https://pubmed.ncbi.nlm.nih.gov/37244849/ hepatoblastoma

J Pediatr Surg. 2023 May 8;S0022-3468(23)00287-7.
doi: 10.1016/j.jpedsurg.2023.05.001.Online ahead of print.

Surgical Management and Outcomes of Patients with Multifocal Hepatoblastoma

Richard S Whitlock 1, Jorge I Portuondo 1, Andres F Espinoza 1, Rachel Ortega 1, N Thao N Galván 2, Daniel H Leung 3, Dolores Lopez-Terrada 4, Prakash Masand 5, HaiThuy N Nguyen 5, Kalyani A Patel 4, John A Goss 2, Andras M Heczey 6, Sanjeev A Vasudevan 7

Abstract

Objective: To compare the outcomes of patients with multifocal hepatoblastoma (HB) treated at our institution with either orthotopic liver transplant (OLTx) or hepatic resection to determine outcomes and risk factors for recurrence.

Background: Multifocality in HB has been shown to be a significant prognostic factor for recurrence and worse outcome. The surgical management of this type of disease is complex and primarily involves OLTx to avoid leaving behind microscopic foci of disease in the remnant liver.
Methods: We performed a retrospective chart review on all patients <18 years of age with multifocal HB treated at our institution between 2000 and 2021. Patient demographics, operative procedure, post-operative course, pathological data, laboratory values, short- and long-term outcomes were analyzed.

Results: A total of 41 patients were identified as having complete radiologic and pathologic inclusion criteria. Twenty-three (56.1%) underwent OLTx and 18 (43.9%) underwent partial hepatectomy. Median length of follow-up across all patients was 3.1 years (IQR 1.1-6.6 years). Cohorts were similar in rates of PRETEXT designation status identified on standardized imaging re-review (p = .22). Three-year overall survival (OS) estimate was 76.8% (95% CI: 60.0%-87.3%). There was no difference in rates of recurrence or overall survival in patients who underwent either resection or OLTx (p = .54 and p = .92 respectively). Older patients (>72 months), patients with a positive porta hepatis margin, and patients with associated tumor thrombus experienced worse recurrence rates and survival. Histopathology demonstrating pleomorphic features independently associated with worse rates of recurrence.

Conclusions: Through proper patient selection, multifocal HB was adequately treated with either partial hepatectomy or OLTx with comparable outcome results. HB with pleomorphic features, increased patient age at diagnosis, involved porta hepatis margin on pathology, and the presence of associated tumor thrombus may be associated with worse outcomes regardless of the local control surgery offered.