Hepatoblastoma

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Hepatoblastoma Microchapters
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Patient Information
Overview
Historical Perspective
Classification
Pathophysiology
Causes
Epidemiology and Demographics
Risk Factors
Screening
Differentiating Hepatoblastoma from other Diseases
Natural History, Complications and Prognosis
Diagnosis
Staging
History and Symptoms
Physical Examination
Laboratory Findings
X Ray
CT
MRI
Ultrasound
Other Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Surgery
Primary Prevention
Secondary Prevention
Cost-Effectiveness of Therapy
Future or Investigational Therapies
Case Studies
Case #1
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American Roentgen Ray Society Images of Hepatoblastoma All Images X-rays Echo & Ultrasound CT Images MRI
Ongoing Trials at Clinical Trials.gov
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Hepatoblastoma in the news
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Directions to Hospitals Treating Hepatoblastoma
Risk calculators and risk factors for Hepatoblastoma

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Nima Nasiri, M.D.[2] Nawal Muazam M.D.[3]

Synonyms and keywords: Epithelial hepatoblastoma; Fetal hepatoblastoma; Embryonal hepatoblatoma; Macrotrabecular hepatoblastoma; Mixed hepatoblastoma; Rhabdoid hepatoblastoma; Small cell hepatoblastoma; Undifferentiated hepatoblastoma; Cholangioblastic hepatoblastoma; Teratoid hepatoblastoma; Chondroid hepatoblastoma; Osteoid hepatoblastoma; Rhabdomyoblastic hepatoblastoma; Neuroid-melanocytic hepatoblastoma; Well differentiated hepatoblastoma; HB

Overview

Hepatoblastoma is the most common primary liver tumor in young children, usually less than 3 years old, accounting for over 1% of pediatric cancers. The etiology is unknown, but it has been associated with Beckwith-Weidemann syndrome, familial adenomatosis polypi, and low birth weight. The primary treatment is surgical resection, however, chemotherapy plays an important role by increasing the number of tumors that are respectable. The prognosis for patients with resectable tumors is fairly good, however, the outcome for those with nonresectable or recurrent disease is poor.

PMID: 11110595

Historical Perspective

• In 1898, the first case of a child with HB was published in the English literature. A 6-week-old boy was described whose autopsy showed a large tumor that occupied the lower half of the right liver lobe.

Classification

• Hepatoblastoma are divided into 2 broad categories:
  • Epithelial type (E-HB)
    • Fetal, which has four subtypes:
      • Well differentiated
      • Crowded or mitotically active
      • Pleomorphic
      • Poorly differentiated
      • Anaplastic
    • Embryonal
    • Macrotubular small cell undifferentiated (SCU)
    • Cholangioblastic
  • Mixed epithelial and mesenchymal type (MEM-HB). while the mixed type is subdivided into
    • Stromal derivatives
    • Teratoid variants

Pathophysiology

• The exact pathogenesis of hepatoblastoma is not fully understood.
• Loss of function mutations in APC leads to intracellular accumulation of the protooncogene b-catenin, an effector of Wnt signal transduction.
• Hepatoblastomas originate from primitive hepatic stem cells.
• B-catenin mutations have been shown to be common in the majority of sporadic hepatoblastomas.
• Studies revealed that tumor occurs more often in families affected by Familial adenomatous polyposis(FAP), which is caused by inactivation of the adenomatous polyposis coli(APC), a tumor-suppressor gene that down-regulate the amount of b-catenin.

Hepatoblastoma H&Esource:wikipedia

Causes

• Underlying causes of hepatoblastoma poorly understood and most tumors are sporadic but there are some risk factors and conditions that have a strong association with this tumor such as:
  • Beckwith-Weidemann syndrome
  • Familial adenomatous polyposis (FAP)
  • Down syndrome
  • Edward syndrome (trisomy 18)
  • Nephroblastoma,
  • Low birth weight infants are at higher risk of developing a hepatoblastoma
  • Preeclampsia
  • Parental tobacco smoking
  • Oxygen therapy
  • Certain medication (furosemide)
  • Radiation
  • Total parenteral nutrition (TPN)

• The most common genetic mutation which plays role in etiology of sporadic cases include:
  • The Wnt signaling pathway which results in the accumulation of beta-catenin

Differentiating hepatoblastoma from Other Diseases

• Hepatoblastoma must be differentiated from other diseases that cause rapidly enlarging abdominal mass in pediatrics such as:
  • Hepatocellular carcinoma (HCC),
  • Focal nodular hyperplasia
  • Hepatic adenoma
  • Lymphoma, and metastases

Epidemiology and Demographics

• The incidence/prevalence of hepatoblastoma is approximately 0.05–0.15 patients per 100 000 population in children younger than 15 years.
• Peak incidence means of 18 months, mostly in infants and children younger than 3 years old, with a male predilection.
• Hepatoblastoma is the most common primary liver cancer in infants and children, accounts for one percent of all primary malignancies in pediatrics.

