A1 Refereed original research article in a scientific journal
Juvenile nasopharyngeal angiofibroma: no evidence for inheritance or association with familial adenomatous polyposis
Authors: Klockars Tuomas, Renkonen Suvi, Leivo Ilmo, Hagström Jaana, Mäkitie Antti A
Publisher: SPRINGER
Publication year: 2010
Journal: Familial Cancer
Journal name in source: FAMILIAL CANCER
Journal acronym: FAM CANCER
Volume: 9
Issue: 3
First page : 401
Last page: 403
Number of pages: 3
ISSN: 1389-9600
DOI: https://doi.org/10.1007/s10689-010-9331-z
Abstract
Juvenile nasopharyngeal angiofibromas (JNAs) are rare tumors with prominent vascularity and locally destructive growth. The pathogenesis of JNA is largely unknown. A causal association between JNA and familial adenomatous polyposis has been suggested. Twenty-one patients diagnosed with juvenile angiofibroma filled out a detailed patient questionnaire. No patients reported any relatives with nasopharyngeal angiofibroma or familial adenomatous polyposis. No significant regional clustering suggestive for founder effect could be identified. We believe that if there were a strong genetic predisposition or association with familial adenomatous polyposis, it should have been seen in this patient sample.
Juvenile nasopharyngeal angiofibromas (JNAs) are rare tumors with prominent vascularity and locally destructive growth. The pathogenesis of JNA is largely unknown. A causal association between JNA and familial adenomatous polyposis has been suggested. Twenty-one patients diagnosed with juvenile angiofibroma filled out a detailed patient questionnaire. No patients reported any relatives with nasopharyngeal angiofibroma or familial adenomatous polyposis. No significant regional clustering suggestive for founder effect could be identified. We believe that if there were a strong genetic predisposition or association with familial adenomatous polyposis, it should have been seen in this patient sample.
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