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KMID : 0357920020360060425
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Korean Journal of Pathology
2002 Volume.36 No. 6 p.425 ~ p.428
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Mesenchymal Hamartoma of the Liver in Beckwith-Wiedemann Syndrome: A Case Report
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Yoo Seong-Ho
Park Hyo-Jin
Cho Soo-Yoen
Kim Jong-Jae
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Abstract
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Beckwith-Wiedemann syndrome is a well-known overgrowth syndrome associated with the presence of a wide variety of anomalies and increased risk of cancers. Less frequently, benign neoplasms also develop. We report a female infant with Beckwith-Wiedemann syndrome who developed a mesenchymal hamartoma of the liver. The patient was born with macroglossia, ear lobe crease, and abdominal distension. Laboratory data showed hypoglycemia, and magnetic resonance image revealed both adrenal enlargement, enhancing mass of the pancreas, and multiple hepatic nodules. The histologic findings of the resected distal pancreas and both adrenals were those of Beckwith-Wiedemann syndrome. Microscopic findings of the liver biopsy specimens were compatible with mesenchymal hamartoma. Hamartoma of the urinary bladder, cardiac fibrous hamartoma, and mixed hamartoma of the liver have been documented previously in association with Beckwith-Wiedemann syndrome. However, to our knowledge, this is the first case report of hepatic mesenchymal hamartoma in Beckwith-Wiedemann syndrome. Because of the paucity of hamartomas in childhood, we should be cautious of other features of Beckwith-Wiedemann syndrome and the present case extends the spectrum of tumor formation in this syndrome.
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KEYWORD
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Beckwith, Wiedemann Syndrome, Liver Neoplasms, Hamartoma
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