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一例低级别阑尾黏液性肿瘤与神经内分泌阑尾碰撞瘤:病例报告及文献复习

A Low-Grade Appendiceal Mucinous Neoplasia and Neuroendocrine Appendiceal Collision Tumor: A Case Report and Review of the Literature.

作者信息

Villa Massimo, Sforza Daniele, Siragusa Leandro, Guida Andrea Martina, Ciancio Manuelli Matteo, Pirozzi Brunella Maria, Pocci Marco, Palmieri Giampiero, Grande Michele

机构信息

Department of Emergency, Policlinico Tor Vergata Hospital, Rome, Italy.

Department of Surgery, Tor Vergata University of Rome, Rome, Italy.

出版信息

Am J Case Rep. 2021 Feb 23;22:e927876. doi: 10.12659/AJCR.927876.

Abstract

BACKGROUND Incidental appendiceal neoplasms account for 1-2% of appendectomies. Mucinous neoplasms and carcinoids are the most frequent lesions, with an incidence of 0.6% and 0.3-0.9%, respectively. Appendiceal collision tumors are extremely rare and result from the proliferation of 2 different cellular lines. This report describes a young woman with a collision tumor composed of a low-grade appendiceal mucinous neoplasia (LAMN) and an appendiceal neuroendocrine tumor (ANET). CASE REPORT A 31-year-old woman was admitted to our institution presenting with abdominal pain and dysuria. After ultrasound assessment of a dilated appendix with wall thickening and distension by anechogenic material, a diagnosis of acute appendicitis was made. The patient, after a period of antibiotic therapy and observation, underwent an urgent laparoscopic appendectomy due to worsening condition. Surprisingly, the histological exam revealed a Tis LAMN extending from the base of the appendix to the resection margins, and a T3 grade-1 ANET, chromogranin-A and synaptophysin-positive, with a Ki67 less than 1%. On the basis of histological examination and European Neuroendocrine Tumor Network guidelines, in light of the positive LAMN resection margin and ANET mesoappendiceal invasion, after multidisciplinary team discussion, an elective laparoscopic hemicolectomy was indicated. The patient is now in good condition following a regular 5-year follow-up. CONCLUSIONS A collision LAMN and ANET is an exceedingly rare condition. The heterogeneity of clinical presentation and lack of solid evidence seem to recommend a tailored management. Laparoscopy is a safe and useful tool in localized mass excision.

摘要

背景

偶然发现的阑尾肿瘤占阑尾切除术的1%-2%。黏液性肿瘤和类癌是最常见的病变,发病率分别为0.6%和0.3%-0.9%。阑尾碰撞瘤极为罕见,由两种不同细胞系的增殖引起。本报告描述了一名年轻女性,患有由低级别阑尾黏液性肿瘤(LAMN)和阑尾神经内分泌肿瘤(ANET)组成的碰撞瘤。病例报告:一名31岁女性因腹痛和排尿困难入院。超声检查发现阑尾扩张,壁增厚,腔内有液性暗区,诊断为急性阑尾炎。患者经一段时间抗生素治疗和观察后,因病情恶化接受了急诊腹腔镜阑尾切除术。令人惊讶的是,组织学检查显示Tis期LAMN从阑尾根部延伸至切除边缘,以及T3期1级ANET,嗜铬粒蛋白A和突触素阳性,Ki67小于1%。根据组织学检查和欧洲神经内分泌肿瘤网络指南,鉴于LAMN切除边缘阳性和ANET阑尾系膜侵犯,多学科团队讨论后,建议进行择期腹腔镜半结肠切除术。经过5年的定期随访,患者目前状况良好。结论:LAMN和ANET碰撞瘤是一种极其罕见的疾病。临床表现的异质性和缺乏确凿证据似乎建议采用个体化管理。腹腔镜检查是局部肿块切除的安全有效工具。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7d8a/7913780/b9e9274c3068/amjcaserep-22-e927876-g001.jpg

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