Rhabdoid tumours (clinicians)
About rhabdoid tumours
Rhabdoid tumours are rare tumour that occur in childhood. Only a few children are diagnosed every year in the UK. There are 3 main types of rhabdoid tumours.
- Renal rhabdoid tumours – rhabdoid tumours that affect the kidney
- Atypical teratoid / rhabdoid tumours – rhabdoid tumours that affect the brain and CNS
- Extra renal rhabdoid tumours – rhabdoid tumours that occur outside the kidney, for example in the liver or soft tissue
Often, children with renal rhabdoid tumours are thought to have Wilms tumours until histology identifies a rhabdoid tumour. Â Most malignant rhabdoid tumours have characteristic genetic changes, with a SMARCB1 mutation of the INI1 gene.
Typically, malignant rhabdoid tumours are found in very young children. Two-thirds of cases occur in infants under 12 months of age.
Treatment of rhabdoid tumours
Rhabdoid tumours are highly aggressive tumours that are very challenging to treat. The mainstay of treatment is intensive chemotherapy, surgery and in some children radiotherapy.
The prognosis of infants and children diagnosed with rhabdoid tumours is poor. Children who have evidence of metastases at diagnosis have an even poorer prognosis and very few sadly survive.
For parents and carers of children who have received a diagnosis of a rhabdoid tumour, we run a rhabdoid parents support group.
