A Norepinephrine-secreting Glomus Jugulare Tumor Presenting as a Pheochromocytoma

Abstract

Tumors of the glomus jugulare have been known since 1945. The patient presented is the first reported instance of a glomus jugulare tumor which secreted large amounts of norepinephrine. The patient, a 33 year old white female, related symptoms of a pheochromocytoma since 1959. Pharmacologic tests and urinary assays for vanillylmandelic acid confirmed the presence of a catecholamine secreting tumor. Thoracotomy and two laparotomies failed to reveal the tumor. Catheterization of the vena cavae and internal jugular veins localized norepinephrine production to the right jugular vein. At operation, a glomus jugulare tumor, containing 14.1 mg of norepinephrine per gram of tissue, was partially removed. Subsequently the tumor was irradiated, but continued to function at 5 months following operation. The clinical features of glomus jugulare tumors are discussed. The debate concerning the embryologic origin of glomic tissue is briefly reviewed. The conditions comprising the neurocutaneous syndromes are mentioned in relation to glomic tissue. Kohn''s hypothesis of glomic tissue origin from neural crest receives support from these observations. Tentatively, inclusion of glomic tissue tumors into the syndromes of neuro-ecto-dermal dysplasia seems plausible.