Barbareschi M, Frigo B, Aldovini D, Leonardi E, Cristina S, Falleni M
3rd Chair of Morbid Anatomy and Histopathology, School of Medicine, University of Milan, Italy.
Virchows Arch A Pathol Anat Histopathol. 1989;416(1):81-9. doi: 10.1007/BF01606473.
A case of gangliocytic paraganglioma (GP) of the ampulla of Vater is reported and the literature reviewed, with special attention to immunohistochemical studies. The present case, which occurred in a 56-year-old woman, shows the typical histological admixture of epithelioid, ganglion and spindle cells. Immunohistochemistry reveals strong reactivity for synaptophysin, Leu-7, somatostatin, S-100 protein and vimentin. A few ganglion cells are reactive for neurofilaments. Chromogranin A, myelin basic protein, desmin and cytokeratin are absent. Immunohistochemical data from literature regarding the cytoskeletal composition of GPs are not unequivocal: cytokeratin and neurofilament positivity is reported by some authors and denied by others. More uniformity is reported concerning the peptides produced by GPs: somatostatin and pancreatic polypeptide are the most frequently found antigens, followed by serotonin. General neuroendocrine markers like neuron specific enolase and protein gene product 9.5 are always positive, whereas chromogranins are rarely found. S-100 protein is always positive in the spindle cell component. Our data are in keeping with those previously reported and add the diffuse positivity for the Leu-7 antigen and the positivity of ganglion cells for synaptophysin. The nature of the tumour is still a matter of debate and it is difficult to agree with either of the proposed hypotheses--hamartoma/choristoma versus true neoplasm. However the recent reports of the occasional malignant evolution of GPs may support their true neoplastic nature.
本文报告了一例发生于 Vater 壶腹的神经节细胞性副神经节瘤（GP），并对相关文献进行了综述，特别关注免疫组织化学研究。本病例发生在一名 56 岁女性身上，显示出上皮样细胞、神经节细胞和梭形细胞典型的组织学混合。免疫组织化学显示突触素、Leu-7、生长抑素、S-100 蛋白和波形蛋白呈强阳性反应。少数神经节细胞对神经丝有反应。嗜铬粒蛋白 A、髓鞘碱性蛋白、结蛋白和细胞角蛋白均为阴性。关于 GP 细胞骨架组成的文献中的免疫组织化学数据并不明确：一些作者报告细胞角蛋白和神经丝呈阳性，而另一些作者则予以否认。关于 GP 产生的肽类报道更为一致：生长抑素和胰多肽是最常发现的抗原，其次是血清素。一般神经内分泌标志物如神经元特异性烯醇化酶和蛋白基因产物 9.5 总是呈阳性，而嗜铬粒蛋白很少被发现。S-100 蛋白在梭形细胞成分中总是呈阳性。我们的数据与先前报道的数据一致，并增加了 Leu-7 抗原的弥漫性阳性和神经节细胞对突触素的阳性。肿瘤的性质仍然存在争议，很难认同所提出的两种假设中的任何一种——错构瘤/迷离瘤与真正的肿瘤。然而，最近关于 GP 偶尔发生恶性演变的报道可能支持其真正的肿瘤性质。
