A patient with malignant paraganglioma that simultaneously produces adrenocorticotropic hormone and interleukin-6.

Abstract

To the authors' knowledge, there have been no reports describing a case of malignant paraganglioma that produces both adrenocorticotropic hormone (ACTH) and interleukin-6 (IL-6). The clinical course and pathophysiology of a patient with Cushing's syndrome induced by ectopic ACTH syndrome caused by a cervical malignant paraganglioma with elevated plasma levels of IL-6 was investigated. Ultrasonography, computed tomography, and magnetic resonance imaging of the neck revealed the presence of a tumor around the area from which the blood sample that showed the highest levels of ACTH and IL-6 was obtained by selective catheterization. Immunohistochemical staining of the removed tissue demonstrated the presence of ACTH and IL-6 proteins in the tumor cells. Reverse transcriptase-polymerase chain reaction also revealed the existence of IL-6 mRNA in those cells. The malignant paraganglioma of ectopic ACTH syndrome may produce IL-6. The present investigation provides new observations concerning ectopic ACTH syndrome.