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Original Article
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A proposed explanation for female predominance in alveolar soft part sarcoma
Noninactivation of X; Autosome translocation fusion gene?
Article first published online: 3 FEB 2005
DOI: 10.1002/cncr.20899
Copyright © 2005 American Cancer Society
Additional Information
How to Cite
Bu, X. and Bernstein, L. (2005), A proposed explanation for female predominance in alveolar soft part sarcoma. Cancer, 103: 1245–1253. doi: 10.1002/cncr.20899
Publication History
- Issue published online: 2 MAR 2005
- Article first published online: 3 FEB 2005
- Manuscript Accepted: 24 NOV 2004
- Manuscript Revised: 21 NOV 2004
- Manuscript Received: 29 JUN 2004
- Abstract
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Keywords:
- alveolar soft part sarcoma;
- ASPS;
- X-inactivation;
- female predominance;
- nonreciprocal translocation
Alveolar soft part sarcoma (ASPS), a rare malignant soft tissue tumor with a predilection for females, was recently found to have a nonreciprocal der(17)t(X;17) translocation. A statistical association between the female predominance observed for ASPS and females' possession of an extra X-chromosome/noninactivation of the ASPS X;autosome translocation fusion gene was found.
Abstract
BACKGROUND
Alveolar soft-part sarcoma (ASPS) is a rare malignant soft tissue tumor with both clinically and morphologically distinct features. It often involves the extremities of adolescents and young adults and shows a predilection for females. Recently, ASPS was found to have a nonreciprocal der(17)t(X;17) translocation with the corresponding fusion gene located in chromosome 17. Because females have an extra X-chromosome, their likelihood of developing an X;autosome translocation is theoretically double that of males, and thus, this extra X-chromosome is a likely explanation for female predominance of ASPS.
METHODS
The authors used data from the National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) registry program, which included 87 ASPS cases (33 males and 54 females), and published ASPS cases, which included 317 cases (121 males and 196 females), to test our hypothesis. The authors compared the observed proportion of female cases with that expected under the two X-chromosomes–double-risk hypothesis including the consideration of X-inactivation status.
RESULTS
The hypothesis that the fusion gene is not subject to X-inactivation is supported by data (P = 0.6, 0.24, and 0.20 for SEER cases, published cases, and their combination, respectively). In contrast, the competing hypothesis that the fusion gene is subject to X-inactivation is rejected (P = 0.007, < 0.00001, and < 0.00001 for SEER cases, published cases, and their combination, respectively).
CONCLUSIONS
Therefore, the authors found a statistical association between the female predominance observed in ASPS and female possession of an extra X-chromosome/noninactivation of the ASPS X;autosome translocation fusion gene. Cancer 2005. © 2005 American Cancer Society.