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目的探讨胃肠道外间质瘤(EGIST)临床病理、免疫组化特点及鉴别诊断。方法收集30例经组织学诊断为胃肠道外间质瘤患者的组织切片,对其临床资料进行回顾性分析,并行免疫组化染色,检测DOG1、CD117、CD34、S-100、vimentin、SMA、CD68、Ki-67和desmin等的表达。结果 EGIST最主要的临床表现为腹痛或发现腹部包块。肿瘤源于肠系膜15例,网膜6例,腹膜后5例,其他部位4例。肿瘤平均直径12 cm,其中梭形细胞为主型23例,上皮细胞为主型3例,混合型4例。免疫组化:DOG1、CD117、CD34、vimentin、Ki-67、SMA、CD68和S-100(+),desmin(-)。结论 EGIST的病理诊断必须依据大体形态、组织学、免疫组化以及基因检测结果综合判断。DOG1和CD117在EGIST的诊断中具有重要价值。EGIST需与相同部位的平滑肌肿瘤、恶性神经鞘膜瘤等鉴别。
Objective To investigate the clinicopathological features, immunohistochemical features and differential diagnosis of extraintestinal stromal tumors (EGIST). Methods Tissue sections of 30 patients with histologically diagnosed gastrointestinal stromal tumors were collected. The clinical data of the patients were retrospectively analyzed. The expressions of DOG1, CD117, CD34, S-100, vimentin and SMA were detected by immunohistochemistry. CD68, Ki-67 and desmin expression. Results The main clinical manifestation of EGIST was abdominal pain or abdominal mass. Tumors originated from mesentery in 15 cases, reticula in 6 cases, retroperitoneal in 5 cases and other parts in 4 cases. The average diameter of the tumor was 12 cm, of which 23 were spindle-shaped, 3 were predominant and 4 were mixed. Immunohistochemistry: DOG1, CD117, CD34, vimentin, Ki-67, SMA, CD68 and S-100 (+), desmin (-). Conclusion The pathological diagnosis of EGIST must be based on general morphology, histology, immunohistochemistry and genetic test results. DOG1 and CD117 are of great value in the diagnosis of EGIST. EGIST with the same parts of the smooth muscle tumor, malignant nerve sheath tumor identification.