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十二指肠神经节细胞性副神经节瘤的治疗与管理:一例报告

Treatment and management of duodenal gangliocytic paraganglioma: A case report.

作者信息

Xiao Na, Zhu Sumin, Liu Huan, Li Longfei, Shi Lihong

机构信息

Department of Gastroenterology, The Second Affiliated Hospital of Xuzhou Medical University, Xuzhou, Jiangsu 221002, P.R. China.

Department of Oncology, Xuzhou Cancer Hospital, Xuzhou, Jiangsu, 221002, P.R. China.

出版信息

Exp Ther Med. 2024 Sep 19;28(6):435. doi: 10.3892/etm.2024.12723. eCollection 2024 Dec.

Abstract

Gangliocytic paraganglioma (GP) is a rare neuroendocrine tumor primarily found in the duodenum, most commonly in the second and third sections of the duodenum. Diagnosis of GP is based on its distinctive histopathological characteristics, which include three types of tumor cells in varying proportions: i) Epithelioid, ii) spindle-like and iii) ganglion-shaped cells. The distribution of the three tumor cell components varies from case to case and a patient may be easily misdiagnosed if one of the components is predominant. Endoscopic submucosal dissection (ESD) or surgical resection is the ideal treatment for duodenal GP (DGP); however, biotherapy, nuclide therapy, chemotherapy, targeted therapy and immunotherapy can be selected individually for patients with postoperative recurrence, metastasis or not suitable for surgery. In the present study, a male patient with DGP experienced recurrence after ESD surgery, and so received octreotide (Novartis; 30 mg/28 days) for 12 consecutive cycles. The patient had no further symptoms of gastrointestinal bleeding and no new lesions or metastases were observed after 47 months of follow-up.

摘要

节细胞性副神经节瘤(GP)是一种罕见的神经内分泌肿瘤,主要发生于十二指肠,最常见于十二指肠的第二和第三段。GP的诊断基于其独特的组织病理学特征,其中包括三种比例不同的肿瘤细胞:i)上皮样细胞、ii)梭形细胞和iii)神经节样细胞。三种肿瘤细胞成分的分布因病例而异,如果其中一种成分占主导,患者很容易被误诊。内镜黏膜下剥离术(ESD)或手术切除是十二指肠GP(DGP)的理想治疗方法;然而,对于术后复发、转移或不适合手术的患者,可单独选择生物治疗、核素治疗、化疗、靶向治疗和免疫治疗。在本研究中,一名DGP男性患者在ESD手术后复发,因此连续12个周期接受奥曲肽(诺华公司;30mg/28天)治疗。随访47个月后,患者无进一步胃肠道出血症状,未观察到新的病变或转移。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/546a/11443592/9b01b2a99800/etm-28-06-12723-g00.jpg

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