Serum α-fetoprotein screening for hepatoblastoma in children with Beckwith-Wiedemann syndrome or isolated hemihyperplasia

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Abstract

An elevated risk of hepatoblastoma for children with Beckwith-Wiedemann syndrome or isolated hemihyperplasia is well established. We describe five children with Beckwith-Wiedemann syndrome or isolated hemihyperplasia for whom serial serum α-fetoprotein screening, usually in combination with abdominal ultrasound, led to early detection of hepatoblastoma.

Section snippets

Methods

The case series consists of five children from five medical centers who underwent screening for HBL in the course of routine medical care.

Results

Five children were identified with Stage 1 HBL after the AFP measurement increased when compared with the previous measurement, even when the US was normal (Cases 4 and 5) or AFP was done alone and prompted US (Cases 1, 2, and 3) (Table). The magnitude of the increase in AFP measurement that led to the diagnosis varied greatly, from 9% to 669% (absolute increase in values ranged from 89 ng/mL to 26,000 ng/mL). The average age for diagnosis of HBL was 6.3 months.

Discussion

The potential benefit of early detection of HBL is underscored by the markedly better prognosis of Stages I and II (91% five-year event-free survival) when compared with Stages III and IV (64% and 25% five-year event-free survival, respectively). All five of our patients had stage 1 tumors, compared with a series of 182 children with HBL, two thirds of whose tumors were stage III or IV at presentation.9 The long-term outcome of our five patients is not available to us, but would not be expected

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