Skip to main content
PMC home page
The Yale Journal of Biology and Medicine logoLink to The Yale Journal of Biology and Medicine
. 1979 Mar-Apr;52(2):181–189.

Ectopic ACTH syndrome due to pheochromocytoma: case report and review of the literature.

B H Forman, E Marban, R D Kayne, N M Passarelli, S N Bobrow, V A Livolsi, M Merino, M Minor, L R Farber
PMCID: PMC2595434  PMID: 222080

Abstract

A 51-year-old female was diagnosed preoperatively to have a pheochromocytoma producing ACTH. This diagnosis was based upon her paroxysmal hypertension, hyperpigmentation, and hypokalemia. Elevated levels of serum and urine corticosteroids, plasma ACTH, urinary VMA, and catecholamines fell after a right adrenal pheochromocytoma was removed. Subsequently this tumor was found to have a high content of ACTH. Review of the literature indicates a mortality rate of 57% for this syndrome. Proper preoperative recognition and management can result in total cure.

Full text

PDF
181

Images in this article

183FIG. 1
on p.183
184FIG. 2
on p.184
184FIG. 3
on p.184
185FIG. 4
on p.185
186FIG. 5
on p.186
186FIG. 6
on p.186

Selected References

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

  1. ANTON A. H., SAYRE D. F. A study of the factors affecting the aluminum oxide-trihydroxyindole procedure for the analysis of catecholamines. J Pharmacol Exp Ther. 1962 Dec;138:360–375. [PubMed] [Google Scholar]
  2. ARMSTRONG M. D., SHAW K. N., WALL P. E. The phenolic acids of human urine; paper chromatography of phenolic acids. J Biol Chem. 1956 Jan;218(1):293–303. [PubMed] [Google Scholar]
  3. ARRIETA F., MORROS J. J., OLIVA H., ZUMEL M. F. [Cushing's syndrome and pheochromocytoma]. Rev Clin Esp. 1961 Feb 28;80:242–246. [PubMed] [Google Scholar]
  4. Azzopardi J. G., Williams E. D. Pathology of 'nonendocrine' tumors associated with Cushing's syndrome. Cancer. 1968 Aug;22(2):274–286. doi: 10.1002/1097-0142(196808)22:2<274::aid-cncr2820220204>3.0.co;2-w. [DOI] [PubMed] [Google Scholar]
  5. BOURGOIGNIE J., DUPONT J. C., NOIRET R. ASSOCIATION D'UN PH'EOCHROMOCYTOME ET D'UN HYPERCORTICISME DU TYPE CUSHING AVEC HYPERALDOST'ERONURIE. Ann Endocrinol (Paris) 1964 May-Jun;25:269–284. [PubMed] [Google Scholar]
  6. BUCALOSSI P., PAGNONI A., ROCK T. FEOCROMOCITOMA INTRATORACICO CON SEGNI DI IPERFUNZIONE CORTICALE. Tumori. 1964 May-Jun;50:213–221. doi: 10.1177/030089166405000304. [DOI] [PubMed] [Google Scholar]
  7. Berenyi M. R., Singh G., Gloster E. S., Davidson M. I., Woldenberg D. H. ACTH-producing pheochromocytoma. Arch Pathol Lab Med. 1977 Jan;101(1):31–35. [PubMed] [Google Scholar]
  8. Berson S. A., Yalow R. S. Radioimmunoassay of ACTH in plasma. J Clin Invest. 1968 Dec;47(12):2725–2751. doi: 10.1172/JCI105955. [DOI] [PMC free article] [PubMed] [Google Scholar]
  9. Burmeister P., Simon A. Episodische Sekretion von ACTH durch einen Tumor des Nebennierenmarks. Dtsch Med Wochenschr. 1975 May 16;100(20):1132–1135. doi: 10.1055/s-0028-1106347. [DOI] [PubMed] [Google Scholar]
