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Myeloid leukemia in Prader-Willi syndrome
H Dele Davies1, Geraline L Leusink, Athena McConnell
1Departments of Pediatrics and Oncology, University of Calgary, Alberta Children's Hospital, Calgary, Alberta, Canada.
The Journal of Pediatrics
|February 14, 2003
Summary
Individuals with Prader-Willi syndrome (PWS) show a significantly higher risk for myeloid leukemias. This study found no increased risk for other cancers in the PWS population.
Area of Science:
- Oncology
- Genetics
- Epidemiology
Background:
- Prader-Willi syndrome (PWS) is a complex genetic disorder.
- Cancer surveillance in rare genetic conditions is crucial for understanding disease risks.
Purpose of the Study:
- To assess cancer incidence in individuals with Prader-Willi syndrome.
- To compare cancer occurrence in PWS patients to the general US population.
Main Methods:
- A survey was distributed to 1852 individuals registered with the PWS Association (USA).
- Cancer diagnoses (benign tumors, malignant tumors, leukemia) were recorded.
- Observed cancer cases in the PWS population (1975-1994) were compared to expected rates using SEER data (1971-1994).
Main Results:
- A response rate of 63% (1160/1852) was achieved.
- Eight cancer cases were observed in the PWS cohort versus 4.80 expected (P =.1610).
- Three cases of myeloid leukemia were observed, significantly higher than the 0.075 expected (P =.0001).
Conclusions:
- Individuals with Prader-Willi syndrome exhibit an elevated risk of developing myeloid leukemias.
- No increased risk for other cancer types was identified in the PWS population.