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[Hepatoblastoma: a catamnestic study and proposal of a therapy concept]
H Mildenberger1, D Bürger, P Weinel
1Abteilung für Kinderchirurgie, Medizinischen Hochschule Hannover.
Insights
This study on hepatoblastoma in children found that 40% of patients were alive and disease-free after treatment. Aggressive surgical strategies and preoperative chemotherapy may improve outcomes for this rare liver cancer.
Area of Science:
- Pediatric Surgery
- Pediatric Oncology
- Hepatobiliary Surgery
Context:
- Hepatoblastoma is a rare malignant liver tumor in children.
- Management of hepatoblastoma requires a multidisciplinary approach.
- Surgical resection is a cornerstone of treatment.
Purpose:
- To evaluate treatment outcomes for hepatoblastoma patients.
- To correlate therapeutic strategies with patient survival.
- To propose an optimized treatment protocol for hepatoblastoma.
Summary:
- Twenty-eight pediatric hepatoblastoma cases were reviewed, with 81% undergoing resection (trisegmentectomy or lobectomy).
- Operative mortality was 14%, and 11 of 27 patients (40%) were alive and disease-free long-term.
- Chemotherapy and aggressive surgical approaches, including liver transplantation, were evaluated.
Impact:
- Findings support more aggressive surgical strategies for hepatoblastoma.
- Preoperative chemotherapy may enhance resectability and survival.
- A proposed national multicenter prospective study protocol aims to standardize hepatoblastoma treatment.
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)