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

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 r...

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Main Authors: Rebecca Maxwell, Beate Häberle, Roland Kappler, Dietrich von Schweinitz, Mark Rassner, Julia von Frowein, Irene Schmid
Format: Article
Language:English
Published: MDPI AG 2024-02-01
Series:Cancers
Subjects:
Online Access:https://www.mdpi.com/2072-6694/16/4/696
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author Rebecca Maxwell
Beate Häberle
Roland Kappler
Dietrich von Schweinitz
Mark Rassner
Julia von Frowein
Irene Schmid
author_facet Rebecca Maxwell
Beate Häberle
Roland Kappler
Dietrich von Schweinitz
Mark Rassner
Julia von Frowein
Irene Schmid
author_sort Rebecca Maxwell
collection DOAJ
description 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.
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spelling doaj.art-edd3819de4264aa088a9b243ad7c01cd2024-02-23T15:10:38ZengMDPI AGCancers2072-66942024-02-0116469610.3390/cancers16040696Hepatoblastoma Relapse—Findings from the German HB99 Trial and the German Liver Tumor RegistryRebecca Maxwell0Beate Häberle1Roland Kappler2Dietrich von Schweinitz3Mark Rassner4Julia von Frowein5Irene Schmid6Department of Pediatrics, Dr. von Hauner Children’s Hospital, University Hospital, LMU Munich, Lindwurmstraße 4, 80337 Munich, GermanyDepartment of Pediatric Surgery, Dr. von Hauner Children’s Hospital, University Hospital, LMU Munich, 80337 Munich, GermanyDepartment of Pediatric Surgery, Dr. von Hauner Children’s Hospital, University Hospital, LMU Munich, 80337 Munich, GermanyDepartment of Pediatric Surgery, Dr. von Hauner Children’s Hospital, University Hospital, LMU Munich, 80337 Munich, GermanyDepartment of Pediatrics, Dr. von Hauner Children’s Hospital, University Hospital, LMU Munich, Lindwurmstraße 4, 80337 Munich, GermanyDepartment of Pediatrics, Dr. von Hauner Children’s Hospital, University Hospital, LMU Munich, Lindwurmstraße 4, 80337 Munich, GermanyDepartment of Pediatrics, Dr. von Hauner Children’s Hospital, University Hospital, LMU Munich, Lindwurmstraße 4, 80337 Munich, GermanySurvival 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.https://www.mdpi.com/2072-6694/16/4/696hepatoblastomarelapseAFPirinotecan
spellingShingle Rebecca Maxwell
Beate Häberle
Roland Kappler
Dietrich von Schweinitz
Mark Rassner
Julia von Frowein
Irene Schmid
Hepatoblastoma Relapse—Findings from the German HB99 Trial and the German Liver Tumor Registry
Cancers
hepatoblastoma
relapse
AFP
irinotecan
title Hepatoblastoma Relapse—Findings from the German HB99 Trial and the German Liver Tumor Registry
title_full Hepatoblastoma Relapse—Findings from the German HB99 Trial and the German Liver Tumor Registry
title_fullStr Hepatoblastoma Relapse—Findings from the German HB99 Trial and the German Liver Tumor Registry
title_full_unstemmed Hepatoblastoma Relapse—Findings from the German HB99 Trial and the German Liver Tumor Registry
title_short Hepatoblastoma Relapse—Findings from the German HB99 Trial and the German Liver Tumor Registry
title_sort hepatoblastoma relapse findings from the german hb99 trial and the german liver tumor registry
topic hepatoblastoma
relapse
AFP
irinotecan
url https://www.mdpi.com/2072-6694/16/4/696
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