A case of ectopic ACTH syndrome associated with Zollinger-Ellison syndrome: long-term survival with chemical adrenalectomy.
The female patient in this report was suffering from a pancreatic islet cell tumor which exhibited ectopic ACTH syndrome associated with Zollinger-Ellison syndrome. When this combined syndrome was diagnosed, there were already multiple metastasis to the liver. Zollinger-Ellison syndrome was treated with a histamine-H2-receptor blocker, and ectopic ACTH syndrome was controlled with trilostane, metyrapone and o,p'-DDD. She survived more than 6 years after the diagnosis, mostly as an outpatient. During treatment she became cachexic. After she died, an autopsy was done. Immunohistochemical staining established that tumor cells contained ACTH- and gastrin-like immunoreactivity. Our treatment suggested the efficacy of chemical adrenalectomy in managing ectopic ACTH syndrome associated with Zollinger-Ellison syndrome.
Fourth Department of Internal Medicine, University of Tokyo School of Medicine, Japan.
H
H
Ishido
N
N
Yamashita
M
M
Kitaoka
Y
Y
Tanaka
E
E
Ogata
D000182:ACTH Syndrome, Ectopic / Q000209:etiology / Q000401:mortality / Q000601:surgery*; D000315:Adrenalectomy / Q000379:methods*; D018273:Carcinoma, Islet Cell / Q000150:complications / Q000401:mortality / Q000556:secondary / Q000601:surgery*; D005260:Female; D006801:Humans; D008875:Middle Aged; D010190:Pancreatic Neoplasms / Q000150:complications / Q000401:mortality / Q000601:surgery*; D015996:Survival Rate; D013997:Time Factors; D015043:Zollinger-Ellison Syndrome / Q000209:etiology / Q000401:mortality / Q000601:surgery*