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 isle t cell tumor which exhibited ectopic ACTH syndrome associated with Zol linger-Ellison syndrome. When this combined syndrome was diagnosed, th ere were already multiple metastasis to the liver. Zollinger-Ellison s yndrome was treated with a histamine-H-2-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 out patient. During treatment she became cachexic. After she died, an auto psy was done. Immunohistochemical staining established that tumor cell s contained ACTH- and gastrin-like immunoreactivity. Our treatment sug gested the efficacy of chemical adrenalectomy in managing ectopic ACTH syndrome associated with Zollinger-Ellison syndrome.