Rhabdoid tumours

Last revised by Ayush Goel on 29 Sep 2014

Citation, DOI, disclosures and article data

Citation:

Gaillard F, Goel A, Rhabdoid tumours. Reference article, Radiopaedia.org (Accessed on 24 Apr 2024) https://doi.org/10.53347/rID-12421

DOI:

https://doi.org/10.53347/rID-12421

Permalink:

https://radiopaedia.org/articles/12421

rID:

12421

Article created:

23 Nov 2010, Frank Gaillard ◉ ◈

Disclosures:

At the time the article was created Frank Gaillard had no recorded disclosures.

View Frank Gaillard's current disclosures

Last revised:

29 Sep 2014, Ayush Goel ◉

Disclosures:

At the time the article was last revised Ayush Goel had no recorded disclosures.

View Ayush Goel's current disclosures

Revisions:

1 time, by 1 contributor - see full revision history and disclosures

Systems:

Paediatrics

Synonyms:

Rhabdoid tumors

Rhabdoid tumours are rare and extremely aggressive tumours of early childhood. They occur in a number of locations:

kidney: see malignant rhabdoid tumour of the kidney, most common
intracranial: see atypical teratoid/rhabdoid tumour (AT/RT)
soft tissues, including:
- breast ³
- skin
- orbit
- liver etc... ⁴

This article touches on the generalities of these tumours, but each is then discussed separately.

Epidemiology

Almost all reported cases of rhabdoid tumours, regardless of site of origin, occur in young children, between birth and 2 years of age ⁴.

In approximately 10% of cases of renal malignant rhabdoid tumours, patients then subsequently develop intracranial atypical teratoid/rhabdoid tumour (AT/RT) ².

Pathology

Irrespective of site of origin, rhabdoid tumours share deletions in the long arm of chromosome 22, mapped to the INI1 gene, believed to be a tumour suppressor ^1,4.

These tumours contain characteristic rhabdoid cells, so-called on account of the light microscopy resemblance to skeletal muscle cells ².