Management and Outcomes of Hepatoblastoma in Patients With Trisomy 18: A Systematic Review and Pooled Analysis of 70 Patients.

Autor: Ziogas IA; Division of Pediatric Surgery, Department of Surgery, University of Colorado School of Medicine, Children's Hospital Colorado, Aurora, CO 80045, USA. Electronic address: ioannis.ziogas@cuanschutz.edu., Kakos CD; Department of Surgery, Aristotle University of Thessaloniki School of Medicine, 54124, Thessaloniki, Greece., Kokkinakis S; Department of Surgery, University of Crete School of Medicine, 71500, Heraklion, Greece., Hills-Dunlap JL; Division of Pediatric Surgery, Department of Surgery, University of Colorado School of Medicine, Children's Hospital Colorado, Aurora, CO 80045, USA., Corkum KS; Division of Pediatric Surgery, Department of Surgery, University of Colorado School of Medicine, Children's Hospital Colorado, Aurora, CO 80045, USA., Acker SN; Division of Pediatric Surgery, Department of Surgery, University of Colorado School of Medicine, Children's Hospital Colorado, Aurora, CO 80045, USA., Diaz-Miron JL; Division of Pediatric Surgery, Department of Surgery, University of Colorado School of Medicine, Children's Hospital Colorado, Aurora, CO 80045, USA., Lovvorn HN 3rd; Department of Pediatric Surgery, Monroe Carell, Jr. Children's Hospital at Vanderbilt, Nashville, TN 37232, USA., Roach JP; Division of Pediatric Surgery, Department of Surgery, University of Colorado School of Medicine, Children's Hospital Colorado, Aurora, CO 80045, USA., Gosain A; Division of Pediatric Surgery, Department of Surgery, University of Colorado School of Medicine, Children's Hospital Colorado, Aurora, CO 80045, USA.
Jazyk: angličtina
Zdroj: Journal of pediatric surgery [J Pediatr Surg] 2024 Oct; Vol. 59 (10), pp. 161596. Date of Electronic Publication: 2024 Jun 11.
DOI: 10.1016/j.jpedsurg.2024.06.005
Abstrakt: Introduction: Predicted 1-year survival of children with trisomy 18 (T18) has increased to 59.3%. We aimed to systematically review the characteristics, management, and outcomes of children with T18 and hepatoblastoma.
Methods: A systematic literature review of the PubMed, Embase, Scopus, Web of Science, and Cochrane Library databases was performed according to the PRISMA 2020 statement (end-of-search date: 03/03/2024).
Results: Fifty studies reporting on 70 patients were included. The median age at diagnosis was 11.5 months, 85.9% were female (n = 55/64), and 15.0% had mosaic T18 (n = 6/40). Diagnosis was made during symptom evaluation (most commonly hepatomegaly or abdominal mass) in 45.5% (n = 15/33), incidentally in 24.2% (n = 8/33), during surveillance with abdominal ultrasound in 18.2% (n = 6/33), and at autopsy in 12.1% (n = 4/33). The median tumor size was 6.4 cm, 33.3% had multiple tumors (n = 14/42), and metastasis was present in one patient (3.8%; n = 1/26). Neoadjuvant chemotherapy was administered in 42.6% (n = 26/61) and adjuvant chemotherapy in 31.6% (n = 18/57). Surgical treatment was performed in 64.2% (n = 43/67). Of the patients not diagnosed on autopsy, overall mortality was 35.5% (n = 22/62) over a median follow-up of 11.0 months. Among the 26 deceased patients (including those diagnosed on autopsy), the most common causes of death were cardiopulmonary disease (38.5%, n = 10/26) and tumor progression (30.8%, n = 8/26).
Conclusions: T18 does not preclude resection with curative intent for hepatoblastoma. Combination of surgery and chemotherapy should be considered in children on an individualized basis depending on tumor characteristics and underlying cardiopulmonary comorbidities. Locoregional modalities may have a role in the setting of severe comorbidities.
Level of Evidence: Level IV evidence.
Competing Interests: Conflicts of interest The authors have no competing interests to declare.
(Copyright © 2024 Elsevier Inc. All rights reserved.)
Databáze: MEDLINE
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