Hepatoblastoma in an infant with Beckwith-Wiedemann Syndrome.
A 3-month-old Chinese male infant with typical manifestations of Beckwith-Wiedemann Syndrome (BWS), such as macroglossia, hepatomegaly, umbilical hernia and hypoglycemia, presented with a large hepatic tumor. The tumor measured 7.6 x 8.0 x 7.5 cm. An open biopsy of the tumor revealed hepatoblastoma. The family refused chemotherapy, so only supportive care was given. The tumor grew very rapidly and the infant died 17 days after admission due to respiratory failure. To our knowledge, this is the first report of BWS associated with hepatoblastoma in a Chinese infant. This patient was a typical example of the association of BWS and hepatoblastoma, and the possible effect of growth factors on the rapid proliferation of the neoplasm in BWS.
Tsai
S Y
SY
Department of Pediatrics, National Taiwan University Hospital, Taipei.
Jeng
Y M
YM
Hwu
W L
WL
Ni
Y H
YH
Chang
M H
MH
Wang
T R
TR
D001506:Beckwith-Wiedemann Syndrome / Q000150:complications*; D018197:Hepatoblastoma / Q000150:complications* / Q000473:pathology; D006801:Humans; D007223:Infant; D008113:Liver Neoplasms / Q000150:complications* / Q000473:pathology; D008297:Male