mesenchymal component

and tumor giant cells

dense fibrous stroma

From Pizzo PA, Poplack DG, editors. Principles and Practice of Pediatric Oncology. 4th ed. Philadelphia: JB Lippincott, 2002, with permission.

From Pizzo PA, Poplack DG, editors. Principles and Practice of Pediatric Oncology. 4th ed. Philadelphia: JB Lippincott, 2002, with permission.

Table 24-15. Frequency of Signs and Symptoms in Children with Hepatoblastoma and Hepatocarcinoma

Sign/symptom Hepatoblastoma (%) Hepatocarcinoma (%)

Abdominal mass 80 60

Abdominal distention 27 34

Anorexia 20 20

Weight loss 19 19

Abdominal pain 15 21

Vomiting 10 10

Pallor 7 Rare

Jaundice 5 10

Fever 4 8

Diarrhea 2 Rare

Constipation 1 Rare

Pseudoprecocious puberty Occasional Not reported

All patients should have the following evaluations:

• Physical examination

• Complete blood count (anemia with moderate leukocytosis is commonly seen; thrombocytosis with platelet counts greater than 500,000/mm3 is the most frequent abnormality)

• Urinalysis

• Liver profile and electrolytes

• Fibrinogen, partial thromboplastin time (PTT), and prothrombin time (PT)

Hepatitis B surface antigen (HBsAg), core antigen (HBcAg), and core antibody

• Carcinoembryonic antigen (CEA)

• Radiographic evaluation of intrahepatic disease: sonogram, abdominal CT, magnetic resonance imaging (MRI)

• Radiographic evaluation of extrahepatic disease: chest radiographs (PA and lateral), chest CT, bone scan

• Bone marrow aspirate/biopsy.

Approximately 90% of children with hepatoblastomas and 50% of children with hepatocarcinoma will have elevated AFP. AFP is a valuable marker for monitoring residual or metastatic disease following resection of the primary tumor or for monitoring the response of an unresectable primary tumor to therapy. AFP is normally elevated in the newborn period and then declines (Table 24-4). Concentrations greater than 500,000 ng/mL of AFP are not unusual in hepatoblastoma. Failure of AFP to return to normal after surgery is an indication of incomplete tumor removal or metastases. Serial determinations of AFP are the most precise measurement of the effectiveness of hepatoblastoma therapy.

P-hCG is measured for the occasional patient with hepatoma who has associated precocious puberty. Unlike AFP, P-hCG levels do not necessarily reflect the clinical course of the tumor.

MRI angiography and MRI cholangiogram are useful complements to CT for evaluation of intrahepatic disease because they aid in determining surgical resectability. In hepatoblastoma, the right lobe of the liver is more commonly involved than the left, but the tumor involves both lobes in 30% of patients.

Approximately 10% of patients with hepatoma will have demonstrable pulmonary metastases on plain radiographs. The widespread use of chest CT has increased the number of patients detected with lung metastases.

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