Multidisciplinary management of hepatoblastoma in children: Experience from a developing country

Pediatr Blood Cancer. 2017 Mar;64(3). doi: 10.1002/pbc.26249. Epub 2016 Oct 26.

Abstract

Background: Advances in chemotherapy, liver resection techniques, and pediatric liver transplantation have vastly improved survival in children with hepatoblastoma (HB). These are best managed by a multidisciplinary team (MDT) in a setting where all treatment options are available. Until recently, this was difficult to achieve in India.

Methods: All children (<16 years) with HB treated in a pediatric liver surgery and transplantation unit between January 2011 and July 2016 were reviewed. Data regarding the clinical presentation, preoperative management, surgical treatment, postoperative course, and outcomes were extracted from a prospectively managed database.

Results: Thirty children were treated for HB during the study period. Nine children were PRETEXT 4, 7 were PRETEXT 3, 13 were PRETEXT 2, and 1 was PRETEXT 1 (where PRETEXT is pretreatment extension). All children received a neoadjuvant chemotherapy before surgery followed by an adjuvant chemotherapy. Nineteen children had complete resection, while six underwent primary living donor liver transplantation. There were six mortalities including five children who poorly responded to chemotherapy with progressive tumor extension. At a median follow-up of 30 months, two children who underwent resection and one child who underwent liver transplant had disease recurrence.

Conclusion: Improved outcomes can be achieved in children with HB even in countries with limited resources when they are managed by MDTs with expertise in pediatric oncology, liver resection, and liver transplantation.

Keywords: SIOPEL; chemotherapy; hepatoblastoma; liver transplantation; multidisciplinary.

Publication types

  • Observational Study

MeSH terms

  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use*
  • Chemotherapy, Adjuvant
  • Child
  • Child, Preschool
  • Combined Modality Therapy
  • Developing Countries
  • Female
  • Follow-Up Studies
  • Hepatectomy*
  • Hepatoblastoma / pathology
  • Hepatoblastoma / therapy*
  • Humans
  • India
  • Infant
  • Liver Neoplasms / pathology
  • Liver Neoplasms / therapy*
  • Liver Transplantation*
  • Living Donors
  • Male
  • Neoadjuvant Therapy*
  • Neoplasm Recurrence, Local / pathology
  • Neoplasm Recurrence, Local / therapy*
  • Neoplasm Staging
  • Prognosis
  • Prospective Studies
  • Survival Rate