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目的探讨腹腔软组织平滑肌肿瘤临床病理、免疫组化、超微结构特点及鉴别诊断。方法用desmin、α-SMA、MSA以及CD117、CD34为主的一组抗体对腹腔软组织原诊断为平滑肌瘤、平滑肌母细胞瘤及平滑肌肉瘤等病例进行研究,获得34例腹腔、系膜及腹膜后平滑肌肿瘤,其中2例行超微结构观察。结果男性8例,女性26例,年龄26~77岁,平均47·6岁,中位年龄48岁,其中腹膜后28例,肠系膜4例,腹腔2例,肿瘤直径5~18cm,平均9·86cm。梭形细胞型32例,其中1例伴有反应性破骨样巨细胞,1例有高度恶性软组织肉瘤区域(即去分化平滑肌肉瘤),上皮型和混合型各1例。此组抗体表达分别为desmin(21/22)、α-SMA(21/21)、MSA(21/21)、CD117(1/21)、CD34(1/21)、S-100蛋白(3/21)、PGP9·5(2/21)。超微结构可见基板、质膜下连续的微饮泡、胞质内丰富的密体和密斑,而细胞器较少。其中4例检测c-kit基因11号外显子序列,均为野生型。平滑肌瘤4例,其中2例分别健在5年和10年;交界性1例;平滑肌肉瘤29例,获访13例,11例(85·0%)在3年内复发、转移或死亡,1例5年后死亡,仅1例无瘤生存10年。结论腹腔软组织平滑肌肿瘤主要发生于腹膜后,女性多见,以恶性居多,肿瘤性坏死、核分裂象≥4/50HPF及细胞明显异型性对判断恶性有重要参考价值。需与相同部位的胃肠道外间质瘤、恶性神经鞘膜瘤等鉴别。
Objective To investigate the clinicopathological, immunohistochemical, ultrastructural characteristics and differential diagnosis of abdominal soft tissue smooth muscle tumors. Methods A group of antibodies including desmin, α-SMA, MSA, and CD117 and CD34 were used to study peritoneal soft tissue original cases diagnosed as leiomyoma, smooth muscle blastoma and leiomyosarcoma. 34 cases of abdominal cavity, mesentery and peritoneum were obtained. After smooth muscle tumors, 2 of them underwent ultrastructural observation. Results There were 8 males and 26 females, aged 26 to 77 years, mean 47. 6 years old, median age 48 years, including 28 retroperitoneal, 4 mesenteric, 2 abdominal, tumor diameter 5~18cm, average 9· 86cm. Among the 32 cases of spindle cell type, 1 case had reactive osteoclast-like giant cells, 1 case had high malignant soft tissue sarcoma area (ie, dedifferentiated leiomyosarcoma), and 1 case had epithelial and mixed type. This group of antibodies were expressed desmin (21/22), α-SMA (21/21), MSA (21/21), CD117 (1/21), CD34 (1/21), S-100 protein (3/ 21), PGP 9·5 (2/21). The ultrastructure shows that the substrate, continuous microbubbles under the plasma membrane, abundant dense and dense patches in the cytoplasm, and fewer organelles. Among them, 4 cases of c-kit gene exon 11 sequences were detected and all were wild-type. There were 4 cases of leiomyomas, of which 2 cases were alive for 5 years and 10 years respectively; 1 case was borderline; 29 cases were leiomyosarcoma, 13 cases were visited, 11 cases (85.0%) relapsed, metastasized or died within 3 years, 1 The case died after 5 years, and only 1 case survived for 10 years. Conclusion Abdominal soft tissue smooth muscle tumors mainly occur in the retroperitoneum and are more common in women. Most of them are malignant. Neoplasms necrosis, mitotic figures ≥4/5O HPF and obvious cell atypicality have important reference value in judging malignancy. Need to identify with the same site of extra-intestinal stromal tumors, malignant nerve sheath tumors.