An Unusual Case of Cushing's Syndrome Due to Bihormonal ACTH-prolactin Secreting Pituitary Macroadenoma with Rapid Response to Cabergoline

Overview

Journal BMJ Case Rep

Specialty General Medicine

Date 2017 Aug 9

PMID 28784879

Citations 3

Authors

Shalini Kunasegaran

Michael S Croxson

Ian Holdaway

Rinki Murphy

Affiliations

Soon will be listed here.

Abstract

A 23-year-old man presenting with florid Cushing's syndrome was found to have high plasma ACTH and very high serum prolactin. Pituitary MRI showed a large invasive macroadenoma. Low-dose cabergoline promptly suppressed both ACTH and prolactin levels within 2 weeks, with unexpected clinical and biochemical hypocortisolism requiring hydrocortisone replacement. Secondary hypogonadism was reversed. Clinical and biochemical remission of his Cushing's syndrome together with significant shrinkage of his macroadenoma has been maintained for 1 year on cabergoline 0.5 mg twice weekly. Reduction in pituitary tumour volume and brisk fall in serum prolactin in response to low-dose cabergoline is regularly observed in patients with macroprolactinomas, but the concurrent fall in the plasma ACTH level and hypocortisolism was a pleasant surprise. We assume that he most likely has a single bihormonal adenoma that is enriched with dopamine-2 receptors.

Citing Articles

Case report: Complete restoration of the HPA axis function in Cushing's disease with drug treatment.

Nguyen J, Ferriere A, Tabarin A Front Endocrinol (Lausanne). 2024; 15:1337741.

PMID: 38390203 PMC: 10882091. DOI: 10.3389/fendo.2024.1337741.

Targeting Aggressive Pituitary Adenomas at the Molecular Level-A Review.

Voellger B, Zhang Z, Benzel J, Wang J, Lei T, Nimsky C J Clin Med. 2022; 11(1).

PMID: 35011868 PMC: 8745122. DOI: 10.3390/jcm11010124.

Dopamine agonist-responsive Cushing's disease.

Anand G, Bink A, Beuschlein F, Schmid C BMJ Case Rep. 2019; 12(2).

PMID: 30765454 PMC: 6381956. DOI: 10.1136/bcr-2018-228045.

References

Barausse M, Attanasio R, Dallabonzana D, Oppizzi G, Veronese S, Lasio G . From macroprolactinoma to concomitant ACTH-PRL hypersecretion with Cushing's disease. J Endocrinol Invest. 2000; 23(2):107-11. DOI: 10.1007/BF03343688. View

TSjoen G, Defeyter I, Van De Saffele J, Rubens R, Vandeweghe M . Macroprolactinoma associated with Cushing's disease, successfully treated with cabergoline. J Endocrinol Invest. 2002; 25(2):172-5. DOI: 10.1007/BF03343983. View

De Rosa M, Zarrilli S, Vitale G, Di Somma C, Orio F, Tauchmanova L . Six months of treatment with cabergoline restores sexual potency in hyperprolactinemic males: an open longitudinal study monitoring nocturnal penile tumescence. J Clin Endocrinol Metab. 2004; 89(2):621-5. DOI: 10.1210/jc.2003-030852. View

Miyoshi T, Otsuka F, Takeda M, Inagaki K, Suzuki J, Ogura T . Effect of cabergoline treatment on Cushing's disease caused by aberrant adrenocorticotropin-secreting macroadenoma. J Endocrinol Invest. 2005; 27(11):1055-9. DOI: 10.1007/BF03345309. View

Mahler C, Verhelst J, Klaes R, Trouillas J . Cushing's disease and hyperprolactinemia due to a mixed ACTH- and prolactin-secreting pituitary macroadenoma. Pathol Res Pract. 1991; 187(5):598-602. DOI: 10.1016/S0344-0338(11)80153-6. View

Saeger W, Ludecke D, Buchfelder M, Fahlbusch R, Quabbe H, Petersenn S . Pathohistological classification of pituitary tumors: 10 years of experience with the German Pituitary Tumor Registry. Eur J Endocrinol. 2007; 156(2):203-16. DOI: 10.1530/eje.1.02326. View

