Surgery for hepatoblastoma in children with trisomy 18: a monocentric study
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In brief
In brief
This monocentric study examines surgical outcomes for seven children with trisomy 18 and hepatoblastoma who underwent multidisciplinary treatment including chemotherapy and complete tumor resection. All patients achieved recurrence-free survival with acceptable perioperative complications, suggesting that children with trisomy 18 and stable cardiopulmonary status may benefit from active oncologic treatment for hepatoblastoma.
- Children with trisomy 18 and hepatoblastoma can achieve complete surgical resection with multidisciplinary treatment when cardiopulmonary status is stable.
- Neoadjuvant chemotherapy effectively reduced tumor size in 3/7 cases, facilitating complete resection across PRETEXT I-III classifications.
- All seven trisomy 18 patients who underwent multidisciplinary treatment remained recurrence-free (follow-up: 3 months to 11 years).
- Perioperative complications were manageable: one circulatory failure case and two bile leakage cases among seven surgical patients.
- Active treatment paradigm shift: trisomy 18 patients with stable cardiopulmonary function are now viable candidates for hepatoblastoma surgery.
Written by the GCMD Library team from the article.
Abstract
Purpose
Recently, children with trisomy 18 have been receiving more active treatment for malignancies. We report herein seven cases complete resection was achieved, and discuss multidisciplinary treatment for hepatoblastoma in patients with trisomy 18.
Method
The medical records of children with trisomy 18 who were treated at the study center between 2010 and 2023 were reviewed.
Result
Six of 69 patients had hepatoblastoma development, and three of these underwent multidisciplinary treatment. In addition, 6 patients had been referred by another hospital for treatment, and four of these underwent multidisciplinary treatment. Among the seven patients who underwent multidisciplinary treatment, three, two, and two were categorized in Pre-treatment Extent of Disease (PRETEXT) classification group I, II, and III, respectively. Neoadjuvant chemotherapy resulting in tumor reduction was performed in three cases. In all the cases, complete resection was achieved with pathologically safe margins. Perioperative complications included circulatory failure in one case and bile leakage in two cases. Adjuvant chemotherapy was administered in four cases. The postoperative observation period ranged from 3 months to 11 years, and all the patients are recurrence-free.
Conclusion
Children with trisomy 18 complicated with hepatoblastoma whose cardiopulmonary conditions are stable may be good candidates for chemotherapy and surgery.