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下消化道不常见的间叶性肿瘤:当你听到夜间的马蹄声时,不要忘记斑马。

Unusual Mesenchymal Tumors of the Lower Gastrointestinal Tract: When You Hear Hoofbeats in the Night, Do Not Forget the Zebras.

机构信息

Division of Pathology, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

Department of Biomedical, Surgical and Dental Sciences, University of Milan, Milan, Italy.

出版信息

Pathobiology. 2021;88(6):434-442. doi: 10.1159/000517962. Epub 2021 Oct 4.

DOI:10.1159/000517962
PMID:34814148
Abstract

INTRODUCTION

Little information about clinical presentation of mesenchymal tumors of the lower gastrointestinal (GI) tract due to their extreme heterogeneity is available for clinical management. Usually, small solitary asymptomatic polyps are accidently found during a screening colonoscopy performed for hematochezia, abdominal pain, constipation, diarrhea, and bowel obstruction. In this case series, we illustrate our experience with mesenchymal tumors of the lower GI tract, which are a group of unusual and quite challenging lesions.

CASE PRESENTATION

We retrospectively collected mesenchymal tumors of the lower GI tract in our institution (Fondazione IRCSS Ca' Granda - Ospedale Maggiore Policlinico di Milano) during the last 10 years. We reviewed the histological slides, and, when necessary, we performed immunohistochemical analyses to better characterize the tumors. A total of 99 cases were identified: 45 GISTs, 42 lipomas, 4 leiomyomas, 3 Kaposi sarcomas, 1 schwannoma, 1 ganglioneuroma, 1 hemangioma, 1 inflammatory fibroid polyp, and 1 challenging case of spindle cell melanoma. We focused on the most rare entities excluding therefore all GISTs and lipomas from re-evaluation.

CONCLUSION

Mesenchymal tumors of the lower GI tract represent a highly heterogeneous group of lesions encompassing GISTs, lipomas, smooth muscle tumors (leiomyoma and leiomyosarcoma), GI schwannomas, inflammatory fibroid polyps, solitary fibrous tumors, and other unusual spindle cell tumors. Immunohistochemistry and, in selected cases, molecular biology remain a useful tool which, in addition to a meticulous study of the morphology, helps the pathologist in the tangled jungle of differential diagnosis.

摘要

简介

由于其极度异质性,关于下胃肠道(GI)间质瘤的临床表现的信息很少,这对临床管理不利。通常,在因便血、腹痛、便秘、腹泻和肠梗阻而进行的筛查性结肠镜检查中,偶然会发现小的单发无症状息肉。在本病例系列中,我们展示了我们在下胃肠道间质瘤方面的经验,这些肿瘤是一组不常见且极具挑战性的病变。

病例介绍

我们回顾性地收集了过去 10 年来我们机构(米兰的 IRCCS 基金会 - 卡格拉大医院)的下胃肠道间质瘤。我们复习了组织学切片,并在必要时进行了免疫组织化学分析以更好地对肿瘤进行特征化。共确定了 99 例:45 例 GIST、42 例脂肪瘤、4 例平滑肌瘤、3 例卡波西肉瘤、1 例神经鞘瘤、1 例神经节瘤、1 例血管瘤、1 例炎性纤维息肉和 1 例具有挑战性的梭形细胞黑色素瘤。我们专注于最罕见的实体,因此排除了所有 GIST 和脂肪瘤进行重新评估。

结论

下胃肠道间质瘤代表一组高度异质性的病变,包括 GIST、脂肪瘤、平滑肌肿瘤(平滑肌瘤和平滑肌肉瘤)、胃肠道神经鞘瘤、炎性纤维息肉、孤立性纤维瘤和其他不常见的梭形细胞肿瘤。免疫组织化学,并且在某些情况下,分子生物学仍然是一种有用的工具,除了对形态学的细致研究外,还可以帮助病理学家在复杂的鉴别诊断中。

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