Sharma Divya, Subbarao Girish, Saxena Romil
Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, United States.
Department of Pediatric Gastroenterology, Hepatology and Nutrition, Indiana University School of Medicine, United States.
Semin Diagn Pathol. 2017 Mar;34(2):192-200. doi: 10.1053/j.semdp.2016.12.015. Epub 2016 Dec 23.
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.
肝母细胞瘤是婴幼儿期最常见的原发性肝脏恶性肿瘤，主要发生在出生后的头两年。在过去30年中，肝母细胞瘤的治疗方法发生了显著变化；新辅助化疗现已成为标准治疗方法，尤其是对于无法切除的肿瘤，可使肿瘤在术前显著缩小，有时甚至接近完全消融。一名20个月大的婴儿在因左输尿管狭窄进行常规筛查超声检查时，偶然发现右侧腹膜后有一个7.6厘米的肿瘤。血清甲胎蛋白升高。活检显示为肝母细胞瘤，混合型上皮和胚胎型，无间质成分。他接受了新辅助化疗。尽管肿瘤大小已显著减小，但由于其紧邻主要血管结构，无法进行安全切除。于是进行了肝移植；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，他正在继续进行第六周期化疗。
