Surgery for hepatoblastoma in children with trisomy 18: a monocentric study

• Published in: Pediatric surgery international 2024-08, Vol.40 (1), p.223
• Main Authors: Hirohara, Kazuki, Tomita, Hirofumi, Shimojima, Naoki, Tsukizaki, Ayano, Mori, Teizaburo, Minegishi, Hidehiro, Makimoto, Atsushi, Yuza, Yuki, Matsuoka, Kentaro, Shimotakahara, Akihiro
• Format: Article
• Language: English
• Subjects: Chemotherapy; Child; Child, Preschool; Female; Hepatectomy - methods; Hepatoblastoma - genetics; Hepatoblastoma - surgery; Humans; Infant; Liver Neoplasms - genetics; Liver Neoplasms - surgery; Male; Medicine; Medicine & Public Health; Original Article; Pediatric Surgery; Pediatrics; Retrospective Studies; Surgery; Treatment Outcome; Trisomy - genetics; Trisomy 18 Syndrome - complications; Trisomy 18 Syndrome - genetics; Trisomy 18 Syndrome - surgery
• ISSN: 0179-0358; 1437-9813; 1437-9813
• DOI: 10.1007/s00383-024-05813-x

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.