JUVENILE NASOPHARYNGEAL ANGIOFIBROMA AND FAMILIAL ADENOMATOUS POLYPOSIS - AN ASSOCIATION

Juvenile nasopharyngeal angiofibroma is a benign neoplasm affecting th e nasopharynx of male adolescents, Two patients treated at Temple Univ ersity Hospital for this condition were also diagnosed with familiar a denomatous polyposis. Familial adenomatous polyposis results from the inheritance of a mutated adenomatous polyposis coli gene in an autosom al dominant pattern, The development of colorectal carcinoma in middle age is seen almost invariably in familiar adenomatous polyposis, if a prophylactic colectomy is not performed. To identify a possible assoc iation between juvenile nasopharyngeal angiofibroma and familial adeno matous polyposis, chart reviews and patient interviews were carried ou t for all patients treated for juvenile nasopharyngeal angiofibroma at Temple University Hospital between 1985 and 1993, Single-strand confo rmational 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 fou nd to have familial adenomatous polyposis. Although no more patients w ith both juvenile nasopharyngeal angiofibroma and familial adenomatous polyposis were found by these methods, the two patients with both dis orders previously identified constitute 22% of our juvenile nasopharyn geal angiofibroma series. The implications of these findings are discu ssed.