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胃肠道间质瘤76例的临床病理及免疫组织化学特征
作者姓名:Hou Y  Wang J  Zhu X  Du X  Sun M  Zheng A
作者单位:200032,上海,复旦大学附属肿瘤医院病理科
基金项目:上海市科委基金资助项目 (9741190 2 5 )
摘    要:目的:探讨胃肠道间质瘤(GIST)的临床表现、病理组织形态学和免疫组织化学特点及良恶性参考指标。方法:用CD117、CD34等抗体,通过免疫组织化学EnVision法对原发性消化道间叶源性肿瘤进行研究,确诊76例GIST,并结合随访资料,对其生物学行为进行分析。结果:本组GIST均为成年人,年龄32-81岁(平均54岁),男性39例,女性37例,发生于胃34例,小肠23例,结肠2例,直肠17例,最常见症状为腹部肿块、腹部胀痛不适及消化道出血。黏膜下生长者3例,浆膜外生长者25例,余48例主要位于肌壁。大体上,肿瘤较易出现出血、坏死、囊性变等继发性改变。镜下观察,梭形细胞型46例,上皮样细胞型9例,梭形/上皮样细胞混合型21例,呈交叉束状、弥漫片状、栅栏状、漩涡状、小巢状、器官样及假菊形团样排列,瘤细胞胞质空亮、淡伊红、轻至中度嗜伊红及略嗜碱,核梭形、卵圆、圆形或镰状。CD117和CD34多为弥漫强阳性,阳性率分别为98.7%、68.4%,α-平滑肌肌动蛋白( α-SMA)、肌特异性肌动蛋白、S-100、蛋白基因产物9.5呈片状或局灶阳性,阳性率分别为25.0%、19.7%、23.7%、17.1%,波形蛋白均阳性,结蛋白、胶质纤维酸性蛋白、神经丝蛋白均阴性。76例GIST中,良性9例,交界性19例,恶性48例。良性及交界性获访20例均健在,恶性组获访34例,10例无瘤生存,10例复发及转移,14例死亡。各组之间术中黏连、瘤体直径大于5cm、核分裂象大于5/50HPF差异有显著性意义,肿瘤性坏死、核分裂象大于10/50HPF、细胞密集明显异型者均在恶性组。恶性组内,肿瘤性坏死或核分裂象大于5/50HPF者3年生存率的差异有显著性意义。结论:GISF好发于中老年人,肿瘤细胞形态多变,排列结构多样;免疫组织化学特征为CD117、CD34阳性,结蛋白阴性;除转移和浸润外,肿瘤性坏死、核分裂象大于10/50HPF、细胞密集明显异型等提示恶性,此外,术中粘连、瘤体直径大于5cm、核分裂象大于5/50HPF可作为良恶性参考指标。

关 键 词:胃肠肿瘤  间叶瘤  病理学  原癌基因蛋白质c-kit类  CD34抗原  间质瘤  GIST  免疫组织化学
修稿时间:2001年4月11日

A clinicopathologic and immunohistochemical study on 76 cases of gastrointestinal stromal tumors
Hou Y,Wang J,Zhu X,Du X,Sun M,Zheng A.A clinicopathologic and immunohistochemical study on 76 cases of gastrointestinal stromal tumors[J].Chinese Journal of Pathology,2002,31(1):20-25.
Authors:Hou Yingyong  Wang Jian  Zhu Xiongzeng  Du Xiang  Sun Menghong  Zheng Aihua
Institution:Department of Pathology, Cancer Hospital, Fudan University, Shanghai 200032, China.
Abstract:OBJECTIVE: To study the morphologic and immunohistochemical features of gastrointestinal stromal tumors (GISTs) and to explore the reference parameters for malignancy. METHODS: Seventy six (76) cases of primary GISTs were distinguished from a group of gastrointestinal mesenchymal tumors by use of a panel of antibodies such as CD117, CD34 by immunohistochemical EnVision method, their biologic behaviors were analyzed by including their follow-up data. RESULTS: All patients were adults, age range 32 to 81 years (mean 54 year), male 39 cases and female 37 cases; the tumors were situated in stomach (36 cases), in small intestine (23 cases), colon (2 cases) and rectum (15 cases). The most common symptoms were abdomen mass, vague pain and GI bleeding. Forty eight (48) cases were mainly located within the muscularis propria, 25 cases outside the serosa, and 3 cases below the mucosa. Grossly, they were of soft consistency often with hemorrhage, cystification or necrosis. Microscopically, the tumors were composed of spindle cells (46 cases) or epithelioid cells (9 cases) and of both cells (21 cases), arranged in interlacing fasicles, diffusing sheets, pallisading, whirling, alveolar and giant pseudo-rosette shapes. Tumor cells often had abundant cytoplasm with light to moderate eosinophilic or slight basophilic in staining, the nuclei generally showed spindle, blunted ends, round or signet in shape with nucleoli. Immunohistochemically, CD117 and CD34 showed diffuse strong expression, the positive rates were 98.7% and 68.4% respectively, alpha-SMA, MSA, S-100, PGP9.5 showed focal expression, the positive rates were 25.0%, 19.7%, 23.7% and 17.1% respectively, vimentin were all positive and desmin, GFAP, NF were all negative. Nine cases were benign, 19 cases borderline and 48 cases malignant. Follow-up of 20 cases with benign and borderline tumors found patients alive without tumor. In the malignant group of 34 cases, 10 cases were alive without tumor, 10 cases developed recurrence or metastasis, and 14 cases died of tumor. Coagulative necrosis, mitotic activity over 10/50HPF, high cellularity and obvious pleomorphism were all in the malignant group. In this group, tumor necrosis, adhesion in operation, tumor, over 5 cm in diameter, mitotic activity over 5/50HPF had significant differences among three groups and the 3 years survival rate had a significant difference in tumors with or without coagulative necrosis and also in tumors with or without mitotic activity over 5/50HPF. CONCLUSIONS: GISTs predominantly occurred in middle aged or old patients, the tumors had varied cell types and different arrangements, the immunohistochemical characters were positive for CD117 and CD34, negative for desmin, occasional positive for alpha-SMA, MSA, S-100 and PGP9.5, which were helpful to differentiate GIST from leiomyomas and Schwannomas. Coagulative necrosis, mitotic activity over 10/50HPF, high cellularity with obvious pleomorphism were also helpful parameters for diagnosis of malignancy aside from metastasis and invasion. Adhesion, over 5 cm in diameter and mitotic activity over 5/50HPF but less than 10/50HPF might be the potential malignant parameters.
Keywords:Gastrointestinal neoplasms  Mesenchymoma  Pathology  surgical  Proto  oncogene proteins c  kit  Antigens  CD34
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