The estimated incidence is at ~1 in 20,000 births.
In the vast majority (~90%) of cases fetal neuroblastomas arise in the adrenal glands. They have been diagnosed as early as 19 weeks of gestation, but usually are seen in the third trimester (with mean age at diagnosis being ~36 weeks).
There may be a right sided predilection.
They are typically seen as a complex mass with cystic components (~50% 10) around the expected position of the fetal adrenal gland. Some lesions may appear entirely solid of predominantly cystic.
Colour Doppler interrogation often shows diffuse vascularity rather than a single feeding vessel.
Staging is similar to standard neuroblastoma staging.
- developmental of hydrops fetalis: uncommon but a concerning complication
- development of maternal pre-eclampsia
Treatment and prognosis
In almost all cases the tumour undergoes maturation and regression, and prognosis is excellent (in contrast to childhood neuroblastoma) with many institutions recommending conservative management.
For ultrasound appearances consider:
- fetal adrenal haemorrhage: no colour flow
- sub diaphragmatic extra-lobar pulmonary sequestration: classically left sided and may demonstrate a single feeder vessel
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