Abdelbaqi Maisoun Qassim, Tahmasbi Maryam, Ghayouri Masoumeh
Department of Anatomic Pathology, H. Lee Moffitt Cancer Center and Research Institution Tampa, Florida ; Department of Pathology and Cell Biology, University of South Florida Tampa, Florida, USA.
Int J Clin Exp Pathol. 2013 Aug 15;6(9):1948-52. eCollection 2013.
We report a case of appendicial paraganglioma in a 40 year old female who presented with acute appendicitis and underwent laparoscopic appendectomy. To the best of our knowledge this is the first reported case of appendicial gangliocytic paraganglioma with features suggestive of malignancy in the modern literature. Van Eeden S. et al. reported the first case of appendicial paraganglioma in a 47 year old man who also presented with acute appendicitis. The appendectomy specimen showed a distended appendix with thickened wall, and a 1.3 cm mucosal based yellow lesion. Microscopically this lesion was centered in the submucosa and consisted of three different cell types: (a) epithelioid cells with pale eosinophilic finely granular cytoplasm containing bland oval nucleus with stippled chromatin, that form solid nests lying in a trabecular pattern and in formations reminiscent of 'Zellballen' as seen in paragangliomas (b) second type cells have large vesicular nuclei with prominent nucleoli and abundant cytoplasm that are scattered singly, (c) third type cells with bland elongated nuclei form broad fascicle and envelop the epithelioid and ganglion cells. Immunohistochemical analysis showed the epithelioid cell nests immunoreactive for synaptophysin and the ganglion-like cells and spindle Schwann cells to be immunoreactive for S100 protein, whereas all three cells populations were negative for CAM5.2 and Pancytokeratin. We do believe that an accurate diagnosis of Gangliocytic paraganglioma (GP) of the appendix was rendered, detailed microscopic examination of doubled hematoxylin and eosinophil stained sections as well as the immunohistochemical phenotype of the three components have been undertaken to confirm the diagnosis of GP.
我们报告了一例40岁女性的阑尾副神经节瘤，该患者表现为急性阑尾炎并接受了腹腔镜阑尾切除术。据我们所知，这是现代文献中首次报道的具有恶性特征的阑尾神经节细胞性副神经节瘤病例。Van Eeden S.等人报道了第一例阑尾副神经节瘤，患者为一名47岁男性，同样表现为急性阑尾炎。阑尾切除标本显示阑尾扩张，壁增厚，有一个1.3厘米的黏膜下黄色病变。显微镜下，该病变位于黏膜下层，由三种不同的细胞类型组成：(a)上皮样细胞，胞质淡嗜酸性，细颗粒状，含有淡染的椭圆形细胞核，核染色质呈点状，形成实性巢状结构，呈小梁状排列，形成类似于副神经节瘤中所见的“细胞球”结构；(b)第二种细胞具有大的泡状核，核仁突出，胞质丰富，单个散在分布；(c)第三种细胞具有淡染的细长细胞核，形成宽束状结构，包绕上皮样细胞和神经节细胞。免疫组织化学分析显示，上皮样细胞巢对突触素呈免疫反应，神经节样细胞和梭形施万细胞对S100蛋白呈免疫反应，而所有三种细胞群体对CAM5.2和全细胞角蛋白均为阴性。我们确实认为对阑尾神经节细胞性副神经节瘤（GP）做出了准确诊断，对双重苏木精和伊红染色切片进行了详细的显微镜检查，并对三种成分的免疫组织化学表型进行了分析，以确认GP的诊断。
