The incidence of carotid body tumors is less than 1 in 30,000. Histologically, they present with a classic pattern of tumor cells arranged in "zellballen" or nests. A new extremely rare sclerosing subtype has been described in the literature with distinct stromal sclerosis and hyalinization, differentiating it from conventional paragangliomata. We present a case series of 3 patients with this rare variant and also discuss the diagnosis and management of carotid body parangliomas.
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