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Clinical Profile of Hepatoblastoma: Experience From a Tertiary Care Centre in a Resource-Limited Setting

Background Hepatoblastoma (HB) is a rare neoplasm of the liver, accounting for about 1% of all pediatric cancers. The aim of the present study is to report our experience with HBs over a period of five years from a tertiary center in Eastern India. Methodology This is a retrospective observational s...

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Autores principales: Dhali, Arkadeep, Mandal, Tuhin S, Das, Somak, Ray, Gautam, Halder, Prasenjit, Bose, Debarshi, Pal, Suparna K, Ray, Sukanta, Chowdhury, Abhijit, Dhali, Gopal Krishna
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9339225/
https://www.ncbi.nlm.nih.gov/pubmed/35919215
http://dx.doi.org/10.7759/cureus.26494
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author Dhali, Arkadeep
Mandal, Tuhin S
Das, Somak
Ray, Gautam
Halder, Prasenjit
Bose, Debarshi
Pal, Suparna K
Ray, Sukanta
Chowdhury, Abhijit
Dhali, Gopal Krishna
author_facet Dhali, Arkadeep
Mandal, Tuhin S
Das, Somak
Ray, Gautam
Halder, Prasenjit
Bose, Debarshi
Pal, Suparna K
Ray, Sukanta
Chowdhury, Abhijit
Dhali, Gopal Krishna
author_sort Dhali, Arkadeep
collection PubMed
description Background Hepatoblastoma (HB) is a rare neoplasm of the liver, accounting for about 1% of all pediatric cancers. The aim of the present study is to report our experience with HBs over a period of five years from a tertiary center in Eastern India. Methodology This is a retrospective observational study. The data of all patients who were diagnosed with HB between August 2015 and December 2020 was reviewed. Results Twenty-three patients who were diagnosed and treated for HB at our center were included in the study. Sixteen (69.5%) of them were male. The median age of presentation was 14 (range, 3-58) months. An abdominal lump (n=23, 100%) and abdominal pain (n=11, 47.8%) were the most common presenting symptoms. The median level of serum alpha-fetoprotein at the time of initial evaluation was 8000 (878-1,280,000) ng/dL. The mean size of the largest focus in its largest dimension was 12.03±3.77 cm. The epithelial variant (n=22, 95.7%) was the most common histological subtype. One (4.3%), 10 (43.4%), 11 (47.8%), and one (4.3%) patient were found to have pre-treatment extent of tumor (PRETEXT) stages 1, 2, 3, and 4, respectively. Fifteen (65.2%) children were classified as standard risk and seven (34.7%) children as high risk. All the patients received neoadjuvant chemotherapy (NACT). The most commonly performed surgery was right hepatectomy (n=12, 52.1%). There were three (13%) cases of perioperative mortality. Four postoperative complications developed in three (13%) patients. Four (17.3%) patients developed chemotherapy-related complications. The median duration of follow-up was 31 (range, 0-58) months. Three (13%) patients developed relapses of the disease. Overall, five-year survival in our series was 73.9%. Conclusion This study shows that the overall outcomes of HB in a resource-limited setting such as ours are good with the adoption of multi-modality treatment. Managing chemotherapy-induced complications and making liver transplantation more feasible will improve the results further.
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spelling pubmed-93392252022-08-01 Clinical Profile of Hepatoblastoma: Experience From a Tertiary Care Centre in a Resource-Limited Setting Dhali, Arkadeep Mandal, Tuhin S Das, Somak Ray, Gautam Halder, Prasenjit Bose, Debarshi Pal, Suparna K Ray, Sukanta Chowdhury, Abhijit Dhali, Gopal Krishna Cureus General Surgery Background Hepatoblastoma (HB) is a rare neoplasm of the liver, accounting for about 1% of all pediatric cancers. The aim of the present study is to report our experience with HBs over a period of five years from a tertiary center in Eastern India. Methodology This is a retrospective observational study. The data of all patients who were diagnosed with HB between August 2015 and December 2020 was reviewed. Results Twenty-three patients who were diagnosed and treated for HB at our center were included in the study. Sixteen (69.5%) of them were male. The median age of presentation was 14 (range, 3-58) months. An abdominal lump (n=23, 100%) and abdominal pain (n=11, 47.8%) were the most common presenting symptoms. The median level of serum alpha-fetoprotein at the time of initial evaluation was 8000 (878-1,280,000) ng/dL. The mean size of the largest focus in its largest dimension was 12.03±3.77 cm. The epithelial variant (n=22, 95.7%) was the most common histological subtype. One (4.3%), 10 (43.4%), 11 (47.8%), and one (4.3%) patient were found to have pre-treatment extent of tumor (PRETEXT) stages 1, 2, 3, and 4, respectively. Fifteen (65.2%) children were classified as standard risk and seven (34.7%) children as high risk. All the patients received neoadjuvant chemotherapy (NACT). The most commonly performed surgery was right hepatectomy (n=12, 52.1%). There were three (13%) cases of perioperative mortality. Four postoperative complications developed in three (13%) patients. Four (17.3%) patients developed chemotherapy-related complications. The median duration of follow-up was 31 (range, 0-58) months. Three (13%) patients developed relapses of the disease. Overall, five-year survival in our series was 73.9%. Conclusion This study shows that the overall outcomes of HB in a resource-limited setting such as ours are good with the adoption of multi-modality treatment. Managing chemotherapy-induced complications and making liver transplantation more feasible will improve the results further. Cureus 2022-07-01 /pmc/articles/PMC9339225/ /pubmed/35919215 http://dx.doi.org/10.7759/cureus.26494 Text en Copyright © 2022, Dhali et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle General Surgery
Dhali, Arkadeep
Mandal, Tuhin S
Das, Somak
Ray, Gautam
Halder, Prasenjit
Bose, Debarshi
Pal, Suparna K
Ray, Sukanta
Chowdhury, Abhijit
Dhali, Gopal Krishna
Clinical Profile of Hepatoblastoma: Experience From a Tertiary Care Centre in a Resource-Limited Setting
title Clinical Profile of Hepatoblastoma: Experience From a Tertiary Care Centre in a Resource-Limited Setting
title_full Clinical Profile of Hepatoblastoma: Experience From a Tertiary Care Centre in a Resource-Limited Setting
title_fullStr Clinical Profile of Hepatoblastoma: Experience From a Tertiary Care Centre in a Resource-Limited Setting
title_full_unstemmed Clinical Profile of Hepatoblastoma: Experience From a Tertiary Care Centre in a Resource-Limited Setting
title_short Clinical Profile of Hepatoblastoma: Experience From a Tertiary Care Centre in a Resource-Limited Setting
title_sort clinical profile of hepatoblastoma: experience from a tertiary care centre in a resource-limited setting
topic General Surgery
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9339225/
https://www.ncbi.nlm.nih.gov/pubmed/35919215
http://dx.doi.org/10.7759/cureus.26494
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