HEPATOBLASTOMA

ALTERNATE NAMES

HB; HBL; Liver Cancer – Children; Pediatric Embryonal Hepatoma; Pediatric Hepatoblastoma

DESCRIPTION

Hepatoblastoma (HB) is a type of liver tumor that usually affects children younger than 3 years old. It is very rarely diagnosed in adolescence and is exceedingly rare in adults. If older children and adults have this disease, the prognosis tends to be worse than in younger children.

The cause of HB is generally unknown. Cirrhosis is not associated with this tumor. A common presentation in children is an asymptomatic abdominal mass.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-CM CODING

Diagnostic testing: The diagnosis of HB is established by:

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  Complete blood count (CBC) with differential;

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  Liver enzyme levels;

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  Abdominal radiography/ultrasonography;

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  Computed tomography (CT) scans/magnetic resonance imaging (MRIs);

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  Radionuclide bone scans;

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  Positron emission tomography (PET) scans;

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  Biopsies; and

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  Pathology reports.

Physical findings:

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  Swollen abdomen (belly) with a large mass;

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  Pain in the belly;

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  Pallor (pale skin);

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  Fatigue;

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  Decreased appetite;

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  Weight loss; and

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  Vomiting.

Other symptoms may include:

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  Fever;

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  Back pain; and

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  Decreased urination.

ICD-9: 155.0

ICD-10: C22.2

PROGRESSION

Staging of HB is based on the degree of surgical resection, histologic evaluation, and presence of metastatic disease. The most common sites for metastasis or spread of tumor are the lungs and abdominal lymph nodes.

TREATMENT

Treatment options include chemotherapy, radiotherapy, surgery to remove the tumor, and liver transplantation. When the tumor is determined to be non-resectable and there are no metastases, liver transplantation is the treatment of choice.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation:

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  Clinical history and examination that describes the diagnostic features of the impairment;

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  Pathology reports;

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  Imaging test results; and

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  Results of biopsies.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets

13.19

HB occurs primarily in children. HB meets listing 13.19 upon confirmed diagnosis, regardless of the effectiveness of treatment.

113.03

HB occurs primarily in children. HB meets the criteria in listing 113.03 upon confirmed diagnosis, regardless of the effectiveness of treatment.

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