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Centre de Recherche sur les Maladies Endocriniennes, Formation Associée Claude Bernard, and the Department of Internal Medicine, Hôpital Cochin; and the Laboratoire dExplorations Fonctionnelles, Hôpital Trousseau Paris, France
Address requests for reprints to: Xavier Bertagna, Centre de Recherches sur les Maladies Endocriniennes, Hopital Cochin, 27 rue du Faubourg Saint Jacques, 75014 Paris, France.
The long-acting somatostatin analog (octreotide) was administered to a 37-yr-old woman with the ectopic ACTH syndrome. The patient had diffuse metastatic spread of a nonpituitary tumor, presumably of pancreatic origin, and severe and rapidly progressive hypercortisolism with extreme myopathy, hypokalemia, and diabetes mellitus. Plasma ACTH and lipotropin levels and 24-h urinary cortisol excretion were greatly elevated [218 pg/mL (48 pmol/L), 1340 pg/mL (220 pmol/L), and up to 830 µg/24 h (2290 nmol/day), respectively]. Urinary cortisol excretion decreased to normal within 3 days after the initiation of octreotide therapy (150, 300, and 600 µg/day), and plasma ACTH and lipotropin levels also decreased. Urinary cortisol excretion remained normal for 2 months during chronic octreotide therapy, and her general condition improved dramatically. The only side-effect was a slight increase in the number of bowel movements. Tumor progression, however, was not controlled, and she eventually died of hepatic insufficiency. These data indicate that octreotide can be a highly effective treatment for patients with the ectopic ACTH syndrome.
* This work was supported in part by INSERM Contrat de Recherche Externe 864003 and Association from la Recherche sur le Cancer Contract 6435.
Received July 5, 1988.
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