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Congenital hepatic angiomyolipoma in a term neonate: Presentation of a rare case and review of the literature


K.P Akowundu
I.B Fajolu
B.N Ezenwa
B Kene-Udemezue
K Oleolo-Ayodeji
A.A Adeleke
G.C Ihediwa
V.C Ezeaka

Abstract

Angiomyolipoma (AML) of the liver is a rare benign, mesenchymal neoplasm that is composed of an admixture of blood vessels, smooth muscles, and variable adipose tissue. Hepatic AML poses a differential diagnostic challenge with other liver tumours. Awareness of the diverse morphological spectrum in liver biopsy is necessary to avoid misdiagnosis.


Hepatic AML has an extremely low risk of malignant transformation. Hepatic AML is usually under recognized by clinicians and is seldom included in differential diagnosis of focal liver lesions. This disease is rare in neonates. We report a rare case of congenital hepatic AML in a term male Nigerian neonate who until histology report was being managed for probable hepatoblastoma. To the best of our knowledge, this is the first report of hepatic angiomyolipoma in a neonate in Africa. This report is to create awareness for congenital hepatic AML and tendency for its misdiagnosis in neonates.


Keywords: Congenital; Hepatic angiomyolipoma; Neonate


Key Messages: Occurrence of hepatic angiomy olipoma is rare in the neonatal period and the diagnosis poses a challenge with other liver tumours. Histology is essential to confirming the diagnosis and providing appropriate treatment.


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eISSN: 0189-2657