胰腺多发性实性-假乳头状肿瘤2例并文献复习

Multicentric solid-pseudopapillary neoplasm of the pancreas:two cases report and literature reviews

目的报道2例临床上极罕见的胰腺多发性实性-假乳头状肿瘤(solid-pseudopapillary neoplasm,SPN),并复习文献探讨多发性SPN的临床及病理学特点。方法分析2例多发性SPN的临床表现,组织病理学以及免疫组化特点,回顾文献中报道的2例多发性SPN,并与单发性SPN进行对比。结果 4例多发性患者中女性3例、男性1例,症状体征无特异性,肿块大小不一,边界清,易囊变,肿块之间彼此完全分隔开。镜下示形态一致的肿瘤细胞围绕纤细的小血管形成特征性的假乳头状结构。肿瘤细胞表达波形蛋白、神经元特异性烯醇化酶(neuron-specific enolase,NSE)、突触素、孕酮受体、CD10及β-连环蛋白的异常核表达。患者肿瘤完全手术切除后,均长期生存。结论胰腺多发性SPN较罕见,其临床及病理学特点与单发性SPN类似。

Objective To report 2 cases of rare multicentric solid-pseudopapillary neoplasm（SPN） of the pancreas,and to discuss its clinical and pathological features by reviewing literatures.Methods The clinical features,histological and immunochemical findings of 4 multicentric SPNs,including 2 cases from literatures,were analyzed.We also compared their features with the corresponding solitary SPN.Results There were 3 females and 1 male among these 4 cases.They manifested nonspecific symptoms.Neoplasms were variable in size,well demarcated frequently with cystic degeneration,and separated from each other.Microscopically,the distinct pseudopapillae were formed by uniform neoplastic cells surrounding delicate blood vessels.Neoplastic cells usually expressed vimentin,neuron-specific enolase,synaptophysin,progesterone receptor,CD10,and with β-catenin of abormal nuclear location.The patients could achieve a long-term survival after complete surgical resection of neoplasms.Conclusions The multicentive SPN was rare,sharing similar clinicopathological features with the solitary SPN.