A 32-year-old primiparous pregnant woman's routine prenatal ultrasound at 30-weeks' gestation revealed a 1.8 cm x 1.8 cm x 1.5 cm medium-to-high echoic mass with a clear boundary surrounded by a low echoic dark halo in the fetus' parenchyma of the right lobe of the liver. Inside the mass, a few strips of blood flow signals could be detected (Figure 1A). A fetal MRI at 31-weeks' gestation showed a 1.8 cm x 1.5 cm x 1.2 cm nodule with a clear boundary and slightly high signal intensity on T2WI, slightly low signal intensity on TIWI, and slightly high signal intensity on DWI, suggesting a mass located in the right lobe of the liver. At 38-weeks' gestation, the mass had increased rapidly to a size of 7.1 cm x 7.9 cm x 5.6 cm (Figure 1B).
A female infant was delivered by cesarean section at 39 + 3-weeks' gestation with a birth weight of 3,040 g and Apgar scores of 10 for the 1st and 5th min. The infant suffered from poor appetite, frequent vomiting, yellow skin, and abdominal distension. On physical examination, the liver could be palpated 10 cm below the ribs; firm consistency, blunt edges, and a smooth surface were observed. Laboratory tests showed alanine aminotransferase (ALT) 380 IU/L, aspartate aminotransferase (AST) 315I U/L, total bilirubin (TBIL) 251 umol/L, direct bilirubin (DBIL) 165 umol/L, and fibrinogen (FIB) 1.2g/L; alpha-fetoprotein (AFP) was significantly increased to 1,002,632 ng/ml (normal 48,406 [+- 34,718] ng/ml). An abdominal ultrasound on day 1 showed a mass of about 8.1 cm x 7.2 cm with mixed echoes, a clear margin, and detectable calcification inside. It was mainly located in the right anterior lobe. An abdominal enhanced CT and a three-dimensional model on day 2 showed a large round mass (8.5 cm x 6.6 cm), a clear boundary, and uneven enhancement (Figures 2A,B). The hepatic vein was displaced, and the lumen had become thinner because of compression from this mass. A bone puncture (marrow and biopsy) on day 3 after birth was normal, and no evidence of distant metastasis was found. Finally, a clinical diagnosis of congenital hepatoblastoma was reached.
Due to the large size and rapid growth of the tumor, the compression on the surrounding organs led to liver and coagulation dysfunction and feeding difficulties. Additionally, the tumor surface tension was so high that the risk of tumor rupture during biopsy was increased significantly. After full communication with the surgeon, it was clear that there were too many temporary contraindications for puncture and operation. After the parents understood the condition and situation of the infant, they all hoped to start chemotherapy as soon as possible to reduce the load of the tumor. Preoperative chemotherapy with Cisplatin-5fluorouracil-Vincristine (C5V) was initiated on day four. After four courses of chemotherapy, the tumor was reduced to 5.8 cm x 5.3 cm, and the AFP had declined to 2,516 ng/ml (normal 323 [+- 278] ng/ml). During chemotherapy, infectious diarrhea and transient acute kidney injury (type I) occurred on the third day of the first chemotherapy remission. The highest serum creatinine was 1.55 mg/dl, urinary microalbumin 193 mg/L (normal 0-19 mg/L), urinary transferrin 9.48 mg/L (normal 0-2 mg/L), and urine alpha1 microglobulin 94.7 mg/L (normal 0-12 mg/L), indicating early kidney injury. These readings returned to normal after anti-infection and symptomatic treatment. Complete resection of the tumor in the right lobe and part of the left lobe of the liver was performed 3 months after birth. The tumor size was about 5.5 cm x 5 cm x 4 cm, and no intravascular tumor thrombus was found.