Das Hepatoblastom: Eine katamnestische Untersuchung und Vorschlag eines Therapiekonzeptes

Abstract

Since 1977, 28 infants and children with hepatoblastomas were seen in the Department of Paediatric Surgery, Hanover Medical School. Five tumours could only be biopsied. One 11-months-old infant had a successful liver transplantation done at the Dept. of Transplantation Surgery (Professor R. Pichlmayr). Following a biopsy for unresectable tumour, chemotherapy was instituted in 5 cases: three of these could be resected in a second look operation 10 to 26 weeks later. Altogether, 22 tumours (= 81%) were resected: 14 had an extended resection (trisegmentectomy), and 8 had a lobectomy. Operative mortality was 14%. Out of 27 patients which were operated upon (resection or biopsy) longer than one year ago, 11 are alive and free from disease (= 40%). Early metastatic disease was diagnosed 3 weeks to 5 months postoperatively in 5 cases: only one of these children survived, following an intensive course of chemotherapy. In another two patients lung metastases were found 14 resp. 20 months after operation: both of these are alive and free from disease following chemotherapy and thoracotomy. One patient died of brain metastases 3 years postoperatively. Results of therapy are correlated to primary findings, type of operation, chemotherapy and histological subtype of the tumour. In comparison with published data a possible advantage of more aggressive operative strategies is discussed, and the benefit of a preoperative chemotherapy is evaluated. As a result of these considerations a treatment protocol for hepatoblastomas is proposed, designed as a national multicentre prospective study conducted by the German Society of Paediatric Oncology (GPO).(ABSTRACT TRUNCATED AT 250 WORDS)