Hepatoblastoma overview

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

Overview

Hepatoblastoma is the most common primary liver tumor occurs in infant and children, usually less than 3 years old, more frequently in male, and accounting for over 1% of pediatric cancers. The etiology is unknown and not well understood, but it has been associated with Beckwith-Weidemann syndrome, familial adenomatous polyposis, and other factors such as low birth weight, preeclampsia, hemihypertrophy. The primary treatment is surgical resection, however, chemotherapy plays an important role by increasing the number of tumors that are respectable, chemotherapeutic agents helps in shrinkage of the tumor and make it easier to be resected by surgery. Prognosis of the tumor depends on various criteria such as resectability of the tumor, distant metastasis, tumor size, PRETEXT staging, and recurrence of the tumor. The mainstay of treatment is surgery, but the adjuvant chemotherapeutic agents and liver transplantation also are helpful.

Historical Perspective

• In 1898, the first case of hepatoblastoma was published in English literature, the tumor was diagnosed in a 6-week old boy in Prague, by a physician named Misick, who found a large tumor on the autopsy of his liver. Decades later on 1962 Willis used the term, hepatoblastoma for this type of liver tumor because of it's embryonal origin

Classification

• Hepatoblastoma are divided into two 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). 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 beta-catenin, which leads to germline mutation of Wnt signal transduction and pathway.
• 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 beta-catenin.
• Immunohistochemical markers such as expression of CK19, beta-catenin and EpCAM were correlated with tumor behaviour, response to chemotherapy and survival.

Hepatoblastoma H&Esource:wikipedia

Causes

There are no established causes for hepatoblastoma.

Epidemiology and Demographics

Hepatoblastoma is a common tumor that tends to affect children aged less than five years. Males are more commonly affected with hepatoblastoma than females. The annual incidence of hepatoblastoma in the United States appears to have doubled from 8 (1975-1983) to 16 (2002-2009) per 100,000 children aged 19 years and younger.

Risk factors

To view a comprehensive list of risk factors that increase the risk of hepatoblastoma, click here.

Screening

According to the American Association for the Study of Liver Diseases and United States Preventive Services Task Force, there is insufficient evidence to recommend routine screening for hepatoblastoma in the general population. Effective measures for the secondary prevention of hepatoblastoma include use of abdominal ultrasound and alpha-fetoprotein levels in patients with Beckwith-Wiedemann syndrome or isolated hemihyperplasia.

Differentiating Hepatoblastoma from other diseases

Hepatoblastoma must be differentiated from other diseases such as hepatic mesenchymal hamartoma, hepatocellular carcinoma, hepatic metastases, infantile hemangioendothelioma, and rhabdomyosarcoma of biliary tract.

Natural History, Complications and Prognosis

If left untreated, patients with hepatoblastoma may progress to develop failure to thrive, weight loss, spontaneous rupture, hemorrhage, and death. Common complications of hepatoblastoma include paraneoplastic features, erythrocytosis, thrombocytosis, hypocalcemia, isosexual precocious puberty, hypoglycemia. The 5-year survival rate of children with hepatoblastoma is approximately 70%.

Diagnosis

Staging

The staging of hepatoblastoma is based on the Intergroup staging system.

History and Symptoms

Symptoms of hepatoblastoma include an abdominal mass (may be painless or painful), abdominal pain, weight loss, loss of appetite, early puberty in boys, jaundice, nausea, vomiting, back pain, and failure to thrive.

Physical examination

Common physical examination findings of hepatoblastoma include hepatomegaly, abdominal distension, asymptomatic palpable abdominal mass, hemihypertrophy, jaundice, pyrexia, and anemia.

Laboratory Findings

Laboratory tests for liver function are usually normal. An elevated concentration of alpha-fetoprotein is present in patients with hepatoblastoma.

Xray

Findings on abdominal xray are nonspecific for hepatoblastoma and include right upper quadrant abdominal mass and/or calcifications in 10% of cases.

CT

Abdominal CT scan may be helpful in the diagnosis of hepatoblastoma. Findings on CT scan suggestive of hepatoblastoma include well defined heterogeneous mass, intralesional areas of necrosis and hemorrhage, and dense calcifications.

MRI

Abdominal MRI is helpful in the diagnosis of hepatoblastoma. On MRI, hepatoblastoma is characterized by hypointensity on T1-weighted imaging and hyperintensity compared to liver with areas of necrosis and hemorrhage on T2-weighted imaging.

Ultrasound

Ultrasound may be helpful in the diagnosis of hepatoblastoma. Findings on ultrasound suggestive of hepatoblastoma include echogenic soft tissue mass, well defined lesion, and areas of shadowing due to intralesional calcifications.

Biopsy

Biopsy is the gold standard for the diagnosis of hepatoblastoma. Histopathological findings on biopsy can be found here.

Treatment

Medical Therapy

The predominant therapy for hepatoblastoma is surgical resection. Neoadjuvant and adjuvant chemotherapy may be required.

Surgery

Surgery is the mainstay of treatment for hepatoblastoma.

Primary Prevention

There are no primary preventive measures available for hepatoblastoma.

Secondary Prevention

Effective measures for the secondary prevention of hepatoblastoma include use of abdominal ultrasound and alpha-fetoprotein levels in patients with Beckwith-Wiedemann syndrome or isolated hemihyperplasia.

References

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