Grouls V, Pickartz H, Reis H E
Institut für Pathologie Mönchengladbach, Freien Universität Berlin.
Leber Magen Darm. 1987 Oct;17(5):327-33.
A mobile pedunculated polypoid tumor was endoscopically removed from the afferent jejunal loop after gastrojejunostomy of a 54-year-old patient with anamnestic evidence of intestinal bleeding. Histologically epithelial carcinoid-like as well as mesenchymal paraganglioma- and ganglioneuroma-like patterns are mixed in varying portions, characteristic for gangliocytic paraganglioma. Immunohistochemically, serotonin, neuron-specific enolase, cytokeratin, vimentin S-100 protein and neurofilament were demonstrable. Gangliocytic paragangliomas are almost exclusively observed in the second portion of the duodenum, especially around the papilla Vateri and only two have previously been reported in the jejunum. The histogenesis of the tumors is unclear, but they may probably be either hamartomas, hyperplastic or neoplastic proliferations of so called endodermal-neuroectodermal complexes. Although gangliocytic paragangliomas contain a carcinoid-like component, they behave in a benign fashion, and metastases or recidives have not been noticed. Tumors with a pedicle may be endoscopically removed without complications.
一名54岁有肠道出血既往史的患者在胃空肠吻合术后,经内镜从空肠输入袢切除了一个带蒂的息肉样移动性肿瘤。组织学上,上皮类癌样以及间叶性副神经节瘤样和神经节神经瘤样模式以不同比例混合,这是神经节细胞性副神经节瘤的特征。免疫组化显示,血清素、神经元特异性烯醇化酶、细胞角蛋白、波形蛋白、S-100蛋白和神经丝均呈阳性。神经节细胞性副神经节瘤几乎仅见于十二指肠第二部,尤其是在 Vater 乳头周围,此前仅有两例报道发生于空肠。肿瘤的组织发生尚不清楚,但它们可能是所谓内胚层-神经外胚层复合体的错构瘤、增生性或肿瘤性增殖。虽然神经节细胞性副神经节瘤含有类癌样成分,但它们表现为良性,未发现转移或复发。有蒂的肿瘤可经内镜切除而无并发症。