Wilms tumour – risk management

Wilms tumour (WT) or nephroblastoma is the most common renal malignancy of childhood affecting approximately 1 in 10,000 children. Up to 30% of children with Wilms tumour will have a genetic or epigenetic variant that increases their risk of Wilms tumour.^r

Predisposition in Wilms tumour can be broadly divided into two categories:

• Syndromic Wilms
  • large and diverse group of conditions, each with a recognised pattern of clinical features and inheritance ^r
• Non-syndromic Wilms​
  • known/described genetic/epigenetic variant that increases risk of Wilms tumour
  • familial history of Wilms with no identified genetic variant (familial Wilms tumour) 

• Phenotypic features consistent with a known syndrome associated with a greater than 1% lifetime risk of Wilms tumour
• Unaffected known or obligate carrier of a genetic variant associated with a greater than 1% increased risk of Wilms tumour
• Unaffected child who has 2 or more relatives with non-syndromic Wilms tumour in the family where a genetic variant has not been identified (familial Wilms tumour)

Exclusion criteria

• Children already diagnosed with Wilms tumour
• Phenotypic features consistent with a known syndrome associated with a less than 1% lifetime risk of Wilms tumour
• Unaffected known or obligate carrier of a genetic variant associated with a less than 1% increased risk of Wilms tumour
• Beckwith-Wiedemann syndrome (see Beckwith Wiedemann syndrome - risk management)
• Individual with a variant of uncertain significance

*Risk of other malignancies 

The choice of risk management strategy should take into account current age, other health issues, age-related cancer risk and the estimated risk associated with the underlying genetic diagnosis. Risks and benefits of interventions should be discussed with an experienced medical professional.

The impact of lifestyle on cancer risk should be discussed e.g. exercise most days for at least 30 minutes at moderate or strenuous intensity, maintain a healthy weight, have a healthy diet, limit alcohol intake, do not smoke and avoid excess sun exposure.

For individuals with genetic diagnosis within the high and moderate risk category, the recommended risk management strategy is outlined below.

For DICER1 risk management strategy see DICER1 - risk management (child)

The choice of risk management strategy within the group of low risk Wilms tumour (≤2%) is unclear and there is no consensus.

A diverse number of conditions are implicated in predisposition to the development of Wilms tumour and each condition may be associated with a unique array of health problems. These need to be appropriately identified and followed up.

There is limited data on the effectiveness of surveillance in children at increased risk of Wilms tumour. These recommendations represent expert opinion.

Surveillance

There is limited evidence about the efficacy of Wilms tumour surveillance in children at increased risk of Wilms tumour. Wilms tumour predispositions are rare, and this limits the ability of clinical researchers to perform large prospective studies to address the utility of surveillance. In addition, there are uncertainties with regard to the risk and natural history of Wilms tumour in predisposing conditions. Retrospective studies have shown that children with Beckwith-Wiedemann syndrome spectrum undergoing surveillance for Wilms tumour had lower stage Wilms tumour when compared to children not undergoing surveillance.^r

To date international expert opinions support of surveillance in children who are at increased risk of Wilms tumour due to the reasonable assumption that early detection of Wilms tumour will reduce the morbidity associated with the disease and its management through reduced treatment burden.^rr In particular, smaller lesions are more likely to be amenable to nephron-sparing surgery reducing the lifetime risk of renal impairment. The frequency of imaging is based on the expected growth trajectory of Wilms tumour. Approximately 95% of cases of Wilms tumour have presented prior to the age of 7 and so surveillance should continue until this age.^r

Informing family members about hereditary cancer

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