Abstract Rationale Hepatoblastoma is the most common primary liver cancer in children. Primary risks include prematurity, very low birth weight (VLBW), and genetic predisposition syndromes. Other external factors include supplemental oxygen use and exposure to mechanical ventilation and parental nutrition. Since these overlap with risk factors for bronchopulmonary dysplasia (BPD), preterm infants with BPD may be at increased risk for hepatoblastoma. However, clinical characteristics of hepatoblastoma in this growing population are poorly understood. We aimed to describe hepatoblastoma presentations in preterm infants, focusing on BPD and genetic predisposition, and to explore whether identifiable clinical patterns might inform targeted screening strategies. Methods An IRB-approved, retrospective review was completed to determine clinical characteristics in premature infants with hepatoblastoma seen at Children’s Hospital of Philadelphia between January 1, 2005 to July 31, 2025. Inclusion criteria included hepatoblastoma diagnoses in patients born less than 37 weeks gestation. Data were collected via chart review. BPD severity was classified by the 2001 NHLBI consensus statement. Results Fifty-one patients (51% male) met inclusion criteria. Thirty-eight (75%) required mechanical ventilation in the first 28 days of life. Thirty-six (70%) met criteria for BPD (31 severe, 4 mild). We then identified three sub-cohorts. Patients with BPD only (n = 27; 25 severe, 2 mild), patients with genetic syndromes (n = 16; including 7 with severe BPD and 2 with mild BPD), and patients with no BPD and no genetic syndrome (n = 8). Beckwith-Wiedemann Syndrome (n = 12) was the most common genetic syndrome. Birth characteristics differed between these cohorts (p 0.001) with the BPD cohort having the lowest gestational age (mean 25.9 weeks) and birth weight (788g) compared with genetic (34.1 weeks; 2,091g) and no BPD/no genetic (34.9 weeks; 2,473g) cohorts. Small-for-gestational-age status was most common in BPD only (46% vs. 19% and 13%; p = 0.079). Age at diagnosis differed significantly (p = 0.027) with genetic syndrome patients diagnosed earliest (mean 295 days, with 13 detected through cancer surveillance), followed by BPD only (638 days) and no BPD/no genetic (1,041 days). Conclusion Hepatoblastoma in premature infants clustered in those with genetic predisposition syndromes and those with BPD. The BPD cohort was uniquely characterized by extremely low gestational age and birth weight. Earlier diagnosis in the Genetic and BPD groups likely reflects the intensity of postnatal surveillance, with longest diagnostic delays occurring among infants without either risk factor. These findings indicate that preterm infants with BPD may represent a high-risk group in whom enhanced clinical awareness could facilitate timelier hepatoblastoma detection. This abstract is funded by: None
Bowman et al. (Fri,) studied this question.