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Annals of Diagnostic Paediatric Pathology 2006, 10(1–2):17–22
© Copyright by Polish Paediatric Pathology Society Annals of
Primary malignant liver cell tumours in children –
different treatment strategies
Andrzej Igor Prokurat 1,2 ,Ma³gorzata Chrupek 1,2 , Roman KaŸmirczuk 3,4 , Przemys³aw Kluge 5 ,
Andrzej Koœciesza 6 , Pawe³ Rajszys 6 , El¿bieta Dzik 6 , Arthur Zimmermann 7
Diagnostic
Paediatric
Pathology
1
Department of Paediatric Surgery
3
Department of Anaestesiology and Intensive Care Unit
L. Rydygier Collegium Medicum
N. Copernicus University in Toruń, Poland
2
Department of Paediatric Surgery and Organ Transplantation
4
Department of Anaestesiology and Intensive Care Unit
5
Department of Pathology
6
Department of Radiology
The Children's Memorial Health Institute in Warsaw, Poland
7
Department of Surgical Pathology
Institute of Pathology of the University in Berne, Switzerland
Abstract
Address for correspondence
Malignant liver cell tumours in children still present a real diagnstic and therapeutic challenge. The histology
of these tumours is often not clearly defined, and the differentiation between hepatoblastoma (HBL) and
hepatocellular carcinoma (HCC) is sometimes difficult. Intermediate forms between HBL and HCC called
„Transitional Liver Cell Tumours” (TLCT) can also be observed. The introduction of complex treatments
(chemotherapy and operation) has led to an increase in the 3-year survival rate of patients with HBL of over
70% but not improved the survival rate of children with HCC and TLCT, which is still not higher than 40%.
The most important factors affecting the final results of treatment in children with malignant liver cell
tumours still are microscopic radical operations, the presence of extrahepatic spread of disease, and distant
metastases. The aim of this study is the analysis of the results of treatment in children with malignant liver
cell tumours using 3 different treatment strategies: typical anatomcal liver resection, atypical borderline non
anatomical liver resection for giant tumours and liver transplantation. The results of treatment of 59 children
with primary malignant liver cell tumours treated during last 20 years have been analysed; the patients
consisted of 29 patients with HBL (children up to 4 years of age), 10 patients with TLCT (children over
5 years of age) and 20 patients with HCC. Liver resection (typical or atypical) was performed in 39 children
and liver transplantation (primary or secondary) was performed in 8 children. Type and size of tumour,
response of tumour to chemotherapy, the possibility of radical resection, type of operation and its microscopic
radicality and final results of complex treatment against the morphology of the tumour were analysed in all
patients. Clear differences in the treatment response are indicated, depending on the histology of the tumour.
Very good results with a complex treatment have been confirmed in the group of small children with HBL,
except for patients with an unfavourable histology (anaplasia or SCUD-small cell undifferentiated HBL),
patients with TLCT and HCC demonstrated unsatisfactory results after complex treatment. Detailed
histological analysis also demonstrated difficulties in achieving microscopic radicality in the operative
treatment of patients with TLCT and HCC as well as in children with HBL in cases the tumour size exceeded
10 cm in diameter. In conclusions, it is suggested that it worth verifying the indications for traditional surgical
treatment and adapting the treatment to the histology of tumour as well as broadening the indications for
liver transplantation as a primary operation, which may guarantee microscopic surgical radicality.
Key words: liver tumours in children, liver resections, liver transplantation
Prof. Andrzej I. Prokurat Phone: +48 52 585 40 15
Department of Paediatric Surgery L.Rydygier Collegium Medicum Fax: +48 52 585 40 95
N. Copernicus University in Toruñ, Poland E-mail: aprokurat@cm.umk.pl
ul. M. Sk³odowskiej-Curie 9
04-736 Bydgoszcz, Poland