Pediatric Hepatoblastoma: A Single-Institution Case Series

Authors

Keywords:

Hepatoblastoma in Children, Neoadjuvant Therapy, Prune Belly Syndrome, Vesicoureteral Reflux

Abstract

Introduction: Hepatoblastomas comprise up to two-thirds of malignant liver masses in childhood and rank as the third most common malignant neoplasm in children under the age of three. Treatment and prognosis are highly dependent on tumor staging and characteristics. Our study’s aim is to analyze the clinical findings and outcomes of hepatoblastoma at our institution.

Methods: After Institutional Review Board (IRB) approval was granted from Loma Linda University #5240020, we conducted a retrospective review on all patients under the age of 18 with a diagnosis of hepatoblastoma between February 2000 to January 2022. Variables assessed included demographics, work up, surgical intervention, recurrence, and mortality.

Results: Fifteen patients were diagnosed with hepatoblastoma in that timeframe. Mean age was 18.7 months. Associated comorbidities included three patients with prematurity, one patient with Beckwith-Wiedemann Syndrome, and two unique presentations each of Prune Belly Syndrome and grade IV/V vesicoureteral reflux. There were four mortalities, two due to relapse in disease, one due to pulmonary and CNS metastasis at diagnosis, and another due to sepsis and multi-organ failure. Eleven patients continued monitoring without tumor recurrence. All patients were treated based on the Children’s Oncology Group (COG) guidelines.

Conclusion: Risk stratification is an important component of hepatoblastoma management. Our cohort demonstrated novel comorbidities of Prune Belly Syndrome and vesicoureteral reflux. Eleven patients received neoadjuvant chemotherapy that allowed for subsequent surgical resection. Our mortalities were associated with tumor metastasis and recurrence consistent with elevated alpha-fetoprotein (AFP) values. Future research should involve multi-institutional studies focusing on comorbidities and genetic analysis.

Author Biography

Andrei Radulescu, MD, PhD. Division of Pediatric Surgery, Department of Surgery, Loma Linda University Children’s Hospital, Loma Linda, CA, USA.

 

 Loma Linda University School of Medicine, Loma Linda, CA, USA.

References

Küpesiz, F. T., Akınel, A. N., Akbaş, H., Sivrice, Ç., Kintrup, G. T., Karagüzel, G., et al. Multidisciplinary Management of Pediatric Hepatoblastoma: A 20-Year Single-Center Experience. The Turk J Gastroenterol. 2022;33(12):1069.

Zhang Y, Zhang W, Tang S, Chen L, Yi Y, Zhang P, et al. A single-center retrospective study of pediatric hepatoblastoma. Oncoly. Lett. 2016 Nov 1;12(5):3919-25.

Pateva, I. B., Egler, R. A., & Stearns, D. S. Hepatoblastoma in an 11-year-old: Case report and a review of the literature. Medicine. 2017 Jan 1:, 96(2): e5858.

Lack, E. E., Neave, C., & Vawter, G. F. Hepatoblastoma: A clinical and pathologic study of 54 cases. Am J Surg Pathol. 1982 Dec 1;6(8):693-705.

Spector, L. G., & Birch, J. The epidemiology of hepatoblastoma. Pediatr Blood Cancer. 2012 Nov;59(5):776-9.

El Asmar, A., & El Rassi, Z. Hepatoblastoma in childhood, long term survival achieved: 2 case reports and literature review. Int. J. Surg. Case Rep. 2016 Jan 1;21:55-8.

Hager, J., & Sergi, C. M. Hepatoblastoma. Exon Publications. 2021 Apr 6:145-64.

Hiyama, E. Pediatric hepatoblastoma: diagnosis and treatment. Transl. Pediatr. 2014 Oct;3(4):293.

Meyers, R., Hiyama, E., Czauderna, P., & Tiao, G. M. Liver tumors in pediatric patients. Surg. Oncol. Clin. N. Am.. 2021 Apr 1;30(2):253-274.

Kulkarni, S., Brauer, D. G., Turmelle, Y., Stoll, J., Nadler, M., Chapman, W. C., et al. Surgical therapy for pediatric hepatoblastoma in the USA over the last decade: analysis of the national cancer database. J Gastrointest Cancer. 2021 Jun; 52:547-556.

Bharti S, Bharti JN, Sinha A, Yadav T. Common and rare histological variants of hepatoblastoma in children: a pathological diagnosis and review of the literature. Gastrointest. Tumors. 2021 Apr 8;8(2):41-6.

Abdul-Rahman OA, Edghill EL, Kwan A, Enns GM, Hattersley AT. Hypoplastic glomerulocystic kidney disease and hepatoblastoma: a potential association not caused by mutations in hepatocyte nuclear factor 1β. J Pediatr Hemato Oncol. 2009 Jul 1;31(7):527-9.

Sattler B, Gunawan B, Lorf T, Müller D, Ringe B, Füzesi L. Undifferentiated small-cell hepatoblastoma. Der Pathologe. 2000 Nov 1;21(6):456-9.

Sebestyen J, Garg U, Lewing KB, Warady BA, Abdel-Rahman S, Blowey DL. Cisplatin pharmacokinetics in a child receiving peritoneal dialysis. Pediatr Nephrol. 2010 Jun;25:1185-9.

Khosla A. Hepatoblastoma and congenital dysplastic kidney. J Pediatr Surg. 1990 Aug;25(8):924.

Majidinia M, Aghazadeh J, Jahanban-Esfahiani R, Yousefi B. The roles of Wnt/-catenin pathway in tissue development and regenerative medicine. J Cell Physiol. 2018 Aug; 223(8):5598-612

Becknell B, Pais P, Onimoe G, Rangarajan H, Schwaderer AL, McHugh K, Ranalli MA, Hains DS. Hepatoblastoma and prune belly syndrome: a potential association. Pediatr Nephrol. 2011 Aug;26:1269-73.

Sharma, D., Subbarao, G., & Saxena, R. Hepatoblastoma. Seminars in diagnostic pathology. 2017 Mar 1 (Vol. 34, No. 2, pp. 192-200). WB Saunders.

Perilongo, G., Malogolowkin, M., & Feusner, J. Hepatoblastoma clinical research: lessons learned and future challenges. Pediatr Blood Cancer. 2012 Nov; 59(5):818-21.

Agha RA, Sohrabi C, Mathew G, Franchi T, Kerwan A, O’Neill N for the PROCESS Group. The PROCESS 2020 Guideline: Updating Consensus Preferred Reporting Of Case Series in Surgery (PROCESS) Guidelines, Int. J Surg. 2020;60

Zhou S, Malvar J, Chi YY, Stein J, Wang L, Genyk Y, Sposto R, Mascarenhas L. Independent assessment of the children’s hepatic tumors international collaboration risk stratification for hepatoblastoma and the association of tumor histological characteristics with prognosis. JAMA Netw Open. 2022 Feb 1;5(2):e2148013-.

Srinivasan S, Prasad M, Parambil BC, Shrimal A, Gollamudi VR, Subramani V, Ramadwar M, Khanna N, Baheti AD, Gala K, Patil V. Treatment outcomes and prognostic factors in children with hepatoblastoma using a risk‐stratified approach. Pediatr Blood Cancer. 2023 Jul;70(7):e30302.

Downloads

Published

2024-11-19

How to Cite

Sawaya, J., McRae, J., Mladenov, G. D., & Radulescu, A. (2024). Pediatric Hepatoblastoma: A Single-Institution Case Series. International Journal of Medical Students. Retrieved from https://ijms.info/IJMS/article/view/2735

Issue

Section

Experience

Categories