Familiar non-chromaffinic paragangliomas (glomus tumors). Clinical and genetic aspects (abridged).
The presence of glomus tumors in a large family was investigated by clinical and angioscintigraphic screening methods. Of 295 members of this family 162 persons participated in the study. A total of 47 tumors of the head and neck region were found in 26 patients. The study revealed a preponderance for the male sex, a fairly equal distribution of the different locations in the head and neck and a marked tendency towards multiplicity. The inheritance is autosomal dominant, with a clear increase of the penetrance with age.
D000293:Adolescent; D000328:Adult; D000368:Aged; D000375:Aging; D002648:Child; D002675:Child, Preschool; D002869:Chromosome Aberrations; D025063:Chromosome Disorders; D005260:Female; D005925:Glomus Jugulare Tumor / Q000235:genetics*; D006801:Humans; D008297:Male; D008875:Middle Aged; D010236:Paraganglioma, Extra-Adrenal / Q000235:genetics*; D010375:Pedigree; D012737:Sex Factors