Tucci Silvio, Martins Antonio C P, Suaid Haylton J, Cologna Adauto J, Reis Rodolfo B
Division of Urology, Department of Surgery, Medical School of Ribeirão Preto, University of São Paulo, Av. Bandeirantes #3900, Ribeirão Preto, São Paulo, Brazil 14048-900.
J Urol. 2005 Dec;174(6):2338-42, discussion 2342. doi: 10.1097/01.ju.0000180645.89054.fd.
We evaluated treatment outcomes in children with adrenocortical carcinoma.
We studied 34 children with a median age of 3 years. In 27 of 28 patients without intracaval extension complete surgical excision was accomplished, while tumor resection combined with thrombectomy was carried out in 5 of 6 children with vascular invasion. In 2 children with cavoatrial thrombus the thrombectomy required cardiopulmonary bypass with deep hypothermia and circulatory arrest. Children with incomplete excision of the tumor and/or stage IV disease received adjuvant chemotherapy.
Ultrasonography, computerized tomography and magnetic resonance imaging exhibited specificity of 100% in the diagnosis of vascular invasion, and sensitivity of 50%, 66% and 100%, respectively. Patient age, tumor stage or size and vascular invasion were associated with survival in univariate analysis. Tumor stage was the only independent factor associated with survival in multivariate analysis. The overall 5-year survival rates according to tumor stage were 100% in stage I, 85% in stage II, 40% in stage III and 0% in stage IV. Of 11 children with local recurrence only 2 were alive without disease at 96 and 204 months after reoperation with complete tumor excision. Only 2 of 6 patients with vascular invasion were disease-free at 17 and 50 months. A total of 10 children with stage IV disease treated with chemotherapy died within a median of 6 months.
Tumor stage was the most relevant prognostic factor for children with adrenocortical carcinoma. Reoperation for local tumor recurrence and thrombectomy for inferior vena caval tumor invasion should be attempted whenever possible.
我们评估了肾上腺皮质癌患儿的治疗效果。
我们研究了34名中位年龄为3岁的儿童。28例无腔静脉侵犯的患者中有27例完成了完整的手术切除，6例有血管侵犯的儿童中有5例进行了肿瘤切除联合血栓切除术。2例有腔房血栓的儿童进行血栓切除术时需要在深低温和循环停止下进行体外循环。肿瘤切除不完全和/或处于IV期疾病的儿童接受辅助化疗。
超声、计算机断层扫描和磁共振成像在诊断血管侵犯方面的特异性均为100%，敏感性分别为50%、66%和100%。单因素分析显示，患者年龄、肿瘤分期或大小以及血管侵犯与生存率相关。多因素分析显示，肿瘤分期是与生存率相关的唯一独立因素。根据肿瘤分期，I期的5年总生存率为100%，II期为85%，III期为40%，IV期为0%。11例局部复发的儿童中，只有2例在再次手术完整切除肿瘤后分别于96个月和204个月时无病存活。6例有血管侵犯的患者中只有2例在17个月和50个月时无病。10例接受化疗的IV期疾病儿童中位在6个月内死亡。
肿瘤分期是肾上腺皮质癌患儿最相关的预后因素。应尽可能尝试对局部肿瘤复发进行再次手术，对下腔静脉肿瘤侵犯进行血栓切除术。
