Juvenile nasopharyngeal angiofibroma and familial adenomatous polyposis: an association?
Juvenile nasopharyngeal angiofibroma is a benign neoplasm affecting the nasopharynx of male adolescents. Two patients treated at Temple University Hospital for this condition were also diagnosed with familial adenomatous polyposis. Familial adenomatous polyposis results from the inheritance of a mutated adenomatous polyposis coli gene in an autosomal dominant pattern. The development of colorectal carcinoma in middle age is seen almost invariably in familial adenomatous polyposis, if a prophylactic colectomy is not performed. To identify a possible association between juvenile nasopharyngeal angiofibroma and familial adenomatous polyposis, chart reviews and patient interviews were carried out for all patients treated for juvenile nasopharyngeal angiofibroma at Temple University Hospital between 1985 and 1993. Single-strand conformational polymorphism was performed to detect the presence of certain adenomatous polyposis coli gene mutations within the germline DNA of those juvenile nasopharyngeal angiofibroma patients not previously found to have familial adenomatous polyposis. Although no more patients with both juvenile nasopharyngeal angiofibroma and familial adenomatous polyposis were found by these methods, the two patients with both disorders previously identified constitute 22% of our juvenile nasopharyngeal angiofibroma series. The implications of these findings are discussed.
Ferouz
A S
AS
Department of Otorhinolaryngology/Bronchoesophagology, Temple University Health Sciences Center, Philadelphia, Pennsylvania, USA.
Mohr
R M
RM
Paul
P
P
D011125:Adenomatous Polyposis Coli / Q000235:genetics*; D000293:Adolescent; D000328:Adult; D018322:Angiofibroma / Q000235:genetics / Q000473:pathology*; D002648:Child; D002872:Chromosome Deletion; D002874:Chromosome Mapping; D004252:DNA Mutational Analysis; D004273:DNA, Neoplasm / Q000235:genetics; D005799:Genes, Dominant / Q000235:genetics; D006801:Humans; D007407:Interviews as Topic; D008297:Male; D009154:Mutation / Q000235:genetics; D009303:Nasopharyngeal Neoplasms / Q000235:genetics / Q000473:pathology*; D009378:Neoplasms, Multiple Primary / Q000235:genetics / Q000473:pathology*; D018807:Polymorphism, Single-Stranded Conformational; D012189:Retrospective Studies
D004273:DNA, Neoplasm