Hepatoblastoma Relapse—Findings from the German HB99 Trial and the German Liver Tumor Registry

Author:

Maxwell Rebecca1,Häberle Beate2,Kappler Roland2ORCID,von Schweinitz Dietrich2,Rassner Mark1,von Frowein Julia1,Schmid Irene1

Affiliation:

1. Department of Pediatrics, Dr. von Hauner Children’s Hospital, University Hospital, LMU Munich, Lindwurmstraße 4, 80337 Munich, Germany

2. Department of Pediatric Surgery, Dr. von Hauner Children’s Hospital, University Hospital, LMU Munich, 80337 Munich, Germany

Abstract

Survival rates for HB patients have improved; however, outcomes for patients who relapse remain poor. A retrospective review of information gathered for the HB99 study and the German Liver Tumor Registry identified 25 relapse patients (6.9%, 25/362). The median time from initial diagnosis to first relapse was 13 months (range: 5–66 months). Two patients relapsed >36 months after initial diagnosis. A total of 68% (17/25) of relapses were metastatic, 24% local, and 8% combined. 67% of local relapses were alive at the last follow-up, in contrast to 53% of metastatic and 0% of combined relapses. At the last follow-up, 73% (8/11) of patients with lung relapses were still alive (0/4 with peritoneal, 1/2 with CNS involvement). A total of 20% of the patients had AFP-negative relapses, 64% of the relapse patients achieved a second complete remission, 69% were still in complete second remission at the last follow-up (median FU of 66 months), and 83% (5/6) of irinotecan-naïve patients who received relapse treatment including irinotecan were in second complete remission at the last follow-up. The 3-year overall survival/event-free survival from relapse was 63%/48% respectively. There is a good chance that HB patients will achieve a second remission despite a first relapse. However, patients who suffer further relapses tend to have a poorer prognosis.

Funder

Deutsche Kinderkrebsstiftung

European Union’s Horizon 2020 Research and Innovation Programme

Publisher

MDPI AG

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