90例胃肠道神经内分泌肿瘤病理学重新诊断评估

Reassessment of Pathological Diagnosis in 90 Cases of Gastrointestinal Neuroendocrine Neoplasm

目的采用新版WHO消化系统肿瘤分类对过去诊断的81例胃肠道神经内分泌肿瘤(GINEN)和50例胃肠道腺癌伴神经内分泌分化的病例进行再评估,并对重新诊断的GINEN临床病理特点及分型标准进行了初步探讨。方法参照新标准对过去的病例重新进行组织学观察,在组织芯片中应用免疫组织化学方法检测Syn、CgA、CD56和Ki-67的表达。结果 90例GINEN患者中,神经内分泌瘤1级(NET 1级)23例,神经内分泌瘤2级(NET 2级)3例,神经内分泌癌(NEC)59例,混合性腺神经内分泌癌(MANEC)5例。Syn、CgA和CD56的表达率分别为78.89%、53.33%和33.33%。组织学类型与肿瘤大小、周围组织浸润、淋巴结转移及脉管癌栓密切相关(χ2=16.496、15.804、11.617、8.664,均P<0.05)。小细胞癌、大细胞癌和混合型NEC患者的生存时间无显著性差异(Log Rank检验χ2=0.913,P>0.05)。NET和NEC的2年生存率分别为87%和35%。结论 GINEN组织形态学表现复杂多样,但仍具备一定的病理特征,Syn和CgA的联合检测可协助诊断,诊断时须排除低分化腺癌及其他小圆细胞肿瘤,预后与组织学类型有关。

Objective To reassess previously diagnosed 81 cases of gastrointestinal neuroendocrine neoplasm（GINEN）and 50 cases of gastrointestinal adenocarcinoma with neuroendocrine cell differentiation in the past 16 years based on the latest WHO concept,and to explore the clinicopathological features and pathological subtyping criteria of the remaining 90 cases of GINEN.Methods Ninety cases in tissue microarray were studied by immunohistochemistry with panels of Syn,CgA,CD56 and Ki-67 antibodies.Three pathologists reevaluated the slides separately.Results Microscopically 90 cases of GINEN were divided into three types：neuroendocrine tumor（NET,23 cases of grade 1 and 3 cases of grade 2）,neuroendocrine carcinoma（NEC,59 cases）and mixed adenoneuroendocrine carcinoma（MANEC,5 cases）.The positive expression rate of Syn,CgA and CD56 in GINEN was 78.89%,53.33% and 33.33%,respectively.The histologic categories were correlated with tumor size,invasion range,lymph node metastasis and vessel invasion significantly（P〈0.05）.Kaplan-Meier survival curves showed no significant difference in survival among patients with small cell carcinoma,mixed NEC and large cell neuroendocrine carcinoma（P〉0.05）.The 2-year survival rate for NET and NEC was 87% and 35%,respectively.Conclusion GINEN had various and complicated histopathological manifestations,but still had certain clinicopathological characteristics.The combination of Syn and CgA immunohistochemical staining is necessary for the diagnosis of GINEN.Diagnosis must be made by exclusion of poorly differentiated adenocarcinoma and other small round cell tumors.The prognosis was related with histologic categories.