Hepatoblastoma in children with Beckwith-Wiedemann syndrome: does it warrant different treatment?

Hepatoblastoma in children with Beckwith-Wiedemann syndrome: does it warrant different treatment?

Trobaugh-Lotrario AD, Venkatramani R, Feusner JH.
PMID: 24608075

In this paper, we analyzed the cases with hepatoblastoma among 56 BWS patients. Patients with Beckwith-Wiedemann Syndrome (BWS) are 2,000 times more likely to get hepatoblastoma than the average population. At the time of diagnosis of hepatoblastoma, the mean age of the patients was 6 months. Of the 26 patients, 13 were born premature. Of 31 patients, 19 had hemihypertrophy and 32 of 33 had AFP levels at diagnosis. The overall survival rate was about 75%, of which 27 out of 36 survived. Of the 25 survivors, 24 received chemotherapy and surgery. All 9 patients who had undergone routine liver cancer screening were all alive until the time of the report, suggesting a prospective study of whether BWS patients will be regularly screened for further survival after early detection.

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