Teratoid Hepatoblastoma: Clinicopathologic Features and Outcomes

Teratoid hepatoblastoma (HB) is a rare histologic subtype of the most common pediatric liver cancer. Understanding of the clinicopathologic features and outcomes of patients with this disease is currently limited.

Multi-institution, retrospective chart review was conducted to compare 20 children with teratoid HB to 129 children with non-teratoid HB treated from 2004 to 2022.

Of the patients with teratoid HB, 31% showed teratoid histology in pre-treatment biopsies. All 20 patients received neoadjuvant chemotherapy, with treated tumors showing melanin (14), neuroepithelium with or without glial cells (10), squamous epithelium and whorls (9), mucinous and/or non-mucinous glandular elements (8), primitive endoderm/yolk sac-like areas (3), and mature and/or immature ganglion cells (3). Metastatic and relapsed lesions did not show teratoid histology in this cohort. Teratoid HB was more frequent in males (M:F 1.9:1) and children <5 years old. Patients with teratoid HB were less chemoresponsive and had more metastases at presentation. Rates of prematurity, multifocal disease, vascular invasion, primary surgical management strategy (i.e., resection or transplant), relapse, and survival were comparable between the two groups.

Teratoid HB occurs in younger patients and is more likely to be chemoresistant. Despite higher rates of metastases at presentation, survival was comparable between the two groups. This is one of the largest series evaluating the clinicopathologic features and outcomes of children with teratoid-HB and is the first to directly compare outcomes between teratoid and non-teratoid subtypes.