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. 2020 Aug;4(4):630-636.
doi: 10.1002/bjs5.50297. Epub 2020 May 7.

Outcomes for high-risk hepatoblastoma in a resource-challenged setting

A Rammohan  1 , M Rela  2 , G V Kumar  1 , J X Scott  1 , N Shanmugam  1 , M S Reddy  1 , P Ramachandran  1   2
Affiliations

Outcomes for high-risk hepatoblastoma in a resource-challenged setting

A Rammohan et al. BJS Open. 2020 Aug.

Abstract
in English, Spanish

Background: Outcomes of high-risk hepatoblastoma have been dismal, especially in resource-challenged countries where access to chemotherapy and paediatric liver transplantation is limited for the underprivileged. This study aimed to assess the results of treatment of high-risk hepatoblastoma in a tertiary centre, including patients who had non-transplant surgical procedures in the form of extended resection.

Methods: A review of patients with high-risk hepatoblastoma treated between January 2012 and May 2018 was carried out. Perioperative data and long-term outcomes were analysed.

Results: Of 52 children with hepatoblastoma, 22 were considered to have high-risk hepatoblastoma (8 girls and 14 boys). The mean(s.d.) age at diagnosis was 35(20) months. Of these 22 children, five died without surgery. Of the remaining 17 who underwent surgery, six had a resection (4 right and 2 left trisectionectomies) and 11 underwent living-donor liver transplantation. Median follow-up was 48 (range 12-90) months. Thirteen of the 17 children were alive at last follow-up and four developed disseminated disease (3 had undergone liver transplantation and 1 liver resection). The overall survival rate at 1, 3 and 5 years was 77, 64 and 62 per cent for the whole cohort with high-risk hepatoblastoma. In children who had surgery, 1-, 3- and 5-year survival rates were 91, 82 and 73 per cent for transplantation and 100, 83 and 83 per cent for resection. There was no difference in survival between the two surgical groups.

Conclusion: Excellent results in the treatment of high-risk hepatoblastoma are possible, even in resource-challenged countries.

Antecedentes: Los resultados del hepatoblastoma de alto riesgo (high risk hepatoblastoma, HRH) han sido pésimos, especialmente en países con recursos limitados, donde el acceso a la quimioterapia y al trasplante hepático pediátrico es limitado para los menos privilegiados. Este estudio tuvo como objetivo evaluar los resultados del HRH en un centro de tercer nivel, incluyendo a los pacientes que se sometieron a procedimientos quirúrgicos diferentes del trasplante en forma de resecciones extendidas. MÉTODOS: Se realizó una revisión de los pacientes con HRH tratados entre enero del 2012 y mayo de 2018. Se analizaron los datos perioperatorios y los resultados a largo plazo.

Resultados: De 52 niños con hepatoblastomas, 22 fueron considerados HRH (8 pacientes del sexo femenino/14 del sexo masculino). La edad media al diagnóstico fue de 35 ± 20 meses. De estos 22 pacientes, cinco fallecieron sin haber sido intervenidos quirúrgicamente. De los 17 restantes que se sometieron a cirugía, en seis se realizaron resecciones (4 trisectorectomías derechas, 2 trisectorectomías izquierdas) y 11 se sometieron a un trasplante de hígado de donante vivo. La mediana de seguimiento fue de 48 meses (12-90 meses). Trece de 17 niños estaban vivos en el último seguimiento, y cuatro habían desarrollado enfermedad diseminada (3 habían sido sometidos a trasplante hepático y 1 a resección hepática). La supervivencia global a 1, 3 y 5 años fue del 77,3%, 63,6% y 62% para toda la cohorte de HRH. Entre los que se sometieron a cirugía, las supervivencias a 1, 3 y 5 años fueron del 90,9%, 81,8% y 72,7% para el trasplante y del 100%, 83,3% y 83,3% para la resección. No hubo diferencia en la supervivencia entre los dos grupos sometidos a cirugía. CONCLUSIÓN: En países con recursos limitados es posible obtener excelentes resultados en el tratamiento de HRH.

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Figures

Figure 1
Figure 1
Vascular resection in high‐risk hepatoblastoma a High‐risk hepatoblastoma with pretreatment extent of disease (PRETEXT) III and vascular invasion after right trisectionectomy with resection of the main portal vein (MPV) and left portal vein. PII, resected end of segment II portal vein (PV); PIII, resected end of segment III PV. b Autologous internal jugular vein (IJV) graft used as venous conduit for anastomosis between PIII and MPV. c End‐to‐end anastomosis between MPV and PII; end‐to‐side anastomosis between PIII–IJV conduit and MPV. d High‐risk hepatoblastoma with PRETEXT IV V3 after right trisectionectomy with resection of a sleeve of inferior vena cava (IVC); closure of IVC with a prosthetic expanded polytetrafluoroethylene (ePTFE) onlay patch. D, diaphragm; L, liver.
Figure 2
Figure 2
Kaplan–Meier survival curves in patients with high‐risk hepatoblastoma a Overall survival in the whole cohort; b postoperative overall survival; c postoperative disease‐free survival. b P = 0·663, c P = 0·627 (log rank test).
See this image and copyright information in PMC

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References

    1. Aronson DC, Czauderna P, Maibach R, Perilongo G, Morland B. The treatment of hepatoblastoma: its evolution and the current status as per the SIOPEL trials. J Indian Assoc Pediatr Surg 2014; 19: 201–207. - PMC - PubMed
    1. Meyers RL, Maibach R, Hiyama E, Häberle B, Krailo M, Rangaswami A et al Risk‐stratified staging in paediatric hepatoblastoma: a unified analysis from the Children's Hepatic tumors International Collaboration. Lancet Oncol 2017; 18: 122–131. - PMC - PubMed
    1. Manuprasad A, Radhakrishnan V, Sunil BJ, Ramakrishnan AS, Ganesan TS, Ganesan P et al Hepatoblastoma: 16‐years' experience from a tertiary cancer centre in India. Pediatr Hematol Oncol J 2018; 3: 13–16.
    1. Nagral S, Nanavati A, Nagral A. Liver transplantation in India: at the crossroads. J Clin Exp Hepatol 2015; 5: 329–340. - PMC - PubMed
    1. Shanmugam N, Scott JX, Kumar V, Vij M, Ramachandran P, Narasimhan G et al Multidisciplinary management of hepatoblastoma in children: experience from a developing country. Pediatr Blood Cancer 2017; 64: e26249. - PubMed