Outcome of Hepatoblastoma in A Low-and Middle-Income Country; Single Centre  Experience from Pakistan

Rabiha  Manzoor; Tariq  Ghafoor; Awais  Arshad; Beenish  Hira; Ayesha  Latif; Fouzia  Khan

doi:10.51253/pafmj.v75i3.11816

Authors

Rabiha Manzoor Department of Pediatric Oncology, Combined Military Hospital, Rawalpindi/National University of Medical Sciences (NUMS) Pakistan
Tariq Ghafoor Department of Pediatric Oncology, Armed Forces Bone Marrow Transplant Centre, Rawalpindi/National University of Medical Sciences (NUMS) Pakistan
Awais Arshad Department of Pediatric Oncology, Combined Military Hospital, Rawalpindi/National University of Medical Sciences (NUMS) Pakistan
Beenish Hira Department of Pediatric Oncology, Combined Military Hospital, Rawalpindi/National University of Medical Sciences (NUMS) Pakistan
Ayesha Latif Department of Pediatric Oncology, Combined Military Hospital, Rawalpindi/National University of Medical Sciences (NUMS) Pakistan
Fouzia Khan Department of Pediatric Oncology, Combined Military Hospital, Rawalpindi/National University of Medical Sciences (NUMS) Pakistan

DOI:

https://doi.org/10.51253/pafmj.v75i3.11816

Keywords:

Abdominal distension, Hepatoblastoma, Jaundice, Mortality, surgery.

Abstract

Objective: To determine the survival outcome of patients with hepatoblastoma (HB) treated at a tertiary care hospital of Pakistan.

Study Design: A prospective cohort study.

Place and Duration of Study: Pediatric oncology unit, Combined Military Hospital, Rawalpindi Pakistan from Jan 2013 to Dec 2023.

Methodology: All cases of hepatoblastoma (HB) diagnosed in individuals aged up to 12 years were enrolled. The study primarily focused on documenting survival outcomes, encompassing both "overall survival (OS)" and "disease-free survival (DFS).

Results: In a total of 29 patients with HB, 18(62.1%) were boys. The median age at the time of diagnosis was 1.3 years (0.8 to 2.8 years). At presentation, abdominal distension was present in all 29(100%) cases whereas palpable abdominal mass, and jaundice was present in 25(86.2%), and 9(31.0%) patients, respectively. Mortality was reported in 14(48.3%) patients. The median DFS, and OS were 18.64 months (5.27 to 47.04 months), and 0.13 months (0 to 44.00 months), respectively. Surgery emerged as a significant predictor of mortality, with a striking 15.89 times higher odds of mortality if surgery was not performed (95% CI: 1.71-147.39, p=0.015). Overall, 8-year survival was 50.0%. Eight year cumulative DFS was 59.3% for standard risk, 44.4% for high risk, and 26.7% for very high risk patients. OS was 60.0% for standard risk, 44.4% for high risk, and 40.0% for very high risk patients.

Conclusion: Hepatoblastoma is a rare pediatric cancer. A very high relapse/refractory rate needs further investigation to determine the cause of failure of the treatment.

Downloads

Download data is not yet available.

References

Di Giuseppe G, Youlden DR, Aitken JF, Pole JD. Pediatric hepatic cancer incidence and survival: 30-year trends in Ontario, Canada; the United States; and Australia. Cancer 2021; 127(5): 769-776.

https://doi.org/10.1002/cncr.33319

Jeong SU, Kang HJ. Recent updates on the classification of hepatoblastoma according to the International Pediatric Liver Tumors Consensus. J Liver Cancer 2022; 22(1): 23-29.

https://doi.org/10.17998/jlc.2022.02.24

Luo H, Huang T, Lu Q, Zhang L, Xi Y, Yang Y, et al. Molecular prevalence of HBB-associated hemoglobinopathy among reproductive-age adults and the prenatal diagnosis in Jiangxi Province, southern central China. Front Genet 2022; 13: 992073.

https://doi.org/10.3389/fgene.2022.992073

Stiller CA, Pritchard J, Steliarova-Foucher E. Liver cancer in European children: incidence and survival, 1978-1997. Report from the Automated Childhood Cancer Information System project. Eur J Cancer 2006; 42(13): 2115-2123.

