上海交通大学学报（医学版）

上海交通大学学报（医学版）

• 论著（临床研究） • 上一篇    下一篇

儿童横纹肌肉瘤的病理学研究

管雯斌，许恪淳，许艳春，王立峰，姚晓虹，祝明洁   

  1. 上海交通大学 医学院附属新华医院病理科， 上海 200092
  • 出版日期:2014-01-28 发布日期:2014-01-29
  • 通讯作者: 祝明洁, 电子信箱: m-jzhu@hotmail.com。
  • 作者简介:管雯斌（1983—）， 男， 主治医师， 硕士; 电子信箱: celiceli02@126.com。

Pathological study of childhood rhabdomyosarcoma

GUAN Wen-bin, XU Ke-chun, XU Yan-chun, WANG Li-feng, YAO Xiao-hong, ZHU Ming-jie   

  1. Department of Pathology, Xinhua Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200092, China
  • Online:2014-01-28 Published:2014-01-29

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摘要：

目的 探讨儿童横纹肌肉瘤的病理形态学特点。方法 复习和分析30例儿童横纹肌肉瘤的形态学和免疫组织化学染色结果。结果 30例横纹肌肉瘤患儿中，男性22例，女性8例。组织学类型包括胚胎性（20/30，66.67%）、腺泡状（8/30，26.67%）和梭形/硬化性（2/30，6.67%）横纹肌肉瘤3种。发生部位依次为躯干（15/30，50.00%）、头颈（7/30，23.33%）、四肢（6/30，20.00%）和泌尿生殖系统（2/30，6.67%）。免疫组织化学染色示肌特异性肌动蛋白（MSA）、平滑肌肌动蛋白（SMA）、结蛋白（DES）、肌细胞生成素（MyoG）和肌调节蛋白（MyoD1）均有不同程度的阳性表达。结论 胚胎性、腺泡状和梭形/硬化性横纹肌肉瘤是儿童横纹肌肉瘤的主要类型，组织学上主要表现为原始的小圆细胞，部分病例可见横纹肌母细胞。肿瘤细胞不同程度表达MSA、SMA、DES、MyoG和MyoD1等肌源性标志物。鉴别诊断主要包括神经母细胞瘤、外周原始神经外胚层肿瘤等。

关键词: 软组织肿瘤, 横纹肌肉瘤, 病理学, 免疫组织化学

Abstract:

Objective To study the pathologic characteristics of childhood rhabdomyosarcoma. Methods Thirty cases of pediatric rhabdomyosarcoma were reviewed by histological and immunohistochemical observation. Results There were 22 boys and 8 girls. Histologically, all the cases were divided into embryonal, alveolar, and spindle/sclerosing rhabdomyosarcoma types, in which, embryonal type accounted for 66.67% (20/30); alveolar type, 26.67% (8/30); and spindle/sclerosing type, 6.67% (2/30). The location of the tumors included the trunk （50%, 15/30), head and neck (23.33%, 7/30), extremities (20%, 6/30), and urinary system (6.67%, 2/30). Immunohistochemical staining findings indicated that muscle-specific actin (MSA), smooth muscle actin (SMA), Desmin, myogenin (MyoG), and MyoD-1 had different degrees of positive expression. Conclusion Embryonal, alveolar, and spindle cell/sclerosing rhabdomyosarcoma are main histological types of rhabdomyosarcoma of children. They mainly show primitive small round cells with rhabdomyoblast cells in some cases. Tumor cells express different levels of muscle origin marks such as MSA, SMA, Desmin, MyoG, and MyoD-1. Differential diagnosis should be made with neuroblastoma and peripheral primitive neural ectoderm tumor.

Key words: soft tissue tumors, rhabodmyosarcoma, pathology, immunohistochemistry