Hepatoblastomas are the most prevalent primary malignant liver tumor in children, with the majority of cases developing within the first two years of life. There are two basic categories of the histologic types: the mixed type and the epithelial type.
In the majority of patients, neoadjuvant chemotherapy has been the standard treatment for the past three decades.
The 70% cure rate produced by neoadjuvant chemotherapy plus surgical excision is a huge increase over the abysmal 30% cure rate of the 1970s. Multiple factors such as extent of excision, age during diagnosis, alpha-fetoprotein levels, and the stage of the disease affect the prognosis.
Epidemiology
Hepatoblastomas are so rare that they’re only responsible for 1% of pediatric tumors. Males are slightly more likely to be affected, and the incidence rate is on a slow rise in Europe and North America.
Anatomy
Pathophysiology
The exact pathophysiology of hepatoblastoma is not known.
Etiology
One-third of instances are linked to Down syndrome, Beckwith-Weidemann, Edward syndrome, nephroblastoma, or adenomatous polyposis. Infants with a low birth weight are at a greater risk of developing hepatoblastoma, and there is evidence of a link with preeclampsia and the smoking of the parent before and during pregnancy.
In addition to oxygen therapy, certain factors are suspected to have a role in the pathogenesis of this condition. The use of certain medications and plasticizers, as well as the adequacy of parental nutrition, are factors that are linked with hepatoblastoma risk.
These mutations are present in a greater number of sporadic instances and affect the Wnt signaling system, which leads to the buildup of beta-catenin. Immunohistochemistry typically reveals membranous staining of beta-catenin in more differentiated fetal types and nuclear staining in less distinguished histologic types. Human telomerase reverse transcriptase and MYC signaling are activated in aggressive instances.
Genetics
Prognostic Factors
Many factors contribute to the prognosis of hepatoblastoma.
Some of these are:
Degree of metastases
Stage of disease
Degree of resection
PRETEXT group
Alfa fetal protein levels
Histologic subtype of disease
Age
Historically, a younger age upon diagnosis has been associated with a worse prognosis; however, recent research has cast doubt on this notion, showing evidence that younger kids respond as well as older children.
Specifically, the prognosis is better for children younger than 1 and worse for those older than 6. It has been established that the presence of a tumor at the resection margin, multiple tumor sites, and metastases are poor prognostic indicators.
EpCAM expression has been associated with increased tumor viability and a worse response to neo-adjuvant chemotherapy, but beta-catenin expression has been linked to a shorter period of event-free surviva
per day administered as a continuous infusion for days in combination with cisplatin.
Regimen C for Stage I: 20 mg/m2 per day on days 1, 2, and 3 every 3 weeks for 4 cycles.
Future Trends
Media Gallary
References
https://www.ncbi.nlm.nih.gov/books/NBK534795/
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Hepatoblastomas are the most prevalent primary malignant liver tumor in children, with the majority of cases developing within the first two years of life. There are two basic categories of the histologic types: the mixed type and the epithelial type.
In the majority of patients, neoadjuvant chemotherapy has been the standard treatment for the past three decades.
The 70% cure rate produced by neoadjuvant chemotherapy plus surgical excision is a huge increase over the abysmal 30% cure rate of the 1970s. Multiple factors such as extent of excision, age during diagnosis, alpha-fetoprotein levels, and the stage of the disease affect the prognosis.
Hepatoblastomas are so rare that they’re only responsible for 1% of pediatric tumors. Males are slightly more likely to be affected, and the incidence rate is on a slow rise in Europe and North America.
The exact pathophysiology of hepatoblastoma is not known.
One-third of instances are linked to Down syndrome, Beckwith-Weidemann, Edward syndrome, nephroblastoma, or adenomatous polyposis. Infants with a low birth weight are at a greater risk of developing hepatoblastoma, and there is evidence of a link with preeclampsia and the smoking of the parent before and during pregnancy.
