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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
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