HEPATOBLASTOMA

ALTERNATE NAMES

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

DESCRIPTION

Hepatoblastoma (HB) is a type of liver tumor that usually affects children younger than 3 years. 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 hepatoblastoma (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 CODING

Diagnostic testing: The diagnosis of HB is established by:

• CBC count with differential; liver enzyme levels.

• Abdominal radiography/ultrasonography; CT scans/MRIs; radionuclide bone scans; positron emission tomography (PET) scans; biopsies and pathology reports.

Physical findings: The most common symptoms of HB is a swollen abdomen (belly) with a large mass, pain in the abdomen (belly), pallor (pale skin), fatigue, decreased appetite, weight loss, and vomiting. Other symptoms may include fever, back pain, and decreased urination.

ICD-9: 155.0

ONSET AND 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: The adjudicator needs medical evidence from treating sources and hospitals. It may include clinical history and examination that describes the diagnostic features of the impairment, pathology reports, imaging test results, and biopsies.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets Listing

13.19

113.03

Hepatoblastoma occurs primarily in children. Hepatoblastoma meets listing 113.03 or 13.19 upon confirmed diagnosis, regardless of the effectiveness of treatment.

Medical Equals