CASE 68

Clinical Presentation

A 1-month-old infant presents with tachypnea associated with decreased oxygen saturation.

Figure 68A

Figure 68B

Figure 68C

Radiologic Findings

An AP chest radiograph demonstrates cardiac enlargement (Fig. 68A). A longitudinal ultrasonogram shows a smaller caliber of the aorta below the celiac level (Fig. 68B). On transverse view, the liver is occupied by multiple round hypoechoic areas (Fig. 68C).

Figure 68D Differential diagnosis: hemangioma with a focal right lobe mass with central calcification.

Figure 68E Differential diagnosis: The CT image shows a typical enhancement pattern of hemangioma at early scanning.

Diagnosis

Infantile hemangioendothelioma of the liver

Differential Diagnosis

• Hemangioma (Figs. 68D and 68E)
  
• Arteriovenous malformation of the liver (Fig. 68F)
  
• Rhabdomyosarcoma of the biliary tree
  
• Metastatic disease, especially neuroblastoma

Discussion

Background

A rare entity, hemangioendothelioma is the most common benign hepatic tumor and is seen primarily in young infants (95% of cases <1 year of age). This vascular lesion acts as an arteriovenous fistula. It is twice as common in girls. Fifty percent of patients have associated skin hemangiomas.

Clinical Findings

Most affected infants present with congestive heart failure due to high cardiac output during the first 6 months of life. Asymptomatic hepatomegaly, jaundice, anemia, and hemoperitoneum (due to rupture) occur less commonly. A loud systolic bruit may be heard over the liver. Severe arteriovenous shunting may induce fetal hydrops. Serum a-fetoprotein level is typically mildly elevated. Spontaneous involution is observed in small lesions.

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