Sarlomo-Rikala M, El-Rifai W, Lahtinen T, Andersson L C, Miettinen M, Knuutila S
Haartman Institute, Department of Pathology, University of Helsinki, Finland.
Hum Pathol. 1998 May;29(5):476-81. doi: 10.1016/s0046-8177(98)90063-6.
It is not uniformly agreed whether gastrointestinal stromal tumors (GISTs) are phenotypical variants of leiomyomas (cellular leiomyomas) or whether they represent a separate, genotypically definable entity. In an attempt to solve this question, we examined immunohistochemically defined leiomyomas from the esophagus and uterus, gastric schwannomas, and benign gastrointestinal stromal tumors (GIST) by comparative genomic hybridization (CGH). All 14 leiomyomas (nine esophageal, five uterine) were actin- and desmin-positive but negative for CD34 and S100-protein. Changes in DNA copy numbers were seen only in three esophageal leiomyomas. Gains were observed in chromosomes 3, 4, 5, 8, and 17, whereas losses were seen in 16p. All schwannomas were positive for S100-protein and negative for actin, desmin, and CD34. In schwannomas, the only change by CGH was a gain in 11q in one case. The benign GISTs, all from the stomach, were positive for CD34 but negative for desmin and S100-protein; two cases were positive for actin. The CGH findings in the GISTs differed markedly from those in leiomyomas and schwannomas. Ten of the 13 cases (77%) showed DNA copy number losses in 14q, and additional or other losses were found in eight cases, most often in chromosome 22 (seven cases), 15 (three cases), and 1p (two cases). Furthermore, two of the GISTs showed gains in 5q. These results indicate that phenotypically undifferentiated GISTs are also genetically different from leiomyomas and schwannomas and support their classification apart from leiomyomas.
胃肠道间质瘤(GISTs)是平滑肌瘤(细胞性平滑肌瘤)的表型变体,还是代表一个独立的、基因可定义的实体,目前尚未达成一致意见。为了解决这个问题,我们通过比较基因组杂交(CGH)对免疫组织化学定义的食管和子宫平滑肌瘤、胃神经鞘瘤以及良性胃肠道间质瘤(GIST)进行了检查。所有14例平滑肌瘤(9例食管平滑肌瘤,5例子宫平滑肌瘤)肌动蛋白和结蛋白呈阳性,但CD34和S100蛋白呈阴性。仅在3例食管平滑肌瘤中观察到DNA拷贝数变化。在染色体3、4、5、8和17上观察到增益,而在16p上观察到缺失。所有神经鞘瘤S100蛋白呈阳性,肌动蛋白、结蛋白和CD34呈阴性。在神经鞘瘤中,CGH检测到的唯一变化是1例病例中11q出现增益。所有来自胃的良性GIST,CD34呈阳性,但结蛋白和S100蛋白呈阴性;2例肌动蛋白呈阳性。GIST的CGH结果与平滑肌瘤和神经鞘瘤明显不同。13例病例中的10例(77%)在14q出现DNA拷贝数缺失,另外8例还发现了其他缺失,最常见于染色体22(7例)、15(3例)和1p(2例)。此外,2例GIST在5q出现增益。这些结果表明,表型未分化的GIST在基因上也与平滑肌瘤和神经鞘瘤不同,并支持将它们与平滑肌瘤分开分类。