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Complete remission of Nelson’s syndrome after 1-year treatment with cabergoline

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Abstract

In this case report we demonstrated that treatment with the long-acting D2 receptor agonist cabergoline for 1 year induced normalization of plasma ACTH levels and disappearance of the pituitary tumor in a patient with Nelson’s syndrome. A young man underwent bilateral adrenalectomy and subsequent pituitary irradiation for Cushing’s disease after unsuccessful neurosurgical treatment. Thereafter, he was given cortisone acetate replacement at the dose of 62.5 mg a day. Fifteen months after pituitary irradiation, he developed Nelson’s syndrome, having skin hyperpigmentation, high plasma ACTH levels (376 ng/l) and a pituitary microadenoma (5 mm) documented at magnetic resonance imaging (MRI) of the pituitary region. After 6 months of cabergoline treatment, given at the dose of 1 mg a week, plasma ACTH levels were significantly decreased (from 376 to 113 ng/l) but they were not normalized. Cabergoline dose was then increased up to 2 mg a week. Six months later plasma ACTH levels were normalized (22 ng/I) and MRI demonstrated the disappearance of the pituitary adenoma. In order to investigate on the direct effect played by cabergoline treatment on the remission of Nelson’s syndrome, the treatment was withdrawn. Plasma ACTH levels significantly increased (119 ng/I) after 3 months of treatment withdrawal. At the last follow- up, during cabergoline treatment at the dose of 2 mg/week plasma ACTH levels were normalized (40.4 ng/I). This case demonstrated that cabergoline treatment is able to induce the remission of Nelson’s syndrome and may be a valid therapeutic alternative in this syndrome.

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References

  1. Nelson D.H., Meakin J.W., Dealy J.B., Matson D.D., Emerson K., Thorn G.W. ACTH producing tumor of the pituitary gland. N. Engl. J. Med. 1958, 259: 161–164.

    Article  CAS  PubMed  Google Scholar 

  2. Cohen K.L., Noth R.H., Pechinski T. Incidence of pituitary tumors following adrenalectomy: a long-term follow-up study of patients treated for Cushing’s disease. Arch. Intern. Med. 1978, 138: 575–579.

    Article  CAS  PubMed  Google Scholar 

  3. Aron D.C., Findling J.W., Fitzgerald P.A., Forsham P.H., Wilson C.B., Tyrrel J.B. Cushing’s syndrome: problems in management. Endocr. Rev. 1982, 3: 229–244.

    Article  CAS  PubMed  Google Scholar 

  4. Kasperlik-Zaluska A.A., Nielubowics J., Wislawski J., Hartwig W., Zaluska J., Jeske W., Migdalska B. Nelson’s syndrome: incidence and prognosis. Clin. Endocrinol. 1983, 19: 693–698.

    Article  CAS  Google Scholar 

  5. Miller J.W., Crapo L. The medical treatment of Cushing’s syndrome. Endocr. Rev. 1993, 14: 443–458.

    Article  CAS  PubMed  Google Scholar 

  6. Jones T.J., Gilman B., Beckford U. Effect of treatment with sodium valproate and diazepam on plasma corticotropin in Nelson’s syndrome. Lancet 1981, 1: 1179–1181.

    Article  CAS  PubMed  Google Scholar 

  7. Dornhorst A., Jenkins J.S., Lamberts S.W.J., Abraham R.R., Wynn V., Beckford U., Gillham B., Jones M.T. The evaluation of sodium valproate in the treatment of Nelson’s syndrome. J. Clin. Endocrinol. Metab. 1983, 56: 985–991.

    Article  CAS  PubMed  Google Scholar 

  8. Loli P., Berselli M.E., Vignati F., De Grandi C., Tagliaferri M. Size reduction of an ACTH-secreting pituitary macroadenoma in Nelson’s syndrome by sodium valporate: effect of withdrawal and re-institution of treatment. Acta Endocrinol. 1988, 119: 435–442.

    CAS  PubMed  Google Scholar 

  9. Mercado-Asis L.B., Yanovski J.A., Tracer H.L., Chik C.L., Cutler G.B. Acute effect of bromocriptine, cyproheptadine and valproic acid on plasma adrenocorticotropin secretion in Nelson’s syndrome. J. Clin. Endocrinol. Metab. 1997, 82: 514–517.

    CAS  PubMed  Google Scholar 

  10. Krieger D.T. Lack of responsiveness to L-Dopa in Cushing’s disease. J. Clin. Endocrinol. Metab. 1973, 36: 277–284.

    Article  CAS  PubMed  Google Scholar 

  11. Whitehead H.M., Beacom R., Sheridan B., Atkinson A.B. The effect of cyproheptadine and/or bromocriptine on plasma ACTH levels in patients cured of Cushing’s disease by bilateral adrenalectomy. Clin. Endocrinol. 1990, 32: 193–201.

