CLINICOPATHOLOGIC STUDIES FOR HISTOLOGICAL CLASSIFICATION AND PROGNOSIS OF RHABDOMYOSARCOMA

1)我们回顾了6例横纹肌肉瘤作为一种罕见的双侧视网膜母细胞瘤儿童放射治疗后的第二原发性恶性肿瘤。我们的结论是，了解横纹肌肉瘤的发生和适当的免疫组织化学研究有助于避免误诊复发性视网膜母细胞瘤或尤文氏肉瘤，当遇到这些患者并发第二小圆细胞肿瘤时。2)我们研究了77例不同类型骨和软组织肉瘤上皮细胞主要钙粘蛋白E-cadherin的免疫反应性。我们发现E-cadherin在某些类型的软组织肉瘤中表达，特别是那些具有上皮样特征的软组织肉瘤，这表明E-cadherin在其结构构成中起作用。3)我们对24例应与梭形细胞横纹肌肉瘤鉴别的胸外孤立性纤维性肿瘤进行临床病理研究，发现其组织学变异性和潜在的侵袭性行为。4)我们研究了95例成人软组织肉瘤（包括3例四肢、躯干、头部和颈部的横纹肌肉瘤）增生活性评估的组织学分级系统与预后的相关性。基于ki-67 （MIB-1）的客观组织学分级系统在转移获取能力方面更为准确，转移获取能力与总生存期密切相关。

1) We reviewed 6 cases of rhabdomyosarcoma as a rare second primary malignancy in children with bilateral retinoblastoma after irradiation treatment. We have concluded that knowledge of the occurrence of rhabdomyosarcoma and appropriate immunohistochemical study are helpful for avoiding a misdiagnosis of recurrent retinoblastoma or Ewing's sarcoma when encountering these patients who developed second small round cell neoplasms.2) We studied immunoreactivity for E-cadherin, the major cadherin of epithelial cells, in 77 cases of a variety of bone and soft tissue sarcomas. We found that E-cadherin was expressed in certain kinds of soft tissue sarcomas, especially those with epithelioid features, suggesting that E-cadherin plays a role in the constitution of their architecture.3) We conducted clinicopathologic study for 24 cases of extrathoracic solitary fibrous tumor that should be differentiated from spindle cell rhabdomyosarcoma, and found their histological variability and potentially aggressive behavior.4) We investigated the prognostic relevance of a histological grading system based on the assessment of proliferative activity in 95 cases of adult soft-tissue sarcomas including 3 rhabdomyosarcomas of the extremities, trunk, head, and neck. An objective histological grading system using ki-67 (MIB-1) was concluded to be more accurate in accessing ability to metastasize, which was strongly correlate with overall survival.