Nishida T, Nakamura J, Taniguchi M, Hirota S, Ito T, Kitamura Y, Matsuda H
Dept. of Surgery, Osaka University Graduate School of Medicine, Yamada-oka, Suita, Japan.
J Exp Clin Cancer Res. 2000 Dec;19(4):417-25.
Stromal tumors in the gastrointestinal (GI) tract consist of myogenic tumors, neurogenic tumors and gastrointestinal stromal tumors (GISTs). Mutations in the c-kit gene have been found in GISTs, and GISTs with c-kit mutations showed aggressive clinical behavior and histological features. In the present study, we classified stromal tumors into four groups according to histological differentiation and c-kit mutation: myogenic tumors, neurogenic tumors, c-kit mutation (-) GISTs and c-kit mutation (+) GISTs, and examined their clinicopathological importance and validity using data obtained from 125 patients with gastric stromal tumors. There was no difference in preoperative symptoms and signs among the four groups. GISTs with c-kit mutations were large and showed invasion into neighboring structures compared with the other tumors, indicating the clinically aggressive features of mutation (+) GISTs. In histological examinations, c-kit mutation (+) GISTs were higher in cellularity (P < 0.0001) and mitotic cell count (P = 0.0086), and showed frequent histological necrosis (P = 0.0058) and hemorrhage (P = 0.0170), and consequently, were higher in histological grade (P = 0.0001). In prognostic analyses, overall, cause-specific and disease-free survival of patients in the mutation (+) GIST group was the poorest among the four groups. No significant differences were found among the other three groups of myogenic tumors, neurogenic tumors and c-kit mutation (-) GISTs, indicating a similar aggressiveness in clinical presentation and histological features. Thus, this classification is considered to be clinically and pathologically important in the diagnosis of gastric stromal tumors.
胃肠道（GI）间质瘤由肌源性肿瘤、神经源性肿瘤和胃肠道间质瘤（GIST）组成。在GIST中发现了c-kit基因突变，具有c-kit基因突变的GIST表现出侵袭性的临床行为和组织学特征。在本研究中，我们根据组织学分化和c-kit基因突变将间质瘤分为四组：肌源性肿瘤、神经源性肿瘤、c-kit基因突变（-）GIST和c-kit基因突变（+）GIST，并使用从125例胃间质瘤患者获得的数据检查了它们的临床病理重要性和有效性。四组之间术前症状和体征无差异。与其他肿瘤相比，具有c-kit基因突变的GIST体积较大，且显示出对邻近结构的侵犯，表明突变（+）GIST具有临床侵袭性特征。在组织学检查中，c-kit基因突变（+）GIST的细胞密度更高（P < 0.0001），有丝分裂细胞计数更高（P = 0.0086），并频繁出现组织学坏死（P = 0.0058）和出血（P = 0.0170），因此，组织学分级更高（P = 0.0001）。在预后分析中，总体而言，突变（+）GIST组患者的总生存期、病因特异性生存期和无病生存期在四组中最差。在肌源性肿瘤、神经源性肿瘤和c-kit基因突变（-）GIST这其他三组之间未发现显著差异，表明它们在临床表现和组织学特征方面具有相似的侵袭性。因此，这种分类在胃间质瘤的诊断中被认为具有临床和病理重要性。
