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[十二指肠神经节细胞性副神经节瘤1例]

[A case of gangliocytic paraganglioma in duodenum].

作者信息

Kim Jie Hyun, Kim Hee Man, Song Si Young, Kim Yoon Jae, Hahn Chang Hoon, Park Seung Woo, Chung Jae Bock, Kang Jin Kyung, Lee Woo Jung, Cho Nam Hoon

机构信息

Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.

出版信息

Korean J Gastroenterol. 2004 Jan;43(1):47-51.

Abstract

Duodenal gangliocytic paraganglioma derived from neural crest is a peculiar neuroendocrine tumor. It is incidentally found during radiographic studies or due to gastrointestinal hemorrhage caused by frequent ulceration of the overlying mucosa. Most lesions are pedunculated and submucosal with distinctive histology consisting of endocrine cells, ganglion cells and spindle-shaped Schwann cells. We experienced a duodenal gangliocytic paraganglioma in a 40-year-old woman presenting with episodes of melena. Esophagogastroduodenoscopy revealed a submucosal ulcerated tumor in the second portion of duodenum and a biopsy confirmed gangliocytic paraganglioma. The tumor was enucleated through a duodenotomy. It's size was 5.5 X 3 X 1.5 cm sized and revealed positive cellular reaction for chromogranin, synaptophysin, neuron-specific enolase, and neurofilament by immunohistochemistry. The majority of the reported duodenal gangliocytic paraganglioma were of benign nature. Therefore, radical surgery or a lymph node dissection could be avoided if that disease was confirmed. However, thorough investigation for lymph node metastasis and postoperative follow-up are needed. We report the first case of a duodenal gangliocytic paraganglioma in Korea with a review of literature.

摘要

源自神经嵴的十二指肠节细胞性副神经节瘤是一种特殊的神经内分泌肿瘤。它通常在影像学检查时偶然发现,或因覆盖黏膜频繁溃疡导致胃肠道出血而被发现。大多数病变有蒂且位于黏膜下,具有独特的组织学特征,由内分泌细胞、神经节细胞和梭形雪旺细胞组成。我们遇到一名40岁女性患有十二指肠节细胞性副神经节瘤,表现为黑便发作。食管胃十二指肠镜检查发现十二指肠第二部有一个黏膜下溃疡性肿瘤,活检证实为节细胞性副神经节瘤。通过十二指肠切开术将肿瘤摘除。其大小为5.5×3×1.5厘米,免疫组化显示嗜铬粒蛋白、突触素、神经元特异性烯醇化酶和神经丝呈阳性细胞反应。大多数报道的十二指肠节细胞性副神经节瘤为良性。因此,如果确诊该病,可避免根治性手术或淋巴结清扫。然而,需要对淋巴结转移进行全面检查并进行术后随访。我们报告韩国首例十二指肠节细胞性副神经节瘤病例并复习文献。

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