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Unresectable multifocal hepatoblastoma with cardiac extension: excellent response with HB-94 chemotherapy protocol
Nazan Sarper1, Funda Corapçioğlu, Yonca Anik
1Departments of Pediatrics, Division of Hematology-Oncology, Kocaeli University, Izmit-Kocaeli, Turkey. nazan_sarper@hotmail.com
Insights
This case study highlights a rare hepatoblastoma in an infant, successfully treated with chemotherapy despite unresectable disease. The infant achieved long-term remission, challenging traditional treatment paradigms for this pediatric liver cancer.
Area of Science:
- Pediatric Oncology
- Hepatobiliary Malignancies
Background:
- Hepatoblastoma is a rare malignant liver tumor primarily affecting infants and young children.
- Complete surgical resection is considered the primary curative treatment for hepatoblastoma.
Observation:
- A 10-month-old infant presented with advanced hepatoblastoma, including extensive liver involvement, inferior vena cava thrombosis, and elevated alpha-fetoprotein.
- The tumor was deemed unresectable, and liver transplantation was not feasible due to high risks.
Findings:
- Neoadjuvant chemotherapy (IPA regimen, carboplatin, etoposide) led to clinical remission and normalization of tumor markers.
- Despite minimal tumor regression and increased calcification, the patient achieved a 100% Karnofsky score at 43 months post-diagnosis.
Implications:
- This case suggests that aggressive chemotherapy may induce long-term remission in unresectable hepatoblastoma, even without surgical resection or transplantation.
- Further research into chemotherapy protocols for unresectable hepatoblastoma is warranted to improve outcomes for high-risk pediatric patients.
Abstract:
A 10-month-old white infant presented with abdominal distention and bilateral scrotal hernia. Imaging studies of the abdomen and thorax showed a huge liver with multiple tumor masses and calcification involving all the segments. There was thrombosis in the inferior vena cava and right atrium. alpha-Fetoprotein was 246,000 IU/mL. HB-94 chemotherapy protocol was started at once due to rapid deterioration of the patient. Surgical biopsy performed after the first IPA (ifosfamide, cisplatin, doxorubicin) course showed hepatoblastoma with macrotrabecular variant. After a second IPA course and 2 courses of carboplatin and etoposide, the boy's clinical condition was excellent with normal alpha-fetoprotein but minimal regression and increased calcification in the tumor mass. Hepatic tumor was unresectable and no surgical intervention was performed. Transplantation could not be performed because of high morbidity and mortality. Despite general agreement that complete surgical resection is the cornerstone of treatment for patients with hepatoblastoma, the patient is in remission with 100% Karnofsky score in the 43 months of diagnosis.
