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导致1型多发性内分泌腺瘤病诊断的胰腺神经内分泌肿瘤

Pancreatic Neuroendocrine Tumor Leading to a Diagnosis of Multiple Endocrine Neoplasia Type 1.

作者信息

Hirakawa Noriyuki, Kitamura Katsuya, Yamamoto Kei, Tadokoro Kenichi, Akita Yasunosuke, Uemura Jun, Yamanishi Fumito, Abe Masakazu, Nakatsugawa Munehide, Itoi Takao

机构信息

Department of Gastroenterology and Hepatology Tokyo Medical University Hachioji Medical Center Tokyo Japan.

Department of Pathology Tokyo Medical University Hachioji Medical Center Tokyo Japan.

出版信息

DEN Open. 2025 Jun 6;6(1):e70160. doi: 10.1002/deo2.70160. eCollection 2026 Apr.

Abstract

Pancreatic neuroendocrine neoplasms are rare but occasionally encountered. They are generally highly vascularized solid tumors, often round in shape with clear boundaries, defined contours, and a homogeneous internal structure. However, they can also present with atypical features, such as cystic degeneration, hemorrhage, calcification, and fibrosis, making diagnosis difficult in some cases. They are also known as comorbidities of multiple endocrine neoplasia type 1 (MEN1). This report describes a case in which endoscopic ultrasound (EUS) led to a diagnosis of MEN1. A 50-year-old man was referred to our hospital for examination of a mass in the pancreatic body. An EUS-guided fine-needle biopsy was performed, and a histological diagnosis of neuroendocrine tumor (NET) was made. In addition, the NET was also identified in the duodenum. Serum calcium and parathyroid hormone levels were elevated. Examination of the parathyroid and pituitary glands revealed concurrent hyperparathyroidism and a pituitary adenoma, confirming the diagnosis of MEN1, including a NET in the duodenum.

摘要

胰腺神经内分泌肿瘤罕见但偶尔可见。它们通常是血管高度丰富的实体瘤,形态多为圆形,边界清晰,轮廓规整,内部结构均匀。然而,它们也可能呈现非典型特征,如囊性变、出血、钙化和纤维化,这使得某些病例的诊断困难。它们也被认为是1型多发性内分泌腺瘤病(MEN1)的合并症。本报告描述了一例经内镜超声(EUS)诊断为MEN1的病例。一名50岁男性因胰体部肿物来我院检查。进行了EUS引导下细针穿刺活检,组织学诊断为神经内分泌肿瘤(NET)。此外,在十二指肠也发现了NET。血清钙和甲状旁腺激素水平升高。对甲状旁腺和垂体的检查发现同时存在甲状旁腺功能亢进和垂体腺瘤,确诊为MEN1,包括十二指肠NET。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/99a2/12143420/32c206ff5822/DEO2-6-e70160-g002.jpg

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