食管间质瘤与平滑肌肿瘤对照性研究

A comparative study of esophageal stromal tumors and smooth muscle tumors

目的 探讨食管间质瘤与平滑肌肿瘤临床病理、免疫组织化学及分子生物学特点。方法 2 4例食管间叶源性肿瘤用CD117、CD34等一组抗体重新进行分类 ,部分病例同时测定c kit基因 11外显子序列。结果 此组肿瘤分别为间质瘤 3例 (交界性 1例、恶性 2例 ) ,年龄 71、5 6、6 0岁 ,均为男性 ,瘤体直径 4、8、14cm ,源于固有肌层。瘤细胞梭形 ,上皮样多角形及印戒样 ,呈交叉束状、栅栏状及弥漫片状排列 ,免疫表型为CD117、CD34弥漫强阳性。平滑肌瘤 2 0例 ,年龄 30～ 6 0岁 ,平均 41 6岁 ,男性 12例 ,女性 8例 ,15例源于固有肌层 ,直径 0 8～ 10 5cm(平均 4 5cm) ,5例源于黏膜肌层 ,直径为 0 2～ 1 0cm(平均 0 6cm)。平滑肌肉瘤 1例 ,男性 ,6 1岁 ,瘤体直径 5cm ,源于黏膜肌层。平滑肌 (肉 )瘤胞质丰富 ,嗜伊红 ,交叉束状排列 ,免疫表型为平滑肌肌动蛋白、肌特异性肌动蛋白、结蛋白弥漫强阳性。恶性间质瘤有c kit基因 11外显子的突变 ,平滑肌瘤无突变。结论 食管间叶源性肿瘤仍以平滑肌瘤多见 ,可发生与胃肠道间质瘤相同形态及免疫表型的间质瘤 ,典型平滑肌肉瘤极为罕见 ,食管间质瘤与平滑肌瘤具有不同的临床病理学及分子生物学特征。

Objective To explore the clinicopathological, immunohistochemical, and molecular biologic characteristics of esophageal stromal tumors and smooth muscle tumors. Methods Twenty four cases of esophageal mesenchymal tumors were reclassified by a panel of antibodies such as CD117, CD34 etc.. The sequence of 11 exon of c-kit gene were detected in some cases. Results There were 3 cases of esophageal stromal tumors, 20 leiomyomas, and 1 leiomyosarcoma. The 3 esophageal stromal tumors occurred in 3 men aged 71, 56 and 60 years respectively. The tumors originated from muscularis propria with the size of 4 cm,8 cm and 14 cm in diameter. Microscopically, the tumor cells were spindle and epithelioid shaped with slightly basophilic appearance, arranged in intersecting fascicles, diffusing and palisading patterns. Immunohistochemically, the tumors were positive for CD117 and CD34. The mutation of 11 exon of c-kit gene was detected in one case. In comparison, esophageal leiomyomas occurred in a younger population. The age ranged from 30 to 60 years ( mean age 41.6 years),12 male cases,8 female cases. 15 cases of esophageal leiomyomas were intramural tumors with a diameter of 0.8-10.5 cm(mean 4.5 cm )originating from muscularis propria and 5 cases which were intraluminal polyps with a diameter of 0.2-1.0 cm originating from muscularis mucosae. Leiomyomas were strongly eosinophilic in appearance, diffuse positivity for α-SMA, MSA, and desmin, and no c-kit gene mutation. One male case of leiomyosarcoma had a diameter of 5 cm and originated from muscularis mucosae and displayed a sausage-shaped polyp. Conclusions Leiomyoma is still the most common mesenchymal tumor of the esophagus, the stromal tumor can be similar to gastrointestinal stromal tumors. Typical esophageal leiomyosarcoma is very rare and has different clinicopathologic and molecular biologic features.