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因 Peutz-Jeghers 综合征患者的巨大神经内分泌癌引起的十二指肠套叠:病例报告和系统评价。

Duodenal intussusception due to a giant neuroendocrine carcinoma in a patient with Peutz-Jeghers syndrome: case report and systematic review.

机构信息

Department of Gastrointestinal Surgery, West China Hospital, Sichuan University, Sichuan, China.

出版信息

Eur J Gastroenterol Hepatol. 2012 Jun;24(6):722-6. doi: 10.1097/MEG.0b013e328351c1df.

DOI:10.1097/MEG.0b013e328351c1df
PMID:22356785
Abstract

Duodenal intussusception is a rare entity. To date, only a few cases have been reported in the literature. In this report, a case of duodenal intussusception due to an unusual tumor was presented and the clinical features of this entity were discussed. A 42-year-old man with Peutz-Jeghers syndrome presented with epigastric pain, vomiting, and severe anemia. Computed tomography scan revealed synchronous duodenojejunal and jejunojejunal intussusceptions. An emergency laparotomy revealed a polypoid mass originating from the lateral wall of the descending duodenum with intussusception of the distal duodenum. Histological examination demonstrated a poorly differentiated neuroendocrine carcinoma with muscularis infiltration, vascular invasion, and a Ki-67 index of 20%. A comprehensive literature search revealed 44 English reports that provided adequate descriptions of an additional 47 such cases. Clinical presentation was usually chronic and nonspecific. Diagnostic modalities included ultrasonography, upper gastrointestinal series, computed tomography, and endoscopy. Five patients were due to a non-neoplastic lesion; however, the other 43 patients were secondary to a tumor, benign in 35 cases and malignant in eight cases. Only one patient was treated by endoscopic polypectomy, whereas the remaining underwent open surgeries. Duodenal intussusception is a challenging condition due to its rarity and nonspecific presentation. It should be considered in the differential diagnosis of gastric outlet obstruction, upper gastrointestinal bleeding, pancreatitis, and obstructive jaundice.

摘要

十二指肠套叠是一种罕见的疾病。迄今为止,文献中仅报道了少数几例。本报告介绍了一例因罕见肿瘤引起的十二指肠套叠病例,并讨论了该疾病的临床特征。一位 42 岁的 Peutz-Jeghers 综合征患者因上腹痛、呕吐和严重贫血就诊。计算机断层扫描显示同时存在十二指肠空肠和空肠空肠套叠。急诊剖腹手术显示起源于降段十二指肠侧壁的息肉样肿块,伴有远端十二指肠套叠。组织学检查显示为分化差的神经内分泌癌,伴有肌层浸润、血管侵犯和 Ki-67 指数为 20%。全面的文献检索显示,有 44 篇英文报告提供了另外 47 例此类病例的充分描述。临床表现通常为慢性且非特异性。诊断方法包括超声、上消化道造影、计算机断层扫描和内镜。有 5 例患者的病因是非肿瘤性病变,而其余 43 例患者则是由肿瘤引起的,其中良性 35 例,恶性 8 例。仅 1 例患者接受了内镜息肉切除术,其余患者均接受了开放性手术。由于其罕见性和非特异性表现,十二指肠套叠是一种具有挑战性的疾病。在鉴别诊断胃出口梗阻、上消化道出血、胰腺炎和梗阻性黄疸时应考虑该病。

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