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A giant prolactinoma presenting with unilateral exophthalmos: Effect of cabergoline and review of the literature

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Abstract

We report the case of a 45-year-old male presenting with unilateral exophthalmos due to a large tumoral mass invading the skull base. Ophthalmologic examination did not show any visual field defects. Imaging techniques demonstrated extension of a huge tumor (approx. 8×8×8 cm) into the right orbit and nasopharynx. Endocrine work-up revealed grossly elevated serum prolactin (PRL) levels (26,466 μg/l, nl. <12), pointing to a large, invasive macroprolactinoma. Stimulation tests indicated associated partial adrenal and growth hormone deficiencies. Planned surgery was abandoned, and the patient was instead treated with the long-acting dopamine agonist, cabergoline. Over a period of one year, serum PRL dropped to 131 μg/l, while the tumor mass shrank to less than 50% of its original volume (with 3.5 mg/ week of cabergoline). The exophthalmos disappeared, and the patient did not develop rhinorrhea or any other side effects from treatment with cabergoline. The efficacy was maintained throughout the second year (ultimate serum PRL 74 μg/l, and final size less than 10% of the original). With reference to this case, we review other macroprolactinomas reported in the recent literature for associated exophthalmos, grossly elevated serum PRL levels (≥15,000 μg/l), and/or “giant” size (≥4 cm in maximum diameter). We highlight the use of dopamine agonists in the treatment of prolactinomas with such unusual characteristics.

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References

  1. Verhelst J., Abs R., Maiter D., van den Bruel A., Vandeweghe M., Velkeniers B., Mockel J., Lamberigts G., Petrossians P., Coremans P., Mahler C., Stevenaert A., Verlooy J., Raftopoulos C., Beckers A. Cabergoline in the treatment of hyperprolactinemia: a study in 455 patients. J. Clin. Endocrinol. Metab. 1999, 84: 2518–2522.

    Article  CAS  PubMed  Google Scholar 

  2. Murphy F.Y., Vesely D.L., Jordan R.M., Flanigan S., Kohler P.O. Giant invasive prolactinomas. Am. J. Med. 1987, 83: 995–1002.

    Article  CAS  PubMed  Google Scholar 

  3. Davis J.R.E., Sheppard M.C., Heath D.A. Giant invasive prolactinoma: a case report and review of nine further cases. Q. J. Med. 1990, 74: 227–238.

    CAS  PubMed  Google Scholar 

  4. Grebe S.K.G., Delahunt J.W., Feek C.M. Treatment of extensively invasive (giant) prolactinomas with bromocriptine. N. Z. Med. J. 1992, 105: 129–131.

    CAS  PubMed  Google Scholar 

  5. Kolodny J., Dluhy R.G. Recurrent prolactinoma and meningioma following irradiation and bromocriptine treatment. Am. J. Med. 1985, 78: 153–155.

    Article  CAS  PubMed  Google Scholar 

  6. Ross R.J.M., McEniery J.M., Grossman A., Doniach I., Besser G.M., Savage M.O. Massive prolactinoma with galactorrhea in a prepubertal boy. Postgrad. Med. J. 1989, 65: 403–406.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  7. Angyal E.A., Lee H-J., Wolansky L.J., Koenigsberger M.R., Nathanson D., Zimmer A.E. Prolactinoma invasion of superior ophthalmic vein: CT and MR findings. J. Comput. Assist. Tomogr. 1993, 17: 964–966.

    Article  CAS  PubMed  Google Scholar 

  8. Levy R.A., Quint D.J. Giant pituitary adenoma with unusual orbital and skull base extension. Am. J. Rad. 1998, 170: 194–196.

    CAS  Google Scholar 

  9. Barkan A.L., Chandler W.F. Giant pituitary prolactinoma with falsely low serum prolactin: the pitfall of the “high-dose hook effect”, case report. Neurosurgery 1998, 42: 913–916.

