fbpx

Hepatoblastoma

Updated : February 28, 2024





Background

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

Clinical History

Physical Examination

Age group

Associated comorbidity

Associated activity

Acuity of presentation

Differential Diagnoses

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

Medication

 
 

doxorubicin

Regimen B for Stage I, II, III, IV:

20

mg/m^2

Intravenous (IV)

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.



 

Media Gallary

References

https://www.ncbi.nlm.nih.gov/books/NBK534795/

Hepatoblastoma

Updated : February 28, 2024




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

doxorubicin

Regimen B for Stage I, II, III, IV:

20

mg/m^2

Intravenous (IV)

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/

Free CME credits

Both our subscription plans include Free CME/CPD AMA PRA Category 1 credits.

Digital Certificate PDF

On course completion, you will receive a full-sized presentation quality digital certificate.

medtigo Simulation

A dynamic medical simulation platform designed to train healthcare professionals and students to effectively run code situations through an immersive hands-on experience in a live, interactive 3D environment.