腹腔软组织平滑肌肿瘤临床病理分析  

Smooth muscle tumors in abdominal soft tissue:a clinicopathological study

作　　者：侯英勇[1] 谭云山[1] 许建芳[1] 王岫南[1] 宿杰.阿克苏 周燕南[1] 曾海英[1] 高峰[1] 郑爱华[2] 朱雄增[2] 

机构地区：[1]复旦大学附属中山医院病理科,上海200032 [2]复旦大学附属肿瘤医院病理科,上海200032

出　　处：《临床与实验病理学杂志》2006年第4期397-401,共5页Chinese Journal of Clinical and Experimental Pathology

基　　金：国家自然科学基金青年基金(30300152)

摘　　要：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≥4／50 HPF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。Purpose To explore the clinicopathological, immunohistochemical and uhrastructural features of smooth muscle tumors in abdominal soft tissue and their differential diagnosis. Methods Thirty-four cases of smooth muscle tumors from the abdominal cavity or retroperitoneum were studied. Results The tumors occurred in 8 men and 26 women, with age ranged from 26 to 77 years （ mean 47. 6 years） , in which twenty-eight cases arose from the retroperitoneum, 4 from mesentery and 2 from abdominal cavity. The size of tumors ranged from 5 to 18 cm （mean 9. 86 cm） in diameter and the tumor cell components varied, mainly spindle cells （32 cases） , in which one case was accompanied with reactive giant cells and one case displayed area of high grade pleomorphic sarcoma （ dedifferentiated leiomyosarcoma area） , epithelioid cells （ one cases ） , and mixed cells （ one case）. Tumors expressed desmin （ 21/22 ） , α-SMA（21/21 ）, MSA（21/21 ）, CD117（ 1/21 ）, CD34（ 1/21 ）, S-100（3/21 ）, and PGP9. 5（2/21 ）. Electron microscopically, continuous basal lamina, regular caveolae, and dense plaque were observed. Four cases were leiomyomas, one case was borderline and 29 cases were leiomyosarcoma. Follow-up of 2 cases of leiomyomas showed long-term survival of 5 years and 10 years, In the leiomyosarcomas group, follow-up information was available in 13 cases, in which 11 cases developed recurrence, metastasis or death within 3 years, one cases died of disease in 5 years, and only one cases survived for 10 years without tumor. Conclusions The smooth muscle tumor of abdominal soft tissue mainly occurs in retroperitoneum. Most cases are female and malignant. The features of tumor coagulative necrosis, mitoses≥4 per 50 high-power fields and obvious nuclear atypia indicate malignancy. Differential diagnosis includes gastrointestinal stromal tumors, malignant nerve sheath tumors and others.

关 键 词：平滑肌肉瘤 腹膜后肿瘤 胃肠道外间质瘤 DESMIN CD117 诊断 鉴别 

分 类 号：R738.6[医药卫生—肿瘤] R735.2[医药卫生—临床医学]