Rech Angela, Castro Cláudio G, Mattei Jane, Gregianin Lauro, Di Leone Luciane, David Alexandre, Rivero Luis F, Tarrago Ricardo, Abreu Armando, Brunetto Algemir L
Hospital de Clínicas de Porto Alegre (HCPA), Porto Alegre, RS, Brazil.
J Pediatr (Rio J). 2004 Jan-Feb;80(1):65-70.
To identify the clinical features in osteosarcoma and to investigate their influence on the prognosis of children and adolescents presenting this disease.
The records of children and adolescents with osteosarcoma treated by the Bone Tumors Group of the state of Rio Grande do Sul, Brazil, between January 1992 and December 2001 were reviewed.
Fifty consecutive patients were included in this study. Mean age at diagnosis was 13 years (3-22); 68% of the patients were males. The primary site of disease was the femur in 50% of the patients, tibia in 30%, pelvis in 4%, humerus in 10%, fibula in 2% and other sites in 4%. Nineteen patients presented metastases at diagnosis (38%). All patients received chemotherapy and were treated with three different schemes. As for surgical treatment, 26 patients (52%) had an amputation and 17 (34%) received conservative surgery. Serum lactic dehydrogenase > 1,000 UI/ml (p = 0.0159, log rank), tumor necrosis < 90% and presence of metastases had a negative influence on prognosis. The overall 5-year survival was of 33.2+/-7.2% with mean follow-up of 36 months (6-126). Event-free survival was 29.7+/-7%. The 5-year event-free survival in non-metastatic patients was 45+/-10.7%, and zero in metastatic patients (follow-up of 78.4 and 18.7 months, respectively). Only two out of 19 metastatic patients are alive and free of disease at 18 and 30 months respectively.
Metastatic disease at diagnosis, serum levels of serum lactic dehydrogenase > 1,000 UI/ml and tumor necrosis < 90% are predictors of unfavorable prognosis. The excessively high incidence of metastatic patients may suggest the presence of an aggressive pattern of disease in our population, or may indicate late diagnosis.
确定骨肉瘤的临床特征，并研究其对患有该疾病的儿童和青少年预后的影响。
回顾了1992年1月至2001年12月期间巴西南里奥格兰德州骨肿瘤组治疗的骨肉瘤儿童和青少年的记录。
本研究纳入了50例连续患者。诊断时的平均年龄为13岁（3 - 22岁）；68%的患者为男性。疾病的原发部位在50%的患者中是股骨，30%是胫骨，4%是骨盆，10%是肱骨，2%是腓骨，4%是其他部位。19例患者在诊断时出现转移（38%）。所有患者均接受化疗，并采用三种不同方案进行治疗。至于手术治疗，26例患者（52%）进行了截肢，17例（34%）接受了保肢手术。血清乳酸脱氢酶>1000 UI/ml（p = 0.0159，对数秩检验）、肿瘤坏死<90%和转移的存在对预后有负面影响。总体5年生存率为33.2±7.2%，平均随访36个月（6 - 126个月）。无事件生存率为29.7±7%。非转移性患者的5年无事件生存率为45±10.7%，转移性患者为零（分别随访78.4和18.7个月）。19例转移性患者中只有2例分别在18个月和30个月时存活且无疾病。
诊断时的转移性疾病、血清乳酸脱氢酶水平>1000 UI/ml和肿瘤坏死<90%是预后不良的预测因素。转移性患者的过高发病率可能表明我们人群中存在侵袭性疾病模式，或者可能表明诊断延迟。