Moore SW, Millar AJ, Hadley GP, Ionescu G, Kruger M, Poole J, et al. Hepatocellular carcinoma and liver tumors in South African children: a case for increased prevalence. Cancer 2004; 101(3): 642-649. https://doi.org/10.1002/cncr.20398

Espinoza AF, Scheurer ME, Chambers TM, Vasudevan SA, Lupo PJ. A population-based assessment of metastatic hepatoblastoma in Texas reveals ethnic disparities. Front Public Health 2023; 11: 1049727.

https://doi.org/10.3389/fpubh.2023.1049727

Exelby PR, Filler RM, Grosfeld JL. Liver tumors in children in the particular reference to hepatoblastoma and hepatocellular carcinoma: American Academy of Pediatrics Surgical Section Survey--1974. J Pediatr Surg 1975; 10(3): 329-337.

https://doi.org/10.1016/0022-3468(75)90095-0

Brown J, Perilongo G, Shafford E, Keeling J, Pritchard J, Brock P, et al. Pretreatment prognostic factors for children with hepatoblastoma-- results from the International Society of Paediatric Oncology (SIOP) study SIOPEL 1. Eur J Cancer 2000; 36(11): 1418-1425.

https://doi.org/10.1016/s0959-8049(00)00074-5

Vinayak R, Cruz RJ Jr, Ranganathan S, Mohanka R, Mazariegos G, Soltys K, et al. Pediatric liver transplantation for hepatocellular cancer and rare liver malignancies: US multicenter and single-center experience (1981-2015). Liver Transpl 2017; 23(12): 1577-1588.

https://doi.org/10.1002/lt.24847

Abdelahamid S, Khedr RA, El Wakeel M, Younes A, Ahmed G, Elkinaai N, et al. Hepatoblastoma in Developing Countries; Eight Years of Single Centre Experience. J Cancer Ther 2018; 9: 793-806. https://doi.org/10.4236/jct.2018.910065

Benjamin RJ, Anderson KC. What is the proper threshold for platelet transfusion in patients with chemotherapy-induced thrombocytopenia? Crit Rev OncolHematol 2002; 42(2): 163-171. https://doi.org/10.1016/s1040-8428(01)00182-2

Pham TA, Gallo AM, Concepcion W, Esquivel CO, Bonham CA. Effect of Liver Transplant on Long-term Disease-Free Survival in Children With Hepatoblastoma and Hepatocellular Cancer. JAMA Surg. 2015; 150(12): 1150-1158.

https://doi.org/10.1001/jamasurg.2015.1847

Yuan XJ, Wang HM, Jiang H, Tang M, Li Z, Zou X, et al. Multidisciplinary effort in treating children with hepatoblastoma in China. Cancer Lett. 2016; 375(1): 39-46.

https://doi.org/10.1016/j.canlet.2016.02.051

Wang TY, Han YL, Gao YJ, Xu M, Gu S, Yin MZ, et al. Retrospective Analysis of Childhood Hepatoblastoma in a Single Centre in China. Clin Oncol (R Coll Radiol) 2019; 31(7) :471-478. https://doi.org/10.1016/j.clon.2019.03.044

Zhang Y, Zhang W, Tang S, Chen L, Yi Y, Zhang P, et al. A single-center retrospective study of pediatric hepatoblastoma. Oncol Lett 2016; 12(5): 3919-3925.

https://doi.org/10.3892/ol.2016.5195

Yu W, Liu X, Li J, Xi Z, Jin J, Huang H, et al. A single-center retrospective analysis of childhood hepatoblastoma in China. Gland Surg 2020; 9(5): 1502-1512.

https://doi.org/10.21037/gs-20-710

Vasudevan SA, Meyers RL, Finegold MJ, Dolores L, Ranganathan S, Dunn SP, et al. Outcomes of children with well-differentiated fetal hepatoblastoma treated with surgery only: Report from Children's Oncology Group Trial, AHEP0731. J Pediatr Surg 2022; 57(10): 251-256.

https://doi.org/10.1016/j.jpedsurg.2022.05.022

Murawski M, Weeda VB, Czauderna P. Surgical management in hepatoblastoma: points to take. PediatrSurg Int 2023; 39(1): 81. https://doi.org/10.1007/s00383-022-05356-z

Hishiki T. Current therapeutic strategies for childhood hepatic tumors: surgical and interventional treatments for hepatoblastoma. Int J Clin Oncol 2013; 18(6): 962-968.

https://doi.org/10.1007/s10147-013-0625-7

Rammohan A, Rela M, Kumar GV, Scott JX, Shanmugam N, Reddy MS, et al. Outcomes for high-risk hepatoblastoma in a resource-challenged setting. BJS Open 2020; 4(4): 630-636.

https://doi.org/10.1002/bjs5.50297

Leiskau C, Junge N, Mutschler FE. Long-Term Outcome Following Liver Transplantation for Primary Hepatic Tumors-A Single Centre Observational Study over 40 Years. Children (Basel) 2023; 10(2): 202.

https://doi.org/10.3390/children10020202