Menge Franka, Jakob Jens, Kasper Bernd, Smakic Armand, Gaiser Timo, Hohenberger Peter
Division of Surgical Oncology, Mannheim University Medical Center, Medical Faculty Mannheim, University of Heidelberg, Mannheim, Germany.
Department of Surgery, Mannheim University Medical Center, Medical Faculty Mannheim, University of Heidelberg, Mannheim, Germany.
Visc Med. 2018 Oct;34(5):335-340. doi: 10.1159/000494303. Epub 2018 Oct 13.
Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the gastrointestinal tract. They constitute 1-2% of all gastrointestinal neoplasms but are the most common subtype of soft tissue sarcomas, accounting for 20-25%. In the late 1990s, GISTs were more and more recognized as a particular tumor entity. The tumors are supposed to originate from the interstitial pacemaker cells of Cajal. They are usually well circumscribed and can be located in every part of the tubular gastrointestinal tract. Most often GISTs occur in the stomach, followed by the small bowel and colon/rectum. In contrast to epithelial tumors, GISTs grow transmurally and submucosal. GISTs can be found with highly variable growth features including tumors with intraluminal, intra- or transmural, and pedunculated appearance. Here we describe the most common clinical presentation of GISTs on the basis of our 809 patients managed from 2004 to 2017. The median age of our patients was 59 years and the average size of GIST was 75 mm (range: 4 mm to 35 cm). The clinical presentation is very heterogeneous, depending on tumor site, size, and growth pattern. GISTs of the stomach is the group with the lowest rate of acute or emergency symptoms with 31%, followed by GISTs of the duodenum with 42%, whereas GISTs of the small bowel show acute symptoms in more than 50% of the cases and have an emergency surgery rate of almost 15%. Many patients are diagnosed accidentally, through screening examinations, or with latent, unspecific symptoms.
胃肠道间质瘤(GISTs)是胃肠道最常见的间叶性肿瘤。它们占所有胃肠道肿瘤的1%-2%,却是软组织肉瘤最常见的亚型,占20%-25%。在20世纪90年代后期,GISTs越来越被认为是一种特殊的肿瘤实体。这些肿瘤被认为起源于 Cajal 间质起搏细胞。它们通常边界清晰,可位于管状胃肠道的各个部位。GISTs最常发生于胃,其次是小肠和结肠/直肠。与上皮性肿瘤不同,GISTs呈跨壁和黏膜下生长。GISTs可表现出高度可变的生长特征,包括腔内、壁内或跨壁以及带蒂外观的肿瘤。在此,我们根据2004年至2017年管理的809例患者描述GISTs最常见的临床表现。我们患者的中位年龄为59岁,GIST的平均大小为75mm(范围:4mm至35cm)。临床表现非常异质性,取决于肿瘤部位、大小和生长方式。胃GISTs出现急性或紧急症状的比例最低,为31%,其次是十二指肠GISTs,为42%,而小肠GISTs在超过50%的病例中出现急性症状,急诊手术率近15%。许多患者是通过筛查检查意外诊断出来的,或者是有潜在的、非特异性症状。