Risk Factors

• Common risk factors in the development of hepatoblastoma include:
  • Low birth weight infants
  • Preeclampsia
  • Fetal distress
  • Premature labor
  • Chromosomal anomalies such as Down syndrome, Edward syndrome.
  • Parental tobacco smoking before and during pregnancy
  • Oxygen therapy,
  • Certain medication (furosemide)
  • Total parenteral nutrition (TPN)

Screening

• Ultrasound is currently the main screening tool for suspected liver pathology and is ideally suited for evaluation of hepatic lesions in children due to their generally small size.

Natural History, Complications, and Prognosis

• Prognosis is based on different factors including:
  • Age of diagnosis, younger children have a better prognosis.
  • Metastases,
  • Alfa-fetoprotein (AFP) levels, drop in AFP level after chemotherapy means better response to treatment.
  • Histologic subtype, the well-differentiated fetal subtype has a better prognosis compared with small cell undifferentiated ones.
• Complications of hepatoblastoma includes:
  • Pancytopenia
  • Anemia
  • Intraperitoneal tumor rupture
  • Complications related to chemotherapy such as renal failure
  • Post-transplant complications such as hepatic ductal obstruction, thrombosis.
  • Psychosocial effects of treatment and painful procedures

Diagnosis

Diagnostic Study of Choice

The diagnosis of hepatoblastoma is made when at least [number] of the following [number] diagnostic criteria are met: [criterion 1], [criterion 2], [criterion 3], and [criterion 4].

Staging

PRETEXT staging score :

History and Symptoms

• The majority of patients with hepatoblastoma have an abdominal mass or abdominal distension.
• Other symptoms of hepatoblastoma include:
  • Abdominal discomfort,
  • Generalized fatigue,
  • Loss of appetite, due to tumor distension
  • Anemia.
• Ruptured tumor can cause symptoms of peritoneal irritation such as nausea, vomiting, and severe anemia.

Physical Examination

• Physical examination of patients with hepatoblastoma is usually remarkable for single, mildly painful, rapidly enlarging abdominal mass that is found in the right lobe of the liver.
• Most tumors are solitary; but can be multifocal as well.

Laboratory Findings

Laboratory findings consistent with the diagnosis of hepatoblastoma include:

Electrocardiogram

• There are no ECG findings associated with hepatoblastoma.

X-ray

• Chest X-rays can be useful especially since this tumor has the affinity to metastasize to lungs.

Echocardiography or Ultrasound

• Imaging studies play an important role in the diagnosis, staging, and treatment of disease, most tumors can be resected surgically and ultrasound is often used in order to detect tumor size, also the initial diagnosis is made by abdominal ultrasound.

CT scan

CT scan is helpful to diagnose the disease, children with hepatoblastoma undergo either a CT or MRI scan at diagnosis.

Surgeons prefer an angiographic or biphasic CT scan because of better depiction of the hepatic arterial, portal venous and hepatic vein and other liver structures.

Concerns about radiation exposures in pediatrics has changed this modality in favor of MRI, although MRI is much lengthy exam than CT. has the advantage of multiplanar soft-tissue characterization, and when diffusion-weighted imaging techniques are used, MRI is exquisitely sensitive for detecting tiny liver lesions.

There are no CT scan findings associated with [disease name]. However, a CT scan may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

MRI

MRI has the advantage of multiplanar soft-tissue characterization, and when diffusion-weighted imaging techniques are used, MRI is exquisitely sensitive for detecting tiny liver lesions.

OR

[Location] MRI may be helpful in the diagnosis of [disease name]. Findings on MRI suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no MRI findings associated with [disease name]. However, a MRI may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

Other Imaging Findings

There are no other imaging findings associated with [disease name].

OR

[Imaging modality] may be helpful in the diagnosis of [disease name]. Findings on an [imaging modality] suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

Other Diagnostic Studies

There are no other diagnostic studies associated with [disease name].

OR

[Diagnostic study] may be helpful in the diagnosis of [disease name]. Findings suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

Other diagnostic studies for [disease name] include [diagnostic study 1], which demonstrates [finding 1], [finding 2], and [finding 3], and [diagnostic study 2], which demonstrates [finding 1], [finding 2], and [finding 3].

Treatment

Medical Therapy

• The mainstay of therapy for hepatoblastoma is surgery, however, the vast majority of the tumors cannot be completely resected because of their large size or metastasis.
• Liver transplantation can be considered for tumors that cannot be removed by surgery.
• Chemotherapy is an important adjuvant therapy, and cisplatin is the most commonly used chemotherapeutic agent, it can reduce the volume of tumors that are too big for surgical removal.

Surgery

• Surgery is the mainstay of treatment for hepatoblastoma.
• The feasibility of surgery depends on the resectability of tumor at diagnosis.

Primary Prevention

There are no established measures for the primary prevention of hepatoblastoma.

Secondary Prevention

There are no established measures for the secondary prevention of [disease name].

OR

Effective measures for the secondary prevention of [disease name] include [strategy 1], [strategy 2], and [strategy 3].