Gastrointestinal stromal tumors and leiomyosarcomas in the colon - A clinicopathologic, immunohistochemical, and molecular genetic study of 44 cases
Gastrointestinal stromal tumors and leiomyosarcomas in the colon - A clinicopathologic, immunohistochemical, and molecular genetic study of 44 cases
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DOI：
10.1097/00000478-200010000-00003
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发表时间：
2000-10-01
影响因子：
5.6
通讯作者：
Lasota, J
中科院分区：
文献类型：
--
作者：
Miettinen, M;Sarlomo-Rikala, M;Lasota, J
胃肠道间质瘤（gist）是胃肠道特有的间质肿瘤，已经在胃和小肠中得到了明确的定义，但尚未被广泛记录或与结肠中真正的平滑肌肿瘤进行对比。本研究旨在确定结肠（不包括直肠）的胃肠道间质瘤的临床病理特征，并将其与同一部位的平滑肌肉瘤（lms）进行比较。我们分析了来自赫尔辛基大学武装部队病理研究所和Haartman研究所档案的37例结肠gist和7例lms。gist主要发生在50岁以上的成年人中（中位数为67岁），大多数组织学上是恶性的；偶然发现4个小的良性肿瘤（小于或等于1厘米），另外10个有丝分裂活性最小（每50个高倍视场有5个或更少的有丝分裂）。结肠胃肠道间质瘤为典型的跨壁肿瘤，常伴有腔内和外膨出成分。组织学上，它们通常表现为梭形细胞（92%），而上皮样细胞占8%。大多数肿瘤（25例中的19例）CD117 （KIT）和CD34（27例中的16例）阳性；6个肿瘤共表达a-平滑肌肌动蛋白和CD117；没有显示desmin或S-100蛋白。14例结肠gist中有5例（36%）外显子11发生C-kit突变。偶发小肿瘤的患者无一复发，而10例肿瘤大于1cm但有丝分裂活性极低的患者中有2例死于肝转移。肿瘤大于1cm且每50个高倍视场出现5个以上有丝分裂的患者几乎全部死亡；一半有转移的迹象。lms为典型的腔内膨出的息肉样肿块，在组织学上与分化的平滑肌细胞相似。他们发生在5名男性和2名女性中位年龄为61岁。大多数lms在组织学上表现为高级别，并显示平滑肌肌动蛋白，desmin或两者兼而有之。所有患者CD34和CD117均为阴性，且缺乏c-kit突变。7名患者中有5人死于疾病，2人长期存活，尽管有丝分裂活性很高。这些结果表明，kit阳性的胃肠道间质瘤比结肠lms更常见，这些肿瘤组具有临床病理差异，可以将其分开。
Gastrointestinal stromal tumors (GISTs), mesenchymal tumors largely specific for the gastrointestinal tract, have been well defined in the stomach and small intestine, but have not been extensively documented or contrasted with true smooth muscle tumors in the colon. This study was undertaken to determine the clinicopathologic features of GISTs of the colon, excluding the rectum, and to compare them with leiomyosarcomas (LMSs) of the same location. A total of 37 colonic GISTs and seven LMSs from the files of the Armed Forces Institute of Pathology and the Haartman Institute of the University of Helsinki were analyzed. The GISTs occurred predominantly in adults older than 50 years of age (median, 67 yrs), and most were histologically malignant; four small benign tumors (less than or equal to 1 cm) were incidentally detected, and 10 others had minimal mitotic activity (five or fewer mitoses per 50 high-power fields). The colonic GISTs were typically transmural tumors with frequent intraluminal and outward bulging components. Histologically, they usually showed a spindle cell pattern (92%), whereas 8% were epithelioid. Most tumors (19 of 25) were positive for CD117 (KIT) and for CD34 (16 of 27); six tumors coexpressed a-smooth muscle actin and CD117; none showed desmin or S-100 protein. C-kit mutations in exon 11 were seen in 5 (36%) of 14 colonic GISTs. None of the patients with incidental small tumors had a recurrence, whereas 2 of 10 patients with tumors larger than 1 cm but minimal mitotic activity died of the disease with liver metastasis. Nearly all patients whose tumor was larger than 1 cm and showed more than five mitoses per 50 high-power fields died of disease; half had evidence of metastasis. LMSs were typically intraluminally bulging, polypoid masses that showed a histologic likeness to differentiated smooth muscle cells. They occurred in five men and two women with a median age of 61 years. Most LMSs were high-grade histologically and showed smooth muscle actin, desmin, or both. All were negative for CD34 and CD117 and lacked c-kit mutations. Five of the seven patients died of disease, and two had a long-term survival, despite high mitotic activity. These results show that KIT-positive GISTs are more common than LMSs of the colon, and these tumor groups have clinicopathologic differences that warrant their separation.