Korbi S, Kapanci Y, Widgren S
Ann Pathol. 1987;7(1):47-55.
Paragangliomas of the duodenum are rare. Only 40 cases, most of them corresponding to benign tumours, have been published. In this study, we report a malignant paraganglioma of the duodenum with lymph node metastasis occurring in a 73 year old woman. The essential clinical manifestations which brought the patient to surgery were abdominal pain, melaena and anemia. The surgical specimen was investigated histologically, immunohistochemically and ultrastructurally. The tumour was composed of three types of cells: epithelial, gangliocytic and spindle shaped mesenchymal cells. The first contained ultrastructurally intracytoplasmic granules bound with double membranes; they stained with monoclonal antibodies against chromogranin. Gangliocytic cells stained with monoclonal N.S.E. antibodies and the spindle cells stained with S 100 protein. The spindle cells displayed ultrastructurally glial cell characteristics, containing micro-filaments of glial type. These immunological and ultrastructural features argued in favor of the neuroendocrine cell origin of this paraganglioma.
十二指肠副神经节瘤很罕见。仅有40例病例被报道，其中大多数为良性肿瘤。在本研究中，我们报告了1例发生于一名73岁女性的十二指肠恶性副神经节瘤伴淋巴结转移。导致患者接受手术的主要临床表现为腹痛、黑便和贫血。对手术标本进行了组织学、免疫组织化学和超微结构检查。肿瘤由三种类型的细胞组成：上皮细胞、神经节样细胞和梭形间充质细胞。第一种细胞在超微结构上含有与双层膜结合的胞质内颗粒；它们用抗嗜铬粒蛋白的单克隆抗体染色。神经节样细胞用单克隆N.S.E.抗体染色，梭形细胞用S-100蛋白染色。梭形细胞在超微结构上显示出神经胶质细胞特征，含有神经胶质类型的微丝。这些免疫和超微结构特征支持了该副神经节瘤的神经内分泌细胞起源。