  10. COPE O., LABBE J. P., RAKER J. W., BLAND E. F. Pheochromocytoma and adrenal cortical adenoma; report of a case with both tumors and discussion of their relation. J Clin Endocrinol Metab. 1952 Jul;12(7):875–880. doi: 10.1210/jcem-12-7-875. [DOI] [PubMed] [Google Scholar]
  11. Deftos L. J., Lee M. R., Potts J. T., Jr A radioimmunoassay for thyrocalcitonin. Proc Natl Acad Sci U S A. 1968 May;60(1):293–299. doi: 10.1073/pnas.60.1.293. [DOI] [PMC free article] [PubMed] [Google Scholar]
  12. Degroot L. J., Hoye K. Dexamethasone suppression of serum T3 and T4. J Clin Endocrinol Metab. 1976 May;42(5):976–978. doi: 10.1210/jcem-42-5-976. [DOI] [PubMed] [Google Scholar]
  13. FREEDMAN P., MOULTON R., ROSENHEIM M. L., SPENCER A. G., WILLOUGHBY D. A. Phaeochromocytoma, diabetes, and glycosuria. Q J Med. 1958 Jul;27(107):307–321. [PubMed] [Google Scholar]
  14. Gerasimenko P. P., Uranova E. V., Il'enko T. I., Lukicheva T. I., Egorova I. A. Sindrom total'nogo giperkortitsizma u bol'noi feokhromoblastomoi. Probl Endokrinol (Mosk) 1974 Mar-Apr;20(2):38–41. [PubMed] [Google Scholar]
  15. HUTCHISON G. B., EVANS J. A., DAVIDSON D. C. Pitfalls in the diagnosis of pheochromocytoma. Ann Intern Med. 1958 Feb;48(2):300–309. doi: 10.7326/0003-4819-48-2-300. [DOI] [PubMed] [Google Scholar]
  16. Henry D. P., Starman B. J., Johnson D. G., Williams R. H. A sensitive radioenzymatic assay for norepinephrine in tissues and plasma. Life Sci. 1975 Feb 1;16(3):375–384. doi: 10.1016/0024-3205(75)90258-1. [DOI] [PubMed] [Google Scholar]
  17. KAPLAN L. I., SOKOLOFF L. Sympathicoblastoma with metastases, associated with the clinical picture of Cushing's syndrome; report of a case. Arch Neurol Psychiatry. 1949 Nov;62(5):696–698. [PubMed] [Google Scholar]
  18. Liddle G. W., Givens J. R., Nicholson W. E., Island D. P. The ectopic ACTH syndrome. Cancer Res. 1965 Aug;25(7):1057–1061. [PubMed] [Google Scholar]
  19. Liddle G. W., Nicholson W. E., Island D. P., Orth D. N., Abe K., Lowder S. C. Clinical and laboratory studies of ectopic humoral syndromes. Recent Prog Horm Res. 1969;25:283–314. doi: 10.1016/b978-0-12-571125-8.50009-0. [DOI] [PubMed] [Google Scholar]
  20. MARTIN E., RUTISHAUSER E., BACH J. Syndrome de Cushing chez une malade ayant présenté une tumeur du paraganglion carotidien. Ann Endocrinol (Paris) 1952;13(6):938–942. [PubMed] [Google Scholar]
  21. Mathison D. A., Waterhouse C. A. Cushing's syndrome with hypertensive crisis and mixed adrenal cortical adenoma-pheochromocytoma (corticomedullary adenoma). Am J Med. 1969 Oct;47(4):635–641. doi: 10.1016/0002-9343(69)90193-4. [DOI] [PubMed] [Google Scholar]
  22. Mathys S., Ziegler W. H., Francke C. Bilaterales Phäochyozytom-medullares Schiddrüsenkarzinom mit Cushing-Syndrom. Schweiz Med Wochenschr. 1972 Jun 10;102(23):798–803. [PubMed] [Google Scholar]
