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Six-month old Sabina Cimpeanu has hepatoblastoma, a liver condition that affects one in a million children each year. Her Romanian parents have travelled to Scotland in the hope that doctors there can save her life. Read More...

Image courtesy of The Herald & Evening Times picture archive

About Siopel

INTRODUCTION

The SIOPEL Childhood Liver Tumour Study Group represents a committee of the International Society of Paediatric Oncology exclusively dedicated to promote co-operative clinical and basic research in the field of primary childhood liver tumours. The ultimate goal of the study group is to ameliorate the prognosis and the quality of life of children affected by this rare neoplasm. As a whole primary hepatic tumours represent less than 1% of all childhood malignancies. Their frequency according to the different series is of about 0.8 - 1 case per million children less than 15 years of age per year. Hepatoblastoma (HB) is by far the commonest histological subtype of primary malignant hepatic tumours occurring in children less than 15 years of age, followed by hepatocellular carcinoma (HCC).

The headquarter of the SIOPEL Childhood Liver Tumour Study Group is located in the United Kingdom Childrens Cancer Study Group Trial Office, located at the University of Leicester, Department of Epidemiology & Public Health in Leicester. The Group is composed of basic and clinical scientists coming form different European and Extra-European countries.

The Study group "was born" in Jerusalem (Israel) in 1987 at the time of XIX SIOP annual conference. However, it was only three years later that the first co-operative clinical trial was launched. As of to-day the SIOPEL Liver Tumour Study group is at its third generation of clinical trials and almost 400 children affected by hepatoblastoma or hepatocellular carcinoma have been registered in the trials. Among other initiatives the study group has also promoted two international workshops on the biological and clinical aspects of childhood malignant liver tumours (Vienna 1996 and Bern 1999) which saw a large international participation of experts in this field coming form the whole word.

AIMS OF THE SIOPEL LIVER TUMOUR STUDIES - SIOPEL 3

This is the third study for children with Hepatoblastoma (HB) or Hepatocellular Carcinoma (HCC) run by the SIOP (International Society of Paediatric Oncology) Liver Tumour Group.

For 'standard' risk HB patients, i.e. those in whom the tumour has not spread outside the liver and involves, in the liver itself, either one, two, or three of the four sections of the liver, the main aim is to determine whether single agent Cisplatin is less toxic and as effective, in terms of response to chemotherapy and resection rate of the tumour, as Cisplatin plus Doxorubicin.

For 'high' risk HB patients, i.e. those patients with tumour throughout the whole of the liver, or whose tumour has spread beyond the confines of the liver, and all HCC patients, the study aims to evaluate whether more intensive chemotherapy using 3 drugs, Carboplatin and Doxorubicin alternating with Cisplatin, improves the response rate to chemotherapy and subsequent resection rate compared to similar patients treated with Cisplatin and Doxorubicin in the SIOPEL 1 study.

Other objectives of these studies are:

1. to further validate the pre-treatment tumour extension system (PRETEXT) used for the previous SIOPEL studies. This is an assessment of the extent of disease, by radiological investigations, prior to any treatment.
2. to evaluate the feasibility of a rapid central review by a panel of experts of tumour extent and resectability in difficult cases.
3. to evaluate the role of orthotopic liver transplantation for patients whose tumour remains unresectable after chemotherapy.
4. To evaluate an appropriate salvage therapy (initially high dose cyclophosphamide) for relapsed patients.