Juvenile nasopharyngeal angiofibroma: no evidence for inheritance or association with familial adenomatous polyposis.
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.
Klockars
Tuomas
T
Department of Otorhinolaryngology, Head & Neck Surgery, Helsinki University Central Hospital, P.O. Box 220, 00029, Helsinki, Finland. tuomas.klockars@fimnet.fi
Renkonen
Suvi
S
Leivo
Ilmo
I
Hagström
Jaana
J
Mäkitie
Antti A
AA
D011125:Adenomatous Polyposis Coli / Q000235:genetics*; D018322:Angiofibroma / Q000235:genetics*; D020022:Genetic Predisposition to Disease; D006801:Humans; D008297:Male; D009303:Nasopharyngeal Neoplasms / Q000235:genetics*; D011795:Surveys and Questionnaires