Rana Asim Noor, Qidwai Asim, Pritchard Jon, Ashraf M Shamvil
Department of Paediatric and Adolescent Oncology, Middlesex Hospital, London, UK.
Pediatr Hematol Oncol. 2006 Mar;23(2):153-8. doi: 10.1080/08880010500457566.
We report the case of a girl with multi-focal hepatoblastoma in whom chemotherapy alone has resulted in long term event-free survival and possibly cure, without any surgical procedure apart from biopsy for initial diagnosis. At presentation she had a large tumour arising from the left lobe of liver and two other separate masses were noted in the right lobe, but the lungs were free of metastases. Histology showed a foetal type of hepatoblastoma. The serum alpha-feto protein (AFP) level was 44,000 iu/litre. Chemotherapy was started using the triple drug regime recommended for "high risk" (of relapse) patients in the SIOPEL 2 hepatoblastoma protocol of the International Society of Paedaitric Oncology (SIOP). Within a few weeks her abdominal girth decreased, the child became much more comfortable. Drug-induced cardiotoxicity, ototoxicity and nephrotoxicity were not observed. After a total of 4 courses of chemotherapy (completed at the end of August 1998) a CT scan showed that all 3 tumours were smaller but that there were residual multifocal defects in the liver neither hepatic resection nor liver transplantation were considered safe or appropriate. 6.5 years after completion of chemotherapy and now aged 8.5 years the child is in normal health and at school with normal liver size, serum AFP levels and chest imaging.
我们报告了一名患有多灶性肝母细胞瘤的女孩的病例，在该病例中，仅化疗就实现了长期无事件生存且可能治愈，除了用于初始诊断的活检外未进行任何手术。初诊时，她的肝脏左叶有一个大肿瘤，右叶还发现另外两个独立的肿块，但肺部无转移。组织学检查显示为胎儿型肝母细胞瘤。血清甲胎蛋白（AFP）水平为44,000国际单位/升。按照国际儿科肿瘤学会（SIOP）的SIOPEL 2肝母细胞瘤方案中针对（复发）“高危”患者推荐的三联药物方案开始化疗。几周内，她的腹围减小，孩子感觉舒服多了。未观察到药物引起的心脏毒性、耳毒性和肾毒性。总共进行4个疗程的化疗后（于1998年8月底完成），CT扫描显示所有3个肿瘤都变小了，但肝脏仍有残留的多灶性缺损，肝切除和肝移植均被认为不安全或不合适。化疗完成6.5年后，现在孩子8.5岁，身体健康，在学校上学，肝脏大小、血清AFP水平和胸部影像学检查均正常。
