Ectopic adrenocorticotropic hormone syndrome: Localization studies in 28 patients

J. L. Doppman, L. Nieman, D. L. Miller, H. I. Pass, R. Chang, G. B. Cutler, M. Schaaf, G. P. Chrousos, J. A. Norton, H. A. Ziessman, E. H. Oldfield, Donald (Lynn) Loriaux

Research output: Contribution to journalArticle

127 Citations (Scopus)

Abstract

A variety of imaging procedures were performed in 28 patients with ectopic adrenocorticotropic hormone (ACTH) syndrome in an attempt to localize the ACTH-producing tumor. Diagnosis was made on the basis of removal of an ACTH-producing tumor or biopsy of metastases in the 19 patients with a proved source and the absence of ACTH gradients in bilateral samples of the inferior petrosal sinuses in the nine patients in whom an ACTH-secreting tumor had not been localized. Eleven bronchial carcinoids, two thymic carcinoids, three pheochromocytomas, and three islet-cell tumors constituted the proved sources. The condition has been cured in eight patients, six are alive with residual tumor, and five had died. Of the nine patients with undetected sites of ACTH production, one has died of pneumocystis pneumonia and eight are being treated medically or with bilateral adrenalectomy. Computed tomography (CT) of the chest and abdomen was the most helpful study in the detection of these tumors. Selective arteriography (bronchial and visceral), systemic and portal venous sampling, and iodine-131 meta-iodobenzylguanidine scintigraphy failed to demonstrate tumors when findings at CT were negative. Bronchial carcinoids constituted most of the ACTH-secreting tumors in this study (58%) and in a review of four large series (47%). To assure early detection of these potentially malignant tumors, pulmonary CT should be performed every 6 months, even after hypercortisolism has been medically or surgically controlled.

Original languageEnglish (US)
Pages (from-to)115-124
Number of pages10
JournalRadiology
Volume172
Issue number1
StatePublished - 1989
Externally publishedYes

Fingerprint

Paraneoplastic Endocrine Syndromes
Adrenocorticotropic Hormone
Carcinoid Tumor
Neoplasms
Tomography
3-Iodobenzylguanidine
Islet Cell Adenoma
Pneumocystis Pneumonia
Cushing Syndrome
Adrenalectomy
Residual Neoplasm
Pheochromocytoma
Radionuclide Imaging
Iodine
Abdomen
Angiography
Thorax
Neoplasm Metastasis
Biopsy
Lung

ASJC Scopus subject areas

  • Radiological and Ultrasound Technology

Cite this

Doppman, J. L., Nieman, L., Miller, D. L., Pass, H. I., Chang, R., Cutler, G. B., ... Loriaux, D. L. (1989). Ectopic adrenocorticotropic hormone syndrome: Localization studies in 28 patients. Radiology, 172(1), 115-124.

Ectopic adrenocorticotropic hormone syndrome : Localization studies in 28 patients. / Doppman, J. L.; Nieman, L.; Miller, D. L.; Pass, H. I.; Chang, R.; Cutler, G. B.; Schaaf, M.; Chrousos, G. P.; Norton, J. A.; Ziessman, H. A.; Oldfield, E. H.; Loriaux, Donald (Lynn).

In: Radiology, Vol. 172, No. 1, 1989, p. 115-124.

Research output: Contribution to journalArticle

Doppman, JL, Nieman, L, Miller, DL, Pass, HI, Chang, R, Cutler, GB, Schaaf, M, Chrousos, GP, Norton, JA, Ziessman, HA, Oldfield, EH & Loriaux, DL 1989, 'Ectopic adrenocorticotropic hormone syndrome: Localization studies in 28 patients', Radiology, vol. 172, no. 1, pp. 115-124.
Doppman JL, Nieman L, Miller DL, Pass HI, Chang R, Cutler GB et al. Ectopic adrenocorticotropic hormone syndrome: Localization studies in 28 patients. Radiology. 1989;172(1):115-124.
Doppman, J. L. ; Nieman, L. ; Miller, D. L. ; Pass, H. I. ; Chang, R. ; Cutler, G. B. ; Schaaf, M. ; Chrousos, G. P. ; Norton, J. A. ; Ziessman, H. A. ; Oldfield, E. H. ; Loriaux, Donald (Lynn). / Ectopic adrenocorticotropic hormone syndrome : Localization studies in 28 patients. In: Radiology. 1989 ; Vol. 172, No. 1. pp. 115-124.
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