A kindred of four generations had eight individuals with known neck masses. Four of these were bilateral and four were unilateral. Five neck masses were proven carotid body tumors by pathological examination of tissue. Two presumptive diagnoses were made on the basis of history and old family photographs. One was made by history, examination, and arteriography.
Although these cases suggested a sex-linked genetic trait, previous reports in the literature indicate that this conclusion would be invalid. This genetic study does demonstrate hereditary transmission of carotid body tumors. No case of malignancy has been reported in cases of bilateral carotid body tumors.