Abstract：
Children with trisomy 18 tend to develop hepatoblastoma. Since the introduction of appropriate management for organ malfunction, individuals with trisomy 18 have come to have a longer life expectancy. However, the predisposition to hepatoblastoma becomes a significant issue for the quality of a case. Here, we present a rare multifocal hepatoblastoma involving predominantly Couinaud segments 5 and 7 in a 10-month-old boy with trisomy 18. Though the first-line cisplatin monotherapy resulted in unsatisfactory tumor shrinkage, the second-line neoadjuvant chemotherapy administrating irinotecan and vincristine gave rise to significant tumor reduction in volume, leading to the completion of partial resection of the liver without the microscopic residual disease. The patient has been free from recurrence for 44 months. Because anatomical right hepatectomy can cause circulatory instability, including acute onset of pulmonary hypertension in trisomy 18 patients, physicians should balance treatment benefits and potential adverse effects. Our successful experience utilizing a combination of efficacious and less cardiotoxic neoadjuvant chemotherapy followed by the partial hepatectomy encourages physicians to treat a patient with trisomy 18 and tackle hepatoblastoma with a genetic background.
摘要：
18三体的儿童倾向于发展为肝母细胞瘤。自从对器官功能障碍实行适当的管理以来，患有18三体综合征的人已经有了更长的预期寿命。然而,肝母细胞瘤的易感性成为影响病例质量的重要问题。这里,我们介绍了一个10个月大的18三体男孩中罕见的多灶性肝母细胞瘤，主要涉及Couinaud5和7段。尽管一线顺铂单药治疗肿瘤缩小效果不理想,给予伊立替康和长春新碱的二线新辅助化疗导致肿瘤体积显著减小，导致肝脏部分切除完成，而没有显微残留疾病。患者已无复发44个月。因为解剖右肝切除术会导致循环不稳定，包括18三体病患者的肺动脉高压的急性发作，医师应平衡治疗益处和潜在的不良反应.我们利用有效且心脏毒性较低的新辅助化疗以及部分肝切除术的成功经验，鼓励医生治疗18三体综合征患者并解决具有遗传背景的肝母细胞瘤。