    Article  CAS  PubMed  Google Scholar 

  10. Daita G., Yonemasu Y., Hashizume A. Unilateral exophthalmos caused by an invasive pituitary adenoma. Neurosurgery 1987, 21: 716–718.

    Article  CAS  PubMed  Google Scholar 

  11. Landolt A.M. Cerebrospinal fluid rhinorrhea: a complication of therapy for invasive prolactinomas. Neurosurgery 1982, 11: 395–401.

    Article  CAS  PubMed  Google Scholar 

  12. Clayton R.N., Webb J., Heath D.A., Dunn P.J., Rolfe E.B., Hockley A.D. Dramatic and rapid shrinkage of a massive invasive prolactinoma with bromocriptine: a case report. Clin. Endocrinol. (Oxf.) 1985, 22: 573–581.

    Article  CAS  Google Scholar 

  13. Scherrer H., Turpin G., de Gennes J-L., Schaison-Cusin M., Heshmati H.M., Thibierge M., Metzger J. Résultats du traitement par bromocriptine des adénomes à prolactine géants ou expansifs. Ann. Méd. Interne (Paris) 1985, 136: 459–466.

    CAS  Google Scholar 

  14. Barrera C.M., Ruiz A., Banks W.A. A giant prolactinoma and the effect of chronic bromocriptine therapy on basal and TRH-stimulated serum prolactin levels. Horm. Res. 1991, 35: 167–169.

    Article  CAS  PubMed  Google Scholar 

  15. van der Lely A-J., Knegt P.P.M., Stefanko S.Z., Tanghe H.L., Singh R., Lamberts S.W. Nasopharyngeal presentation of pituitary tumors: differential diagnosis and treatment. J. Clin. Endocrinol. Metab. 1992, 74: 811–813.

    Article  PubMed  Google Scholar 

  16. Reuter U., Mehraein S., Arnold G., Lehmann R.A. A “giant” prolactinoma. J. Neurol. Neurosurg. Psychiatry. 1997, 63: 295.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  17. Saeki N., Nakamura M., Sunami K., Yamaura A. Surgical indication after bromocriptine therapy on giant prolactinomas: effects and limitations of the medical treatment. Endocr. J. 1998, 45: 529–537.

    Article  CAS  PubMed  Google Scholar 

  18. Cannavò S., Bartolone L., Blandino A., Spinella S., Galatioto S., Trimarchi F. Shrinkage of a PRL-secreting pituitary macroadenoma resistant to cabergoline. J. Endocrinol. Invest. 1999, 22: 306–309.

    PubMed  Google Scholar 

  19. Symon L., Jakubowski J., Kendall B. Surgical treatment of giant pituitary adenomas. J. Neurol. Neurosurg. Psychiatry 1979, 42: 973–982.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  20. Pia H.W., Grote E., Hildebrandt G. Giant pituitary adenomas. Neurosurg. Rev. 1985, 8: 207–220.

    Article  CAS  PubMed  Google Scholar 

  21. Majós C., Coll S., Aguilera C., Acebes J.J., Pons L.C. Imaging of giant pituitary adenomas. Neuroradiology 1998, 40: 651–655.

    Article  PubMed  Google Scholar 

  22. Perani D., Colombo N., Scotti G., Tonon C. Rapid size reduction of giant prolactinoma following medical treatment. J. Comput. Assist. Tomogr. 1984, 8: 131–133.

    Article  CAS  PubMed  Google Scholar 

  23. Iwai Y., Hakuba A., Khosla V.K., Nishikawa M., Katsuyama J., Inoue Y., Nishimura S. Giant basal prolactinoma extending into the nasal cavity. Surg. Neurol. 1992, 37: 280–283.

    Article  CAS  PubMed  Google Scholar 

  24. Cook R.J., Uttley D., Wilkins P.R., Archer D.J., Bell B.A. Prolactinomas in men masquerading as invasive skull base tumours. Br. J. Neurosurg. 1994, 8: 51–55.

