肝母细胞瘤

摘要(中文译文)

肝母细胞瘤是婴幼儿期最常见的原发性肝脏恶性肿瘤，主要发生于出生后前两年内。过去30年间，肝母细胞瘤的治疗方式发生了显著变化；新辅助化疗现已成为标准治疗，尤其对于无法切除的肿瘤，可导致术前肿瘤明显缩小，有时甚至几乎完全消融。一例20月龄婴儿在因左侧输尿管狭窄行常规筛查超声时偶然发现右侧腹膜后7.6cm肿瘤。血清甲胎蛋白升高。活检显示为肝母细胞瘤，混合上皮型和胚胎型，无间叶成分。患儿接受了新辅助化疗。尽管肿瘤体积显著缩小，但因紧邻主要血管结构而无法安全切除。遂行肝移植术；切除的肝脏显示肿瘤完全坏死，无残留恶性肿瘤。术后过程顺利，患儿正在继续第六个周期的化疗。

Abstract

Hepatoblastoma is the most common primary malignant hepatic tumor of infancy and childhood, occurring predominantly in the first two years of life. The management of hepatoblastoma has changed markedly over the last 3 decades; neoadjuvant chemotherapy is now standard, particularly in unresectable tumors resulting in considerable preoperative tumor shrinkage and sometimes near total ablation of the tumor. A 20 month old infant was incidentally found to have a 7.6cm right sided retroperitoneal tumor on routine screening ultrasonography for left ureteral stenosis. Serum alpha fetoprotein was elevated. Biopsy revealed hepatoblastoma, mixed epithelial and embryonal type without mesenchymal elements. He underwent neoadjuvant chemotherapy. Although the tumor had decreased considerably in size, close proximity to major vascular structures precluded safe resection. Liver transplantation was performed; the explanted liver showed complete tumor necrosis with no residual malignancy. The postoperative course was uncomplicated and he is continuing on sixth cycle of chemotherapy.

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