Tornóczky T, Kálmán E, Jáksó P, Méhes G, Pajor L, Kajtár G G, Battyány I, Davidovics S, Sohail M, Krausz T
Department of Pathology, University Medical School of Pécs, Hungary.
J Clin Pathol. 2001 Mar;54(3):241-5. doi: 10.1136/jcp.54.3.241.
Solid and papillary epithelial neoplasm (SPEN) is an uncommon pancreatic tumour. Very rarely it has also been described outside the pancreas, usually arising from heterotopic pancreatic tissue. This report summarises all the published extrapancreatic SPENs and documents the sixth such case arising from heterotopic pancreatic tissue of the transverse mesocolon in a 15 year old girl.
METHODS/RESULTS: Histological and immunohistochemical examination revealed typical papillary and solid areas composed of columnar, cuboidal, and round cells, which were focally positive for vimentin, cytokeratin, neurone specific enolase, carcinoembryonic antigen, alpha1-antitrypsin, alpha1-antichymotrypsin, and negative for neuroendocrine markers (neurofilament, PGP 9.5, chromogranin A, synaptophysin, and S100), p53, and oestrogen and progesterone receptors. Electron microscopy showed scant zymogen but no neurosecretory granules. In agreement with the flow cytometric result s of diploidy, comparative genomic hybridisation (CGH) did not reveal loss or gain of genetic material, and the in situ hybridisation analysis of the RB1 and p53 genes revealed no abnormality in the 13q and 17p arms.
Immunohistochemical and electron microscopic data support exocrine differentiation. The CGH and the flow cytometric results suggest a subtle, yet unknown genetic change, rather than a large genetic alteration. RB1 and p53 in situ hybridisation ruled out the role of deletion at these sites in the pathogenesis of SPEN. Interestingly, review of the published and the present heterotopic pancreatic SPENs identified the mesocolon as the most common anatomical site (four of six), despite the very rare occurrence of ectopic pancreatic tissue at this site.
实性假乳头性上皮性肿瘤(SPEN)是一种罕见的胰腺肿瘤。极少数情况下,它也在胰腺外被发现,通常起源于异位胰腺组织。本报告总结了所有已发表的胰腺外SPEN病例,并记录了第6例发生于一名15岁女孩横结肠系膜异位胰腺组织的此类病例。
方法/结果:组织学和免疫组化检查显示,典型的乳头和实性区域由柱状、立方状和圆形细胞组成,波形蛋白、细胞角蛋白、神经元特异性烯醇化酶、癌胚抗原、α1-抗胰蛋白酶、α1-抗糜蛋白酶呈局灶性阳性,神经内分泌标志物(神经丝、PGP 9.5、嗜铬粒蛋白A、突触素和S100)、p53以及雌激素和孕激素受体呈阴性。电子显微镜显示酶原稀少,但无神经分泌颗粒。与二倍体的流式细胞术结果一致,比较基因组杂交(CGH)未发现遗传物质的缺失或增加,RB1和p53基因的原位杂交分析显示13q和17p臂无异常。
免疫组化和电子显微镜数据支持外分泌分化。CGH和流式细胞术结果提示存在一种细微但未知的基因变化,而非大的基因改变。RB1和p53原位杂交排除了这些位点的缺失在SPEN发病机制中的作用。有趣的是,回顾已发表的和本研究中的异位胰腺SPEN病例发现,尽管该部位异位胰腺组织非常罕见,但结肠系膜是最常见的解剖部位(6例中有4例)。