Skip to main content
Postgraduate Medical Journal logoLink to Postgraduate Medical Journal
. 1987 Oct;63(744):887–889. doi: 10.1136/pgmj.63.744.887

Ectopic Cushing's syndrome with periodic hormonogenesis--a case suggesting a pathogenetic mechanism.

V Estopiñán 1, C Varela 1, P Riobo 1, J R Dominguez 1, J Sancho 1
PMCID: PMC2428613  PMID: 2833733

Abstract

We report on a case of ectopic Cushing's syndrome due to a thymic carcinoid tumour with periodic hormonogenesis. Periods of hormonal production averaged 27 days. Prior to bilateral adrenalectomy, mean (s.d) values of ACTH and cortisol were 202.1 (50.3) pg/ml and 46 (14.7) micrograms/dl, ACTH rising to 3996 +/- 425 pg/ml (P less than 0.01) and cortisol falling to 6.3 +/- 1.5 micrograms/dl (P less than 0.01) in the immediate postoperative period. During the late postoperative period (2-13 months following surgery) ACTH levels fell to 509.3 (123.8) pg/ml (P less than 0.01), but remaining even higher (P less than 0.01) than before adrenalectomy. The pattern of ACTH in the present case suggests the existence of a negative feedback exerted by the cortisol over tumoral ACTH.

Full text

PDF
889

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Bailey R. E. Periodic hormonogenesis--a new phenomenon. Periodicity in function of a hormone-producing tumor in man. J Clin Endocrinol Metab. 1971 Mar;32(3):317–327. doi: 10.1210/jcem-32-3-317. [DOI] [PubMed] [Google Scholar]
  2. Brown R. D., Van Loon G. R., Orth D. N., Liddle G. W. Cushing's disease with periodic hormonogenesis: one explanation for paradoxical response to dexamethasone. J Clin Endocrinol Metab. 1973 Mar;36(3):445–451. doi: 10.1210/jcem-36-3-445. [DOI] [PubMed] [Google Scholar]
  3. Chajek T., Romanoff H. Cushing syndrome with cyclical edema and periodic secretion of corticosteroids. Arch Intern Med. 1976 Apr;136(4):441–443. [PubMed] [Google Scholar]
  4. Corrigan D. F., Schaaf M., Whaley R. A., Czerwinski C. L., Earll J. M. Selective venous sampling to differentiate ectopic ACTH secretion from pituitary Cushing's syndrome. N Engl J Med. 1977 Apr 14;296(15):861–862. doi: 10.1056/NEJM197704142961508. [DOI] [PubMed] [Google Scholar]
  5. French F. S., Macfie J. A., Baggett B., Williams T. F., Van Wyk J. J. Cushing's syndrome with a paradoxical response to dexamethasone. Am J Med. 1969 Oct;47(4):619–624. doi: 10.1016/0002-9343(69)90191-0. [DOI] [PubMed] [Google Scholar]
  6. Green J. R., van't Hoff W. Cushing's syndrome with fluctuation due to adrenal adenoma. J Clin Endocrinol Metab. 1975 Aug;41(2):235–240. doi: 10.1210/jcem-41-2-235. [DOI] [PubMed] [Google Scholar]
  7. Liberman B., Wajchenberg B. L., Tambascia M. A., Mesquita C. H. Periodic remission in Cushing's disease with paradoxical dexamethasone response: an expression of periodic hormonogenesis. J Clin Endocrinol Metab. 1976 Oct;43(4):913–918. doi: 10.1210/jcem-43-4-913. [DOI] [PubMed] [Google Scholar]
  8. Pimstone B. L., Uys C. J., Vogelpoel L. Studies in a case of Cushing's syndrome due to an ACTH-producing thymic tumour. Am J Med. 1972 Oct;53(4):521–528. doi: 10.1016/0002-9343(72)90149-0. [DOI] [PubMed] [Google Scholar]
  9. Schweikert H. U., Fehm H. L., Fahlbusch R., Martin R., Kolloch R., Higuchi M., Krück F. Cyclic Cushing's syndrome combined with cortisol suppressible, dexamethasone non-suppressible ACTH secretion: a new variant of Cushing's syndrome. Acta Endocrinol (Copenh) 1985 Nov;110(3):289–295. doi: 10.1530/acta.0.1100289. [DOI] [PubMed] [Google Scholar]
  10. Thorner M. O., Martin W. H., Ragan G. E., MacLeod R. M., Feldman P. S., Bruni C., Williamson B. R., Orth D. N. A case of ectopic ACTH syndrome: diagnostic difficulties caused by intermittent hormone secretion. Acta Endocrinol (Copenh) 1982 Mar;99(3):364–370. doi: 10.1530/acta.0.0990364. [DOI] [PubMed] [Google Scholar]

Articles from Postgraduate Medical Journal are provided here courtesy of BMJ Publishing Group

RESOURCES