OBJECTIVE & AIMS
 
OBJECTIVE
Establish a multi-center,international database with prospective registration of pediatric patients presenting with unresectable primary malignant liver tumors (hepatoblastoma-HB, hepatocellular carcinoma-HCC - including HCC developed on chronic liver disease, epithelioid hemangioendothelioma-EHEM), with a remote data entry system, accessible on-line and worldwide
AIMS
The aims of the Observatory will be:

  • to fully define the role of primary liver transplantation in the treatment of pediatric HB/HCC and other rare malignancies
  • to identify patients at diagnosis who will need a total liver resection and a transplant
  • to define the optimal timing of liver transplantation
  • to define the potential role of pre-transplant neo-adjuvant and post-transplant adjuvant chemotherapy
  • to correlate the staging according to the PRETEXT system with other staging systems like the conventional tumor-node mentastases system for (adult) liver carcinomas (TNM) and the CCSG/POG staging system
  • to correlate the PRETEXT staging of individual patients at presentation with the PRETEXT staging at the time of transplantation (after neo-adjuvant chemotherapy) and with the anatomic observations made on the resected liver
  • to define the amount of post-transplant immunosuppression needed by these children already immunosuppressed at the time of transplantation due to neoadjuvant chemotherapy
  • to quantify the immunosuppressive status at transplantation by measuring the total lymphocyte count
  • to study the incidence, on the long term, of the additive nephrotoxicity of Cisplatin and calcineurin inhibitors

For hepatoblastoma

  • to study the impact of vascular invasion (including either the gross or the histologic involvement of vessels) on the relapse rate and the disease-free,patient survival
  • to study the impact of pulmonary metastases,at presentation,on the relapse rate and the disease-free,patient survival
  • to study the impact of the intrahepatic extent and the multifocality of the tumor on the recurrence rate and the disease-free patient survival

For HCC
  • to study the impact of histologic differentiation, gross and histologic vascular invasion, size and number of nodules on the relapse rate and the disease-free,patient survival