1 case of grade G2, 4 cases of grade G3 and 3 cases of MANEC). Conclusion The clinical manifestation of primary PNEN is complex and diverse, thus histopathological examination and immunohis-tochemical analysis are the main basis for accurate diagnosis of primary PNEN and prognosis evaluation.%目的 研究原发性胰腺神经内分泌肿瘤(PNEN)的临床病理学特征.方法 选取57例经手术切除病理证实为原发性PNEN患者的临床资料,结合文献报道分析其临床病理学特征.结果 57例PNEN患者中,45例为神经内分泌瘤(NET),9例为神经内分泌癌(NEC),3例为混合性腺神经内分泌癌(MANEC);病理学巨检显示,NET患者肿瘤的体积最小,NEC患者肿瘤的体积较大,且多为单发实性肿块,MANEC患者肿瘤体积最大,且多可见出血、坏死;免疫组化蛋白表现为弥漫性触突素(SYN)及嗜铬素A(CGA)阳性表达(89.47%、94.74%),并伴有较高的CD56及神经原特异性烯醇化酶(NSE)阳性表达(85.96%、84.21%),广谱角蛋白(CK)、CD10、核β-链蛋白(β-catenin)等标记呈阴性表达;30例功能型PNEN患者伴明显激素过度分泌症状,24例非功能型PNEN患者无明显症状;46例获得随访,8例死亡(G2级1例,G3级4例、MANEC 3例).结论 原发性PNEN的临床表现复杂多样,病理组织学检查及免疫组化分析是准确诊断原发性PNEN和预后评价的主要依据.
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