节细胞性副神经节瘤的影像表现及临床病理分析(附3例报告并文献复习)

Pathological and Imaging Features of Gangliocytic Paraganglioma(3 cases Report and Literature Review)

目的探讨节细胞性副神经节瘤的影像表现及其临床病理学特点。资料与方法结合临床病理特征及文献,回顾性分析3例经手术病理证实的节细胞性副神经节瘤的影像表现。3例均行全消化道钡剂造影及CT平扫与增强。结果 CT检查示肿块分别位于十二指肠降段、上段空肠及中段空肠,3例均表现为肠腔内软组织密度肿块,肿块最大直径1.6～4.9 cm。3例病灶均未见钙化、出血及包膜。增强后上段空肠病灶呈明显周边强化,中央见无强化区,周围见肿大淋巴结;余2例表现为明显均匀强化。上段空肠病灶钡剂造影表现为局部肠管扩张,肠管内见一较大充盈缺损影,局部黏膜变平,肠管柔软,蠕动正常;余2例钡剂造影未见异常。3例均位于黏膜下,肿块实性质软,切面见丰富血管网,未见包膜。镜下全部肿块均由上皮样细胞、神经节样细胞及梭形细胞三种细胞构成。免疫组织化学标记上皮样细胞均表达嗜铬粒素A(CgA)、突触素(Syn)及神经元特异性醇化酶(NSE),1例亦表达细胞角蛋白(CK);梭形细胞均表达神经丝蛋白(NF)及S-100蛋白;神经节样细胞表达NF、NSE。结论节细胞性副神经节瘤的影像表现有一定特点,但缺乏特异性,确诊需结合组织病理学及免疫组织化学检查。

Objective To investigate the pathological and imaging features of gangliocytic paraganglioma.Materials and Methods Combining the clinical manifestations,pathological features with literature review,imaging findings of three gangliocytic paraganglioma were analyzed retrospectively.All of three cases received CT plain and enhanced scan and barium gastroenterography.Results Three cases were respectively located at the second portion of the duodenum and superior segment of jejunum and middle segment of jejunum.All cases were presented as intraluminal soft tissues masses and were 1.6-4.9 cm in maximum diameter,no calcification,hemorrhage and envelope were seen in all patients.Marked periphery enhancement was seen in the mass of superior segment of jejunum,center of the lesion was not enhanced,lymphnodes were seen around mass.The other two cases showed obviously homogeneous enhancement.The lesion of superior segment of jejunum showed local intestinal canal to expand and had a major intraluminal filling defect and local mucous membrane flat and intestinal wall soft on barium gastroenterography,the other two cases had normal findings on barium gastroenterography.All cases were located in submucosa with solid texture soft,cut specimen showed prominent vascular network without envelope.All lesions were composed of three type of cells： epithelioid cells,ganglion cells,spindle cells on photomicrograph.All the epithelial cells were positive for CgA,Syn,NSE,one epithelial cells also expressed CK;the spindle cells were positive for NF,S 100;the ganglion cells were positive for NF,NSE on immunohistochemical examination.Conclusion Gangliocytic paraganglioma is lack of imaging features,definite diagnosis depends on histopathologic and immunohistochemical results.