Das kindliche Hepatoblastom

Zusammenfassung

Das Hepatoblastom (HB) ist ein hoch maligner, embryonaler Lebertumor des frühen Kindesalters. Neben Fieber, tastbarem Oberbauchtumor und Thrombozytose haben 80% der Patienten ein exzessiv erhöhtes Serum-Alpha-Fetoprotein. Die diagnostische Bildgebung beruht auf Sonographie, CT und MRT. Wegen meist gutem Ansprechen sollte das Hepatoblastom in der Regel primär mit Chemotherapie behandelt werden, da danach eine Resektion sicherer und risikoärmer ist. Die wichtigsten Medikamente sind Cisplatin und Doxorubicin. Die Therapie für Standard-Risiko-HB (SR-HB) der GPOH-Studie HB99 enthält Ifosfamid (3 g/m²), Cisplatin (100 mg/m²) und Doxorubicin (60 mg/m²) mit einer Ansprechwahrscheinlichkeit von 95% der Tumoren. Disseminierte und metastasierende Hochrisiko-HB benötigen intensivere Chemotherapie entsprechend den aktuellen kooperativen Studienprotokollen. Voraussetzung für eine Heilung ist die radikale chirurgische Resektion des Tumors, in Einzelfällen auch als Lebertransplantation. Die Gesamtremissionsrate liegt derzeit bei 75%, für SR-HB beträgt sie 90%, für HR-HB jedoch nur 30%.

Summary

The hepatoblastoma (HB) is a highly malignant embryonal liver tumor of early childhood. Besides fever, thrombocytosis and a palpable mass in the upper abdomen, 80% of the patients have an excessively elavated serum-alpha-fetoprotein. Imaging preferably relies on abdominal ultrasound, as well as CT- and MRI-scans. Because of its normally good response, HB should be primarily treated with chemotherapy. Thereafter, a resection usually is easier and less risky. The chemotherapy regimen of the German Cooperative Study HB99 for standard-risk HB consists of ifosfamide (3 g/m²), Cisplatin (100 mg/m²) and doxorubicin (60 mg/m²), which has a response rate of 95%. Disseminated and mestastazising high-risk HB receive intensified chemotherapy according to actual trial protocols. A prerequisite for cure is a radical tumor resection. In selected cases, a liver transplantation is feasible. The overall remission rate reaches 75% nowadays, it is 90% for standard-risk HB but only 30% for high-risk HB.

Schlüsselwörter

Hepatoblastom - Chemotherapie - Chirurgie

Keywords

Hepatoblastoma - chemotherapy - surgery

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