Mesenchymal Hamartoma of the Liver in an Infant With Beckwith-Wiedemann Syndrome: A Rare Condition Mimicking Hepatoblastoma

Patients with Beckwith-Wiedemann syndrome (BWS) are known to be at an increased risk for childhood malignancies, particularly Wilms tumor and hepatoblastoma. We report a case of genetically confirmed BWS in a 5-month-old girl who presented with a 9.5-cm abdominal mass associated with elevated α-feto...

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Detalles Bibliográficos
Autores principales: Abrahao-Machado, Lucas F., de Macedo, Fabiane C., Dalence, Carlos, Stambo, Glenn, Abrahao-Machado, Eduarda F., Abrahao-Machado, Elaine C.F., Bahrami, Armita, Nascimento, Antonio G.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: American College of Gastroenterology 2015
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4508960/
https://www.ncbi.nlm.nih.gov/pubmed/26203458
http://dx.doi.org/10.14309/crj.2015.78
Descripción
Sumario:Patients with Beckwith-Wiedemann syndrome (BWS) are known to be at an increased risk for childhood malignancies, particularly Wilms tumor and hepatoblastoma. We report a case of genetically confirmed BWS in a 5-month-old girl who presented with a 9.5-cm abdominal mass associated with elevated α-fetoprotein levels. The clinical impression was strongly suggestive of hepatoblastoma. Histologic examination of the surgically excised mass revealed mesenchymal hamartoma of the liver (MHL), a benign hepatic neoplasm.

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