Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Multicenter Study
. 2008 Sep;51(3):363-8.
doi: 10.1002/pbc.21632.

Extra-renal non-cerebral rhabdoid tumours

Franck Bourdeaut  1 , Paul Fréneaux, Bénédicte Thuille, Christophe Bergeron, Valérie Laurence, Laurence Brugières, Cécile Vérité, Jean Michon, Olivier Delattre, Daniel Orbach
Affiliations
Multicenter Study

Extra-renal non-cerebral rhabdoid tumours

Franck Bourdeaut et al. Pediatr Blood Cancer. 2008 Sep.

Abstract

Background: Rhabdoid tumours (RTs) are aggressive malignancies of childhood, mainly occurring in the kidney and brain. We describe a national multi-centre retrospective analysis of extra-renal non-cranial RTs (ERRTs).

Procedure: Diagnosis relied on central histological review and/or on hSNF5/INI1 defect, evidenced by immunohistochemistry or molecular screening. Clinical data were obtained from physicians.

Results: Twenty six patients fulfilled the inclusion criteria. Median age at diagnosis was 28 months [0-366], including late childhood and young adults cases. Surgery, either initial or secondary, was complete in three. All but three patients received chemotherapy, with variable regimens. Additional radiotherapy was used in six patients. Median time to recurrence or progression was 5 months [0-44], and one patient remained free of disease at 7 years.

Conclusions: ERRTs share the same chemosensitivity, early recurrence, and poor prognosis as renal and cerebral RTs. No chemotherapy regimen demonstrates a superior response.

PubMed Disclaimer

Publication types

Substances