Takeshi Fujita, Kinji Kamiya, Yoshiaki Takahashi, Shinichiro Miyazaki, Ichirota Iino, Hirotoshi Kikuchi, Yoshihiro Hiramatsu, Manabu Ohta, Hiroyuki Konno, Second Department of Surgery, Hamamatsu University School of Medicine, Shizuoka 431-3192, Japan.
World J Gastrointest Surg. 2013 Mar 27;5(3):62-7. doi: 10.4240/wjgs.v5.i3.62.
We report a rare case of paraganglioma that developed in the mesentery of terminal ileum. A 78-year-old woman complained of right-sided abdominal pain. Abdominal computed tomography revealed a solid heterogeneously enhanced mass in the right lower abdomen. The tumor was laparoscopically excised. The mesenteric tumor was well circumscribed, ovoid, and encapsulated and measured 3 cm × 1.5 cm × 1.5 cm. Histological examination showed a cellular neoplasm comprised of nests and groups of tumor cells separated by fibrovascular connective tissue, giving a characteristic nested Zellballen pattern. Immunohistochemically, the tumor cells were positive for chromogranin, synaptophysin, CD56, and vimentin and negative for cytokeratins, SMA, CD34, CD117/c-kit and S100. On the basis of histologic and immunohistochemical features, a diagnosis of mesenteric paraganglioma was made. The operative and postoperative courses were unremarkable, and the patient was discharged on postoperative day 7. She was doing well 1 year after the surgery with no signs of recurrence. Extra-adrenal paragangliomas most commonly develop adjacent to the aorta, particularly the area corresponding to the organ of Zuckerkandl. Mesenteric paraganglioma, as in our case, is extremely rare; only 11 cases have been reported in the literature. We herein discuss the clinical findings of these cases.
我们报告了一例罕见的发生在回肠末端肠系膜的副神经节瘤。一名 78 岁女性因右侧腹痛来诊。腹部 CT 显示右下腹部有一个实性、混杂强化的肿块。肿瘤经腹腔镜切除。肠系膜肿瘤边界清楚,呈卵圆形,被膜包裹,大小为 3cm×1.5cm×1.5cm。组织学检查显示,由巢状和肿瘤细胞团块组成的细胞性肿瘤,其间有纤维血管结缔组织分隔,具有特征性的巢状 Zellballen 模式。免疫组化染色示肿瘤细胞表达嗜铬粒素、突触素、CD56 和波形蛋白,不表达细胞角蛋白、SMA、CD34、CD117/c-kit 和 S100。根据组织学和免疫组化特征,诊断为肠系膜副神经节瘤。手术和术后过程无异常,患者术后第 7 天出院。术后 1 年,患者恢复良好,无复发迹象。肾上腺外副神经节瘤最常发生在主动脉旁,特别是在 Zuckerkandl 器官对应的区域。肠系膜副神经节瘤,如本例,极为罕见;文献中仅报道了 11 例。我们在此讨论这些病例的临床发现。