Ectopic ACTH syndrome. Diagnostic and therapeutic aspects.

Abstract:

:Twenty-five patients were identified with non-pituitary, nonadrenal ACTH-secreting tumors (bronchial carcinoid, bronchial small cell carcinoma, pancreatic islet cell carcinoma, medullary thyroid carcinoma, thymic carcinoids, metastatic adenocarcinoma, and pancreatic cystadenoma). Clinical features were weakness, hypertension, cushingoid appearance, peripheral edema, personality disorders, and hyperpigmentation. Biochemical features were a markedly increased urinary free cortisol level (all patients), hypokalemia (71 percent of patients), and an elevated ACTH level (72 percent of patients). Surgical therapy consisted of bilateral total adrenalectomy (56 percent of patients). Twelve percent underwent transsphenoidal hypophysectomy and 36 percent had excision of their tumor. No surgical therapy was undertaken in 28 percent. Bilateral total adrenalectomy in patients with a slow-growing malignancy or an unknown tumor secreting ACTH is beneficial in relieving symptoms and prolonging life. Excision of nonmalignant ACTH-producing tumors yields an excellent long-term prognosis.

journal_name

Am J Surg

authors

Jex RK,van Heerden JA,Carpenter PC,Grant CS

doi

10.1016/s0002-9610(85)80085-4

subject

Has Abstract

pub_date

1985-02-01 00:00:00

pages

276-82

issue

2

eissn

0002-9610

issn

1879-1883

pii

S0002-9610(85)80085-4

journal_volume

149

pub_type

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