小儿肾上腺皮质肿瘤10例报告

Adrenocortical tumors in children: Report of 10 cases

目的 探讨小儿肾上腺皮质肿瘤的诊断、治疗及影响预后的因素。方法 回顾性分析1982～2002年间我院收治的10例病例,男4例,女6例,年龄6个月～12岁,平均(4.0±3.5)岁,皮质腺瘤7例,皮质癌3例,均有内分泌功能紊乱,评价治疗结果。结果 肾上腺皮质腺瘤7例,5例手术完整切除肿瘤,4例随访6个月～17年,均健在。肾上腺皮质癌3例,2例手术加化疗,分别存活6个月及1年;1例仅作活检,存活3个月。结论 肾上腺皮质腺瘤通过完整切除肿瘤,围手术期及术后激素监控替代治疗,可获治愈。肾上腺皮质癌需手术加化疗综合治疗,预后仍不佳。由于术前尚无可靠指标可区别肿瘤的良恶性,早期诊断和完整切除肿瘤至关重要。

Objective To evaluate the diagnosis and treatment of pediatric adrenocortical tumours (ACTs). Methods Records of 10 children (4 boys, 6 girls) aging 10 months-12years (mean 4. 0±3. 5) with ACTs between 1982 and 2002 were reviewed retrospectively. There were left side in 8 cases and right in 2. Deta recorded of each patient included historical features, clinical characteristics, diagnostic methods, treatment, pathologic findings and outcome. Results Among the 5 out of 7 cases with adrenocortical ade- noma who underwent complete surgical excision, 4 patients were doing well with a followed-up from 6 months to 17 years. Two out of 3 patients with adrenocortical cancinoma received a complete surgical exci- sion with chemotherapy and died 6 months and one year later respectively, the other one was only taken biopsy and died three months after. Conclusions Adrenocortical adenoma in children can be treated effec- tively by total excision. Early diagnosis and total excision might be useful for children with adrenocortical cancinoma.