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Case Forty - Hemangioendothelioma

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Clinical History: 3-month-old presenting with an abdominal mass, CHF and cutaneous hemangiomas.

Findings: Sonography shows a focal, well-circumscribed hepatic mass containing multiple sonolucent areas with central echogenicity.

Diagnosis: Hemangioendothelioma.

Discussion: Hemangioendothelioma is the most common benign tumor of the liver. Presentation is usually prior to 6 months and physical exam reveals an abdominal mass associated with high-output congestive heart failure. The heart failure is secondary to shunting within the tumor. Additionally, 50% of patients have cutaneous hemangiomas. Abnormal laboratory findings may include thrombocytopenia due to sequestration of platelets within the tumor.

Hemangioendothelioma sonographic appearance is variable and includes multiple sonolucent areas with central echogenicity due to enlarged vascular channels. Sonographic demonstration of large feeding vessels and a marked decrease in the caliber of the aorta below the celiac axis are consistent with hemangioendotheliomatosis. Sonography is valuable for following the response of the tumor.

References:
Kirks, Practical Pediatric Imag. Pg 809

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Submitted by:
Sheila C. Berlin, M.D.
Michelle Garnett, M.D.
Stuart Morrison, M.D.
Melissa Myers, M.D.
Carlos Sivit, M.D.
Dayna Weinert, M.D.
Rainbow Babies and Children's Hospital