Yan Zheng, Bai Wei, Li Li, Li Shuo, Hua Ying, Zhang Xiao-Xiao, Hou Xin-Lin
Department of Pediatrics, Peking University First Hospital, Beijing, China.
Department of Obstetrics and Gynecology, Peking University First Hospital, Beijing, China.
Front Pediatr. 2022 Jul 1;10:905089. doi: 10.3389/fped.2022.905089. eCollection 2022.
This study reports a case of hepatoblastoma with onset at 30-weeks' gestation and rapid growth rate. The postnatal enhanced CT confirmed an intrahepatic mass with a size of 8.5 cm × 6.6 cm and a clear boundary accompanied by uneven enhancement, displacement, and narrow lumen of the hepatic vein due to compression. The alpha-fetoprotein (AFP) at birth was 1,002,632 ng/ml (normal level 48,406 [±34,718] ng/ml). A diagnosis of congenital hepatoblastoma was established based on the imaging and laboratory outcomes. The infant received chemotherapy of Cisplatin-5 fluorouracil-Vincristine (C5V) on the fourth day after birth. After four courses of C5V, a complete tumor resection was performed, and the postoperative pathology was consistent with mixed epithelial and mesenchymal hepatoblastoma. Four more courses of C5V and one course of C5VD (C5V plus doxorubicin) followed the surgery. Infectious diarrhea and acute kidney injury (stage I) occurred during chemotherapy, which recovered after anti-infection and symptomatic treatment. The patient is currently 2 years old and still in complete remission. In this case, the onset of hepatoblastoma was early, and the tumor grew rapidly, resulting in an obvious compression effect. Chemotherapy was started early after birth, and the curative effect was satisfactory, suggesting that the hepatoblastoma based on clinical diagnosis with rapid tumor progression and severe dysfunction of surrounding organs caused by compression should undergo chemotherapy as soon as possible if a pathological diagnosis cannot be obtained temporarily, which also plays an important role in improving the complete resection rate of intraoperative tumor and reducing the recurrence rate of postoperative tumor.
本研究报告了1例妊娠30周发病且生长速度较快的肝母细胞瘤病例。出生后增强CT证实肝内有一肿块,大小为8.5 cm×6.6 cm,边界清晰,伴有不均匀强化,肝静脉因受压而移位且管腔变窄。出生时甲胎蛋白(AFP)为1,002,632 ng/ml(正常水平48,406[±34,718] ng/ml)。根据影像学和实验室检查结果确诊为先天性肝母细胞瘤。患儿出生后第4天接受了顺铂-5氟尿嘧啶-长春新碱(C5V)化疗。经过4个疗程的C5V化疗后,进行了肿瘤完整切除,术后病理与混合上皮和间叶性肝母细胞瘤相符。术后又进行了4个疗程的C5V化疗和1个疗程的C5VD(C5V加阿霉素)化疗。化疗期间发生了感染性腹泻和急性肾损伤(I期),经抗感染及对症治疗后恢复。患儿目前2岁,仍处于完全缓解状态。该病例肝母细胞瘤发病早,肿瘤生长迅速,产生明显压迫效应。出生后早期开始化疗,疗效满意,提示对于临床诊断明确、肿瘤进展迅速且因压迫导致周围器官严重功能障碍而暂时无法获得病理诊断的肝母细胞瘤,应尽早进行化疗,这对提高术中肿瘤完整切除率及降低术后肿瘤复发率也具有重要作用。