In addition to oxygen therapy, certain factors are suspected to have a role in the pathogenesis of this condition. The use of certain medications and plasticizers, as well as the adequacy of parental nutrition, are factors that are linked with hepatoblastoma risk.
These mutations are present in a greater number of sporadic instances and affect the Wnt signaling system, which leads to the buildup of beta-catenin. Immunohistochemistry typically reveals membranous staining of beta-catenin in more differentiated fetal types and nuclear staining in less distinguished histologic types. Human telomerase reverse transcriptase and MYC signaling are activated in aggressive instances.
Many factors contribute to the prognosis of hepatoblastoma.
Some of these are:
Degree of metastases
Stage of disease
Degree of resection
PRETEXT group
Alfa fetal protein levels
Histologic subtype of disease
Age
Historically, a younger age upon diagnosis has been associated with a worse prognosis; however, recent research has cast doubt on this notion, showing evidence that younger kids respond as well as older children.
Specifically, the prognosis is better for children younger than 1 and worse for those older than 6. It has been established that the presence of a tumor at the resection margin, multiple tumor sites, and metastases are poor prognostic indicators.
EpCAM expression has been associated with increased tumor viability and a worse response to neo-adjuvant chemotherapy, but beta-catenin expression has been linked to a shorter period of event-free surviva
per day administered as a continuous infusion for days in combination with cisplatin.
Regimen C for Stage I: 20 mg/m2 per day on days 1, 2, and 3 every 3 weeks for 4 cycles.
https://www.ncbi.nlm.nih.gov/books/NBK534795/
medtigo
Hepatoblastoma
Updated :
July 5, 2022
Hepatoblastomas are the most prevalent primary malignant liver tumor in children, with the majority of cases developing within the first two years of life. There are two basic categories of the histologic types: the mixed type and the epithelial type.
In the majority of patients, neoadjuvant chemotherapy has been the standard treatment for the past three decades.
The 70% cure rate produced by neoadjuvant chemotherapy plus surgical excision is a huge increase over the abysmal 30% cure rate of the 1970s. Multiple factors such as extent of excision, age during diagnosis, alpha-fetoprotein levels, and the stage of the disease affect the prognosis.
Hepatoblastomas are so rare that they’re only responsible for 1% of pediatric tumors. Males are slightly more likely to be affected, and the incidence rate is on a slow rise in Europe and North America.
The exact pathophysiology of hepatoblastoma is not known.
One-third of instances are linked to Down syndrome, Beckwith-Weidemann, Edward syndrome, nephroblastoma, or adenomatous polyposis. Infants with a low birth weight are at a greater risk of developing hepatoblastoma, and there is evidence of a link with preeclampsia and the smoking of the parent before and during pregnancy.
In addition to oxygen therapy, certain factors are suspected to have a role in the pathogenesis of this condition. The use of certain medications and plasticizers, as well as the adequacy of parental nutrition, are factors that are linked with hepatoblastoma risk.
These mutations are present in a greater number of sporadic instances and affect the Wnt signaling system, which leads to the buildup of beta-catenin. Immunohistochemistry typically reveals membranous staining of beta-catenin in more differentiated fetal types and nuclear staining in less distinguished histologic types. Human telomerase reverse transcriptase and MYC signaling are activated in aggressive instances.
Many factors contribute to the prognosis of hepatoblastoma.
Some of these are:
Degree of metastases
Stage of disease
Degree of resection
PRETEXT group
Alfa fetal protein levels
Histologic subtype of disease
Age
Historically, a younger age upon diagnosis has been associated with a worse prognosis; however, recent research has cast doubt on this notion, showing evidence that younger kids respond as well as older children.
Specifically, the prognosis is better for children younger than 1 and worse for those older than 6. It has been established that the presence of a tumor at the resection margin, multiple tumor sites, and metastases are poor prognostic indicators.
EpCAM expression has been associated with increased tumor viability and a worse response to neo-adjuvant chemotherapy, but beta-catenin expression has been linked to a shorter period of event-free surviva
https://www.ncbi.nlm.nih.gov/books/NBK534795/
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