    Article  PubMed  Google Scholar 

  25. Cole D.R., Lees P.D., Armitage M. A good wife, but never a mother... J.R. Soc. Med. 1995, 88: 176P–178P.

    CAS  PubMed Central  PubMed  Google Scholar 

  26. Biller B.M.K., Molitch M.E., Vance M.L., Baker Cannistraro K., Davis K.R., Simons J.A., Schoenfelder J.R., Klibanski A. Treatment of prolactin-secreting macroadenomas with the once-weekly dopamine agonist cabergoline. J. Clin. Endocrinol. Metab. 1996, 81: 2338–2343.

    CAS  PubMed  Google Scholar 

  27. Holness R.O., Schlossberg A.H., Heffernan L.P.M. Cerebrospinal fluid rhinorrhea caused by bromocriptine therapy of prolactinoma. Neurology 1984, 34: 111–113.

    Article  CAS  PubMed  Google Scholar 

  28. Hildebrandt G., Zierski J., Christophis P., Laun A., Schatz H., Lancranjan I., Klug N. Rhinorrhea following dopamine agonist therapy of invasive macroprolactinoma. Acta Neurochir. (Wien) 1989, 96: 107–113.

    Article  CAS  Google Scholar 

  29. Bronstein M.D., Musolino N.R., Benabou S., Marino R. Cerebrospinal fluid rhinorrhea occurring in long-term bromocriptine treatment of macro-prolactinomas. Surg. Neurol. 1989, 32: 246–249.

    Article  Google Scholar 

  30. Pascal-Vigneron V., Weryha G., Braun M., Morel-Jean J., Bisset S., Leclère J. La rhinorrhée et l’otorrhée: des complications rares du traitement médical des prolactinomes invasifs. Ann. Endocrinol. (Paris). 1993, 54: 347–351.

    Google Scholar 

  31. Ferrari C.I., Abs R., Bevan J.S., Brabant G., Ciccarelli E., Motta T., Mucci M., Muratori M., Musatti L., Verbessem G., Scanlon M.F. Treatment of macroprolactinoma with cabergoline: a study of 85 patients. Clin. Endocrinol. (Oxf.) 1997, 46: 409–413.

    Article  CAS  Google Scholar 

  32. 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 

  33. Bevan J.S., Webster J., Burke C.W., Scanlon M.F. Dopamine agonists and pituitary tumor shrinkage. Endocr. Rev. 1992, 13: 220–240.

    Article  CAS  PubMed  Google Scholar 

  34. Cannavò S., Curtò L., Squadrito S., Almoto B., Vieni A., Trimarchi F. Cabergoline: a first-choice treatment in patient with previously untreated prolactin-secreting pituitary adenoma. J. Endocrinol. Invest. 1999, 22: 354–359.

    PubMed  Google Scholar 

  35. Rains C.P., Bryson H.M., Fitton A. Cabergoline: a review of its pharmacological properties and therapeutic potential in the treatment of hyperprolactinaemia and inhibition of lactation. Drugs 1995, 49: 255–279.

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Neuroradiology, Middelheim Hospital, Belgium

    R. Abs

  2. Department of Endocrinology, University Hospital, Antwerp, Belgium

    Joris Berwaerts M.D., J. Verhelst & C. Mahler

  3. SmithKline Beecham ACCI - CRU, Addenbrooke’s Hospital, Hills Road, UK-Cambridge, CB2 2GG, UK

    B. Appel

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  1. Joris Berwaerts M.D.
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  2. J. Verhelst
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  3. R. Abs
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  4. B. Appel
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  5. C. Mahler
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Correspondence to Joris Berwaerts M.D..

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Berwaerts, J., Verhelst, J., Abs, R. et al. A giant prolactinoma presenting with unilateral exophthalmos: Effect of cabergoline and review of the literature. J Endocrinol Invest 23, 393–398 (2000). https://doi.org/10.1007/BF03343743

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