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Hepatic "Hemangiomas": Contrasting Pediatric and Adult Lesions

Charles J Smithers1, Adam M. Vogel1, Harry P. Kozakewich1, Patricia E. Burrows1, Harriet J. Paltiel1, Urmila Khettry2, Heung B. Kim1,2, Roger L Jenkins2,1, John B. Mulliken1, Steven J. Fishman1
1Children's Hospital Boston, Boston, MA;2Lahey Clinic, Burlington, MA

Objective:
To compare and contrast clinical, radiologic, and histologic features of pediatric and adult liver “hemangiomas”.
Design:
Retrospective case series extracted by database search from 1998 to 2004.
Setting:
Two tertiary care teaching hospitals, one a referral center for vascular anomalies, the other for hepatobiliary disease.
Patients:
Ten patients (5 pediatric, 5 adult).
Interventions:
N/A.
Main Outcome Measures:
Clinical course, imaging, and histopathology.
Results:
Infants (3 male, 2 female) presented at age 2 weeks to 9 months with hepatomegaly or abnormal imaging. MRI showed uniform homogeneous T2 hyperintensity and centripetal enhancement, enlarged hepatic arteries and veins, and abnormal arteriovenous shunts. Histology showed a cellular mass of closely packed vascular channels with capillary morphology and plump, proliferative endothelium. Anti-angiogenic treatment with corticosteroid caused accelerated regression of lesions. Adults (1 male, 4 female) presented at age 33 to 53 years with abdominal pain or incidental radiologic diagnosis. Triphasic CT scan revealed centripetal, but delayed patchy enhancement, and normal hepatic arteries and veins. Histology revealed a spongy mass of large vascular spaces lined by flat endothelium, with intraluminal phleboliths and organizing thrombi. Treatment consisted of enucleation or resection.
Conclusions:
Adult and pediatric liver “hemangiomas” are distinct clinical, radiologic, and histopathologic entities. Infantile hepatic hemangiomas are vascular tumors, whereas adult hepatic “cavernous hemangiomas” are comparable to venous malformations that should not respond to anti-proliferative therapy. It is important to accurately classify these lesions to avoid confusion in diagnosis and treatment. In this context, adult liver “hemangiomas” may be more appropriately termed hepatic venous malformations.

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