  23. Meloni C. R., Tucci J., Canary J. J., Kyle L. H. Cushing's syndrome due to bilateral adrenocortical hyperplasia caused by a benign adrenal medullary tumor. J Clin Endocrinol Metab. 1966 Nov;26(11):1192–1200. doi: 10.1210/jcem-26-11-1192. [DOI] [PubMed] [Google Scholar]
  24. Moorhead E. L., 2nd, Caldwell J. R., Kelly A. R., Morales A. R. The diagnosis of pheochromocytoma. Analysis of 26 cases. JAMA. 1966 Jun 27;196(13):1107–1113. [PubMed] [Google Scholar]
  25. O'Neal L. W., Kipnis D. M., Luse S. A., Lacy P. E., Jarett L. Secretion of various endocrine substances by ACTH-secreting tumors--gastrin, melanotropin, norepinephrine, serotonin, parathormone, vasopressin, glucagon. Cancer. 1968 Jun;21(6):1219–1232. doi: 10.1002/1097-0142(196806)21:6<1219::aid-cncr2820210625>3.0.co;2-m. [DOI] [PubMed] [Google Scholar]
  26. Orth D. N., Nicholson W. E., Mitchell W. M., Island D. P., Liddle G. W. Biologic and immunologic characterization and physical separation of ACTH and ACTH fragments in the ectopic ACTH syndrome. J Clin Invest. 1973 Jul;52(7):1756–1769. doi: 10.1172/JCI107357. [DOI] [PMC free article] [PubMed] [Google Scholar]
  27. ROUX G., MARCHAL G., LOUBATIERES R. Phéochromocytome surrénalien latent révélé par un syndrome d'hypercorticisme. 1955 Oct 26-Nov 9Mem Acad Chir (Paris) 81(27-28):847–852. [PubMed] [Google Scholar]
  28. Rees L. H. The biosynthesis of hormones by non-endocrine tumours--a review. J Endocrinol. 1975 Oct;67(1):143–175. doi: 10.1677/joe.0.0670143. [DOI] [PubMed] [Google Scholar]
  29. Schteingart D. E., Conn J. W., Orth D. N., Harrison T. S., Fox J. E., Bookstein J. J. Secretion of ACTH and -MSH by an adrenal medullary paraganglioma. J Clin Endocrinol Metab. 1972 Apr;34(4):676–683. doi: 10.1210/jcem-34-4-676. [DOI] [PubMed] [Google Scholar]
  30. Tannenbaum M. Ultrastructural pathology of adrenal medullary tumors. Pathol Annu. 1970;5:145–171. [PubMed] [Google Scholar]
  31. Vetter H., Fsicher N., Bayer J. M., Schmitz T. E., Werning C., Vetter W. Alodsteron, Cortisol und Plasma-Renin-Aktivität beim Phäochromocytom. Klin Wochenschr. 1974 Aug 1;52(15):719–721. doi: 10.1007/BF01469334. [DOI] [PubMed] [Google Scholar]
  32. WILLIAMS G. A., CROCKETT C. L., BUTLER W. W., 3rd, CRISPELL K. R. The coexistence of pheochromocytoma and adrenocortical hyperplasia. J Clin Endocrinol Metab. 1960 Apr;20:622–631. doi: 10.1210/jcem-20-4-622. [DOI] [PubMed] [Google Scholar]
  33. Wilson R. J., Craig G. M., Mills I. H. Metabolic studies in a patient with a phaeochromocytoma associated with hypokalaemia and hyperaldosteronism. J Endocrinol. 1973 Jan;56(1):69–78. doi: 10.1677/joe.0.0560069. [DOI] [PubMed] [Google Scholar]
  34. Wurtman R. J. Control of epinephrine synthesis in the adrenal medulla by the adrenal cortex: hormonal specificity and dose-response characteristics. Endocrinology. 1966 Sep;79(3):608–614. doi: 10.1210/endo-79-3-608. [DOI] [PubMed] [Google Scholar]

Articles from The Yale Journal of Biology and Medicine are provided here courtesy of Yale Journal of Biology and Medicine

RESOURCES