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十二指肠神经内分泌肿瘤的罕见表现

A Rare Presentation of a Duodenal Neuroendocrine Tumor.

作者信息

Kaliounji Aboud, Alkoutami Sami, Farraj Michael, Kaliounji Haya, Farraj Kristen L

机构信息

Internal Medicine, State University of New York (SUNY) Downstate Medical Center, Brooklyn, USA.

Internal Medicine, St. George's University School of Medicine, St. George's, GRD.

出版信息

Cureus. 2023 Jan 13;15(1):e33747. doi: 10.7759/cureus.33747. eCollection 2023 Jan.

DOI:10.7759/cureus.33747
PMID:36788876
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9922524/
Abstract

Neuroendocrine tumors (NETs), which are a rare type of tumor, are defined as epithelial cells with predominantly neuroendocrine differentiation and consist of a spectrum of tumors emerging from stem cells throughout the body and can occur anywhere in the body. While they are rare, the incidence over the past few decades has increased. Here we present a case of a 64-year-old female who was incidentally found to have a duodenal neuroendocrine tumor. The patient initially presented to the emergency department secondary to syncope and collapse. During her trauma evaluation, an incidental lobulated soft tissue mass inferior to the distal stomach was seen on complete computed tomography (CT) scans. The surgery team was consulted for resection of the mass and an octreotide scan was performed prior to resection to further evaluate the mass and to check for any signs of metastatic disease. The octreotide scan demonstrated intense radiotracer accumulation within the duodenal mass consistent with a neuroendocrine tumor and no areas of abnormal radiotracer accumulation suspicious for metastatic disease. Pathology of the resected mass was positive for a well-differentiated neuroendocrine tumor with an organoid pattern and homogenous oval-round neoplastic cells with a salt-pepper nuclear and pseudo glandular arrangement that was well-circumscribed and partially encapsulated with negative margins. Immunohistochemistry was positive for AE ⅓, CD56, Synaptophysin, and chromogranin and negative for CD117, DOG-1, CD34, and CD45. The prevalence of NETs has increased over the years due to the improvement in diagnostic tools, such as upper gastrointestinal endoscopy. In addition to the fact that the duodenum is a rare location for such tumors, neuroendocrine tumors are also typically found in those under 50 years old. However, our patient was found to have both a duodenal mass and was over the age of 50 at the time of presentation and diagnosis. To date, a consensus on a conclusive treatment of duodenal NETs (D-NETs) has not been reached. This case brings to light the importance of further research in diagnosing and treating neuroendocrine tumors and also raises awareness for clinicians to have this in their differential.

摘要

神经内分泌肿瘤(NETs)是一种罕见的肿瘤类型,定义为主要具有神经内分泌分化的上皮细胞,由全身干细胞产生的一系列肿瘤组成,可发生于身体的任何部位。虽然它们很罕见,但在过去几十年中的发病率有所上升。在此,我们报告一例64岁女性,偶然发现患有十二指肠神经内分泌肿瘤。患者最初因晕厥和虚脱就诊于急诊科。在其创伤评估期间,完整的计算机断层扫描(CT)显示胃远端下方有一个偶然发现的分叶状软组织肿块。手术团队会诊后决定切除该肿块,并在切除前进行了奥曲肽扫描,以进一步评估肿块并检查是否有任何转移疾病迹象。奥曲肽扫描显示十二指肠肿块内有强烈的放射性示踪剂积聚,符合神经内分泌肿瘤表现,且无可疑转移疾病的放射性示踪剂异常积聚区域。切除肿块的病理检查显示为高分化神经内分泌肿瘤,呈器官样结构,肿瘤细胞均匀呈椭圆形,核呈“椒盐”样,呈假腺管排列,边界清楚,部分有包膜,切缘阴性。免疫组化结果显示AE ⅓、CD56、突触素和嗜铬粒蛋白呈阳性,CD117、DOG-1、CD34和CD45呈阴性。由于诊断工具的改进,如上消化道内镜检查,近年来NETs的患病率有所增加。除了十二指肠是此类肿瘤的罕见发病部位外,神经内分泌肿瘤通常还见于50岁以下人群。然而,我们的患者在就诊和诊断时既发现有十二指肠肿块,年龄又超过了50岁。迄今为止,对于十二指肠神经内分泌肿瘤(D-NETs)的确定性治疗尚未达成共识。该病例凸显了进一步研究神经内分泌肿瘤诊断和治疗的重要性,也提高了临床医生对此病进行鉴别诊断的意识。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/51b3/9922524/028a571af515/cureus-0015-00000033747-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/51b3/9922524/028a571af515/cureus-0015-00000033747-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/51b3/9922524/028a571af515/cureus-0015-00000033747-i01.jpg

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