Parham D M, Webber B L, Jenkins J J, Cantor A B, Maurer H M
Department of Pathology and Laboratory Medicine, St. Jude Children's Research Hospital, Memphis, Tennessee, USA.
Mod Pathol. 1995 Sep;8(7):705-10.
Because of the rarity of pediatric nonrhabdomyosarcomatous soft tissue sarcomas, it is difficult to test standardized treatment protocols for individual tumor entities. Grading has been used successfully to predict outcome of adult sarcomas, but pediatric soft tissue tumors display a notable difference in clinical behavior when compared to older patients. To test systematically a standardized treatment strategy for pediatric nonrhabdomyosarcomastous soft tissue sarcomas devised by the Pediatric Oncology Group, the authors devised a grading schema using concepts of adult grading systems and integrating the unique clinical and morphologic features of pediatric sarcomas. Three grading tiers were devised: Grade I, which includes certain pediatric tumors with little propensity for malignancy; Grade II, which is composed of tumors excluded from Grades I or III by virtue of histologic diagnosis and having <5 mitoses/10 high-power fields or <15% geographic necrosis; and Grade III, which comprises certain tumors known to be clinically aggressive by virtue of histologic diagnosis and non-Grade I tumors with >4 mitoses/10 high-power fields or > 15% necrosis. An initial retrospective analysis on a series of lesions treated at a single institution indicated a strong predictive value for grading. Subsequent prospective studies by the Pediatric Oncology Group continue to verify the prognostic value of pediatric nonrhabdomyosarcomatous soft tissue sarcoma grading, and studies on individual pediatric nonrhabdomyosarcomatous soft tissue sarcomas, such as synovial sarcoma, also indicate the relatively poor outcome of higher grade lesions. Grade can be used to predict outcome of pediatric nonrhabdomyosarcomatous soft tissue sarcomas and to devise treatment strategies for experimental protocols.
由于小儿非横纹肌肉瘤性软组织肉瘤罕见，因此难以针对单个肿瘤实体测试标准化治疗方案。分级已成功用于预测成人肉瘤的预后，但与年长患者相比，小儿软组织肿瘤在临床行为上存在显著差异。为了系统测试由小儿肿瘤学组设计的小儿非横纹肌肉瘤性软组织肉瘤的标准化治疗策略，作者设计了一种分级方案，该方案采用成人分级系统的概念，并整合了小儿肉瘤独特的临床和形态学特征。设计了三个分级层次：I级，包括某些恶性倾向较小的小儿肿瘤；II级，由根据组织学诊断被排除在I级或III级之外且有<5个有丝分裂/10个高倍视野或<15%的地图状坏死的肿瘤组成；III级，包括根据组织学诊断已知具有临床侵袭性的某些肿瘤以及有>4个有丝分裂/10个高倍视野或>15%坏死的非I级肿瘤。对在单一机构治疗的一系列病变进行的初步回顾性分析表明分级具有很强的预测价值。小儿肿瘤学组随后的前瞻性研究继续验证小儿非横纹肌肉瘤性软组织肉瘤分级的预后价值，对个别小儿非横纹肌肉瘤性软组织肉瘤（如滑膜肉瘤）的研究也表明高级别病变的预后相对较差。分级可用于预测小儿非横纹肌肉瘤性软组织肉瘤的预后并为实验方案制定治疗策略。