Vilar L, Freitas M, Naves L, Casulari L, Azevedo M, Montenegro Jr R . Diagnosis and management of hyperprolactinemia: results of a Brazilian multicenter study with 1234 patients. J Endocrinol Invest. 2008; 31(5):436-44. DOI: 10.1007/BF03346388. View

Magri F, Villa C, Locatelli D, Scagnelli P, Lagonigro M, Morbini P . Prevalence of double pituitary adenomas in a surgical series: Clinical, histological and genetic features. J Endocrinol Invest. 2009; 33(5):325-31. DOI: 10.1007/BF03346594. View

Bhansali A, Walia R, Dutta P, Khandelwal N, Sialy R, Bhadada S . Efficacy of cabergoline on rapid escalation of dose in men with macroprolactinomas. Indian J Med Res. 2010; 131:530-5. View

10.

Melmed S, Casanueva F, Hoffman A, Kleinberg D, Montori V, Schlechte J . Diagnosis and treatment of hyperprolactinemia: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2011; 96(2):273-88. DOI: 10.1210/jc.2010-1692. View

11.

van der Pas R, de Herder W, Hofland L, Feelders R . New developments in the medical treatment of Cushing's syndrome. Endocr Relat Cancer. 2012; 19(6):R205-23. DOI: 10.1530/ERC-12-0191. View

12.

Pivonello R, de Leo M, Cozzolino A, Colao A . The Treatment of Cushing's Disease. Endocr Rev. 2015; 36(4):385-486. PMC: 4523083. DOI: 10.1210/er.2013-1048. View

13.

Burman P, Eden-Engstrom B, Ekman B, Anders Karlsson F, Schwarcz E, Wahlberg J . Limited value of cabergoline in Cushing's disease: a prospective study of a 6-week treatment in 20 patients. Eur J Endocrinol. 2015; 174(1):17-24. DOI: 10.1530/EJE-15-0807. View

14.

Shiraishi J, Koyama H, Shirakawa M, Ishikura R, Okazaki H, Kurajoh M . Concomitant Cushing's Disease and Marked Hyperprolactinemia: Response to a Dopamine Receptor Agonist. Intern Med. 2016; 55(8):935-41. DOI: 10.2169/internalmedicine.55.5301. View

15.

Hafiez A, Bartke A, Lloyd C . The role of prolactin in the regulation of testis function: the synergistic effects of prolactin and luteinizing hormone on the incorporation of (1- 14 C)acetate into testosterone and cholesterol by testes from hypophysectomized rats in vitro. J Endocrinol. 1972; 53(2):223-30. DOI: 10.1677/joe.0.0530223. View

16.

Sherry S, Guay A, Lee A, Hedley Whyte E, Federman M, Freidberg S . Concurrent production of adrenocorticotropin and prolactin from two distinct cell lines in a single pituitary adenoma: a detailed immunohistochemical analysis. J Clin Endocrinol Metab. 1982; 55(5):947-55. DOI: 10.1210/jcem-55-5-947. View

17.

Verde G, Loli P, Berselli M, Tagliaferri M, Dallabonzana D, Oppizzi G . Cushing's disease and marked hyperprolactinemia in a patient with a pituitary macroadenoma: effectiveness of bromocriptine treatment. J Endocrinol Invest. 1984; 7(1):51-4. DOI: 10.1007/BF03348376. View

18.

Park S, Keenan M, Selmanoff M . Graded hyperprolactinemia first suppresses LH pulse frequency and then pulse amplitude in castrated male rats. Neuroendocrinology. 1993; 58(4):448-53. DOI: 10.1159/000126575. View

19.

de Rosa M, Colao A, Di Sarno A, Ferone D, Landi M, Zarrilli S . Cabergoline treatment rapidly improves gonadal function in hyperprolactinemic males: a comparison with bromocriptine. Eur J Endocrinol. 1998; 138(3):286-93. DOI: 10.1530/eje.0.1380286. View