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


METHODS
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.


RESULTS
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.


CONCLUSIONS
The malignant paraganglioma of ectopic ACTH syndrome may produce IL-6. The present investigation provides new observations concerning ectopic ACTH syndrome.
Omura
M
M
Department of Medicine, Yokohama Rosai Hospital, Japan.
Sato
T
T
Cho
R
R
Iizuka
T
T
Fujiwara
T
T
Okamoto
K
K
Tashiro
Y
Y
Chiba
S
S
Nishikawa
T
T
D000182:ACTH Syndrome, Ectopic / Q000209:etiology*; D000324:Adrenocorticotropic Hormone / Q000032:analysis / Q000097:blood*; D003480:Cushing Syndrome / Q000209:etiology*; D006258:Head and Neck Neoplasms / Q000097:blood / Q000150:complications* / Q000473:pathology; D006801:Humans; D015850:Interleukin-6 / Q000032:analysis / Q000097:blood*; D007601:Jugular Veins / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009479:Neuropeptides / Q000032:analysis / Q000097:blood; D018377:Neurotransmitter Agents / Q000032:analysis / Q000097:blood; D010235:Paraganglioma / Q000097:blood / Q000150:complications* / Q000473:pathology; D051219:Pituitary Adenylate Cyclase-Activating Polypeptide; D011388:Prolactin / Q000097:blood
C495401:ADCYAP1 protein, human; D015850:Interleukin-6; D009479:Neuropeptides; D018377:Neurotransmitter Agents; D051219:Pituitary Adenylate Cyclase-Activating Polypeptide; D000324:Adrenocorticotropic Hormone; D011388:Prolactin