Gastrointestinal stromal tumors, intramural leiomyomas, and leiomyosarcomas in the rectum and anus - A clinicopathologic, immunohistochemical, and molecular genetic study of 144 cases
Gastrointestinal stromal tumors, intramural leiomyomas, and leiomyosarcomas in the rectum and anus - A clinicopathologic, immunohistochemical, and molecular genetic study of 144 cases
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DOI：
10.1097/00000478-200109000-00002
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发表时间：
2001-09-01
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;Furlong, M;Lasota, J
胃肠道间质瘤（GIST），特异性的KIT阳性的胃肠道间质肿瘤，已零星报道在直肠，但很少有临床病理系列。在这项研究中，我们分析了133肛门直肠GIST，3壁间平滑肌瘤（LM），8平滑肌肉瘤（LMS）的临床病理特征，从武装部队病理研究所和哈特曼研究所的文件赫尔辛基大学。96例GIST被记录为KIT阳性，另外3例为CD 34阳性。34例肿瘤因其与KIT或CD 34阳性病例的组织学相似性而被纳入。GIST特异性c-kit基因突变，主要在外显子11，记录在29例（62%）的18。GIST发生于年龄范围为17-90岁（中位数60岁）的成人中，男性占显著优势（71%）。肿瘤的范围从小的无症状的壁内结节到大的肿块，肿块突入骨盆引起疼痛、直肠出血或梗阻。他们大多是高度细胞梭形细胞肿瘤;四个肿瘤有上皮样形态。肿瘤共表达CD 34和KIT，平滑肌肌动蛋白或结蛋白很少阳性，S-100蛋白从未阳性。肿瘤直径>5 cm、核分裂相数超过5个/50个高倍视野（HPF）的患者（n = 31）中，70%死于疾病，而只有1个肿瘤
Gastrointestinal stromal tumors (GISTs), the specific KIT-positive mesenchymal tumors of the gastrointestinal tract, have been sporadically reported in the rectum, but there are few clinicopathologic series. In this study we analyzed the clinicopathologic features of 133 anorectal GISTs, 3 intramural leiomyomas (LMs), and 8 leiomyosarcomas (LMSs) from the files of the Armed Forces Institute of Pathology and the Haartman Institute of the University of Helsinki. Ninety-six GISTs were documented as KIT-positive and three additional ones as CD34-positive. Thirty-four tumors were included by their histologic similarity to KIT- or CD34-positive cases. GIST-specific c-kit gene mutations, mostly in exon 11, were documented in 18 of 29 cases (62%). The GISTs occurred in adults with the age range of 17-90 years (median 60 years) with a significant male predominance (71 %). The tumors ranged from small asymptomatic intramural nodules to large masses that bulged into pelvis causing pain, rectal bleeding, or obstruction. They were mostly highly cellular spindle cell tumors; four tumors had an epithelioid morphology. The tumors coexpressed CD34 and KIT and were rarely positive for smooth muscle actin or desmin and never for S-100 protein. Seventy percent of patients with tumors >5 cm with more than 5 mitoses/50 high power fields (HPF) (n = 31) died of disease, whereas only one tumor