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[Surgical therapy of hepatoblastoma in childhood]
D von Schweinitz1, H Hecker, D Bürger
1Kinderchirurgische Abteilung, Medizinische Hochschule Hannover.
Insights
Surgical therapy significantly improves outcomes for pediatric hepatoblastoma. High resection rates were achieved, with 75% of children remaining tumor-free after treatment. This highlights the importance of aggressive surgical management in treating this rare liver cancer.
Area of Science:
- Pediatric Oncology
- Surgical Oncology
- Hepatobiliary Surgery
Context:
- Hepatoblastoma is a rare, highly malignant liver tumor predominantly affecting young children.
- Treatment protocols have evolved to improve surgical and chemotherapeutic strategies.
- The German Society for Paediatric Oncology and Haematology's HB89 protocol guided treatment for 71 children.
Purpose:
- To evaluate the effectiveness of surgical therapy in children with hepatoblastoma treated under the HB89 protocol.
- To assess the impact of surgical resection and adjuvant chemotherapy on patient outcomes.
- To analyze recurrence-free survival rates based on disease stage.
Summary:
- An initial laparotomy was standard, with lobectomy for localized tumors and neoadjuvant chemotherapy (ifosfamide, cisplatin, adriamycin - IPA) for advanced or metastatic disease.
- Tumor reductive chemotherapy followed by second-look surgery was employed for extended or metastatic hepatoblastomas.
- An overall resection rate of 92% was achieved, with 75% of patients remaining tumor-free after a median follow-up of 3.8 years.
Impact:
- The study demonstrates a high overall resection rate (92%) for hepatoblastoma, leading to a 75% tumor-free survival rate.
- Recurrence-free survival varied significantly by stage, with 100% for Stage I and 29% for Stage IV.
- Findings underscore the critical role of aggressive surgical intervention and multimodal therapy in managing pediatric hepatoblastoma.
Abstract:
Hepatoblastoma is a rare but highly malignant liver tumour, predominantly occurring during early childhood. Clinical data of 71 children treated from 1988 to 1993 according to the protocol of the multicentre liver tumour study (HB89) of the German Society for Paediatric Oncology and Haematology were evaluated to assess the effects of surgical therapy in these patients. The protocol prescribed an initial laparotomy for all children with a liver tumour, except for infants with distant metastases and a high serum-alpha-fetoprotein; for small hepatoblastomas confined to one liver lobe primary resection by a lobectomy; and for those extending into both lobes and metastasizing tumours tumour reductive chemotherapy including ifosfamide, cisplatin and adriamycin (IPA), preparatory to resection at second-look surgery. All patients received adjuvant IPA chemotherapy after tumour resection. In 20 of the 71 cases, the hepatoblastomas were completely resected at initial laparotomy (stage I), while in 6 cases microscopic residues were left (stage II). In 38 of 45 children with extended hepatoblastomas (stage III, 38 patients) or distant metastases (stage IV, 7 patients) the tumour was resected during later surgery, and 1 patient received a liver transplant. Thus, the overall resection rate was 92% (65 of 71 hepatoblastomas). After a median follow-up of 3.8 years (range 1.0-5.9 years), 53 (75%) of the patients were tumor-free and 18 (25%) had died. Recurrence-free survival was 100% for stage I, 50% for stage II, 71% for stage III and 29% for stage IV patients (P = 0.0009).(ABSTRACT TRUNCATED AT 250 WORDS)