    Article  CAS  Google Scholar 

  12. Lamberts S.W.J., Birkenhager J.C. Bromocriptine in Nelson’s syndrome and Cushing’s disease. Lancet 1976, 2: 811.

    Article  CAS  PubMed  Google Scholar 

  13. Lamberts S.W.J., Klijn J.G.M., De Quijada M., Timmermans H.A.T., Uitterlinden P., De Jong F.H., Birkenhager J.C. The mechanism of the suppressive action of bromocriptine on adrenocorticotropin secretion in patients with Cushing’s disease and Nelson’s syndrome. J. Clin. Endocrinol. Metab. 1980, 51: 307–311.

    Article  CAS  PubMed  Google Scholar 

  14. Webster J., Piscitelli G., Polli A., Ferrari C., Ismail I., ScanIon M.F., for the Cabergoline Comparative Study Group. A comparison of cabergoline and bromocriptine on plasma ACTH levels in patients cured of Cushing’s disease by bilateral adrenalectomy. N. Engl. J. Med. 1990, 331: 904–909.

    Article  Google Scholar 

  15. Ferrari C., Barbieri C., Caldara R. Long-Iasting prolactin lowering effect of cabergoline, a new dopamine-agonist, in hyperprolactinemic patients. J. Clin. Endocrinol. Metab. 1986, 63: 941–945.

    Article  CAS  PubMed  Google Scholar 

  16. Pascal-Vigneron V., Weryha G., Bose M., Leclerc J. Hyperprolactinemic amenorrhea: treatment with cabergoline versus bromocriptine. Presse Med. 1995, 24: 753–759.

    CAS  PubMed  Google Scholar 

  17. Colao A., Di Sarno A., Sarnacchiaro F., Ferone D., Di Renzo G., Merola B., Annunziato L., Lombardi G. Prolactinomas resistant to standard dopamine-agonists respond to chronic cabergoline treatment. J. Clin. Endocrinol. Metab. 1997, 82: 876–883.

    Article  CAS  PubMed  Google Scholar 

  18. Colao A., Di Sarno A., Landi M.L., Cirillo S., Sarnacchiaro F., Facciolli G., Pivonello R., Cataldi M., Merola B., Annunziato L., Lombardi G. Long term and low-dose treatment with cabergoline induces macroprolactinoma shrinkage. J. Clin. Endocrinol. Metab. 1997, 82: 3574–3579.

    Article  CAS  PubMed  Google Scholar 

  19. Oldfield E.H., Doppman J.L., Nieman L.K., Chrousos G.P., Miller D.L., Kats D.A., Cutler G.B., Loriaux D.L. Petrosal sinus sampling with and without corticotropin-releasing hormone for the differential diagnosis of Cushing’s syndrome. N. Engl. J. Med. 1991, 325: 897–905.

    Article  CAS  PubMed  Google Scholar 

  20. Jones M.T., Hillhouse E.W., Burden J. Effect of various putative neurotrasmitters on the secretion of corticotrophin-releasing hormone from the rat hypothalamus in vitro: a model of neurotrasmitters involved. J. Endocrinol. 1976, 69: 1–10.

    Article  CAS  PubMed  Google Scholar 

  21. Lamberts S.W.J., De Lange S.A., Stefanko S.Z. Adrenocorticotropin-secreting pituitary adenomas originate from the anterior or intermediate lobe in Cushing’s disease: differences in the regulation of hormone secretion. J. Clin. Endocrinol. Metab. 1982, 54: 286–291.

    Article  CAS  PubMed  Google Scholar 

  22. McNicol M., Teasdale G.M., Beastall G.H. A study of corticotroph adenomas in Cushing’s disease: no evidence of intermediate lobe origin. Clin. Endocrinol. 1986, 24: 715–722.

    Article  CAS  Google Scholar 

  23. Strolin-Benedetti M., Dostert P., Barone D., Efthymiopoulos C., Peretti G., Roncucci R. In vivo interactions of cabergoline with rat brain dopamine receptors labelled with (3H)N-n-propyInorapomorphine. Eur. J. Pharmacol. 1976, 187: 399–408.

    Article  Google Scholar 

  24. Atkinsons A.B., Chestnutt A., Crothers E., Woods R., Weaver J.A., Kennedy L., Sheridan B. Cyclical Cushing’s disease: two distinct rhythms in a patient with a basophil adenoma. J. Clin. Endocrinol. Metab. 1985, 60: 328–332.

    Article  Google Scholar 

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Correspondence to A. Colao M.D., Ph.D..

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Pivonello, R., Faggiano, A., Di Salle, F. et al. Complete remission of Nelson’s syndrome after 1-year treatment with cabergoline. J Endocrinol Invest 22, 860–865 (1999). https://doi.org/10.1007/BF03343660

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