Rhabdoid tumors are rare and extremely aggressive tumors of early childhood. They occur in a number of locations:
- kidney: see malignant rhabdoid tumor of the kidney, most common
- intracranial: see atypical teratoid/rhabdoid tumor (AT/RT)
- soft tissues, including:
- breast 3
- liver etc... 4
This article touches on the generalities of these tumors, but each is then discussed separately.
Almost all reported cases of rhabdoid tumors, 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 tumors, patients then subsequently develop intracranial atypical teratoid/rhabdoid tumor (AT/RT) 2.
Irrespective of site of origin, rhabdoid tumors share deletions in the long arm of chromosome 22, mapped to the INI1 gene, believed to be a tumor suppressor 1,4.
These tumors contain characteristic rhabdoid cells, so-called on account of the light microscopy resemblance to skeletal muscle cells 2.
- 1. Weiss SW, Goldblum JR, Enzinger FM. Enzinger and Weiss's soft tissue tumors. Mosby Inc. (2001) ISBN:0323012000. Read it at Google Books - Find it at Amazon
- 2. Berman JJ. Neoplasms, principles of development and diversity. Jones & Bartlett Learning. (2009) ISBN:0763755702. Read it at Google Books - Find it at Amazon
- 3. Koibuchi Y, Lino Y, Joshita T et-al. Malignant rhabdoid tumor of the breast: a case report. Jpn. J. Clin. Oncol. 1995;25 (6): 273-7. Jpn. J. Clin. Oncol. (abstract) - Pubmed citation
- 4. Biegel JA, Zhou JY, Rorke LB et-al. Germ-line and acquired mutations of INI1 in atypical teratoid and rhabdoid tumors. Cancer Res. 1999;59 (1): 74-9. Cancer Res. (full text) - Pubmed citation