EP07.17: Congenital hepatoblastoma: prenatal diagnosis with postnatal outcome

Hepatoblastoma is the most common hepatic malignancy in newborns and diagnosis is confirmed by histopathology examination (HPE). As 20% of infants have distant metastasis at diagnosis, prenatal diagnosis helps parents to plan postnatal management. In this case report, we present the ultrasound features, intrapartum/postpartum course and postnatal management of a fetus diagnosed with hepatoblastoma at 34 weeks gestation (GA). 36-year-old G2 was referred to us at 34 weeks GA for second opinion of fetal intrabdominal mass lesion. Scan showed a homogenously hyperechoic lesion measuring 6.5 x 4.5 x 4.8cm in the right upper abdomen and crossing the midline. Colour Doppler revealed internal vascularity with no evidence of fetal hemodynamic compromise. On serial follow-up the size of lesion progressively increased. A live male baby of weight 3.11kg with good APGAR scores was delivered at 37 weeks by LSCS. Neonate's abdomen was distended with a hard mass palpable in right hypochondrium. Ultrasound/CT abdomen was suggestive of teratoma. Alfa fetoprotein (AFP) was greater than 1 lakh with hyperbilirubinemia. Laparotomy/Tumour biopsy confirmed epithelial hepatoblastoma- a mix of fetal and embryonal type with no evidence of metastases. Post-neoadjuvant chemotherapy, a right extended hepatectomy with mass resection was done. The infant is now on follow up. Only around 10 percent of hepatoblastomas are detected antenatally. As there are no fetal interventions for hepatoblastoma, serial ultrasound monitoring for tumour growth, early signs of hydrops and amniotic fluid volume is critical. Delivery should be planned at a tertiary referral centre with neonatology, pediatric surgery/oncology inputs. Caesarean section is preferred in these cases to prevent tumour rupture and its complications. AFP though non-specific, is highly elevated in 90% of cases. CT imaging confirms extent and metastasis. HPE is mandatory and preferred treatment is neoadjuvant chemotherapy/tumour resection and postoperative chemotherapy.