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非胃肠道间质瘤,胃肠道间叶性肿瘤:肿瘤遗传学、病理学和影像学表现的综述。

Non-gastrointestinal stromal tumor, mesenchymal neoplasms of the gastrointestinal tract: a review of tumor genetics, pathology, and cross-sectional imaging findings.

机构信息

University of Texas MD Anderson Cancer Center, Houston, USA.

Departments of Radiology, New York University School of Medicine, New York, USA.

出版信息

Abdom Radiol (NY). 2024 May;49(5):1716-1733. doi: 10.1007/s00261-024-04329-1. Epub 2024 May 1.

Abstract

There is a diverse group of non-gastrointestinal stromal tumor (GIST), mesenchymal neoplasms of the gastrointestinal (GI) tract that demonstrate characteristic pathology and histogenesis as well as variable imaging findings and biological behavior. Recent advancements in tumor genetics have unveiled specific abnormalities associated with certain tumors, influencing their molecular pathogenesis, biology, response to treatment, and prognosis. Notably, giant fibrovascular polyps of the esophagus, identified through MDM2 gene amplifications, are now classified as liposarcomas. Some tumors exhibit distinctive patterns of disease distribution. Glomus tumors and plexiform fibromyxomas exhibit a pronounced affinity for the gastric antrum. In contrast, smooth muscle tumors within the GI tract are predominantly found in the esophagus and colorectum, surpassing the incidence of GISTs in these locations. Surgical resection suffices for symptomatic benign tumors; multimodality treatment may be necessary for frank sarcomas. This article aims to elucidate the cross-sectional imaging findings associated with a wide spectrum of these tumors, providing insights that align with their histopathological features.

摘要

存在一组多样化的非胃肠道间质瘤(GIST),即胃肠道(GI)的间叶性肿瘤,它们具有特征性的病理学和组织发生以及可变的影像学表现和生物学行为。肿瘤遗传学的最新进展揭示了与某些肿瘤相关的特定异常,影响了它们的分子发病机制、生物学、对治疗的反应和预后。值得注意的是,通过 MDM2 基因扩增鉴定的食管巨大纤维血管性息肉现在被归类为脂肪肉瘤。一些肿瘤表现出独特的疾病分布模式。血管球瘤和丛状纤维黏液瘤明显倾向于胃窦。相比之下,胃肠道平滑肌肿瘤主要发生在食管和结直肠,在这些部位的发生率超过 GIST。有症状的良性肿瘤仅需手术切除;而明确的肉瘤可能需要多模式治疗。本文旨在阐明与这些广泛肿瘤相关的各种影像学表现,提供与它们的组织病理学特征相一致的见解。

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