Abstract
Purpose
The objective of this case report is to demonstrate that the simple expedient of measuring periodic prolactin levels in patients with MEN1 who have modest hyperprolactinemia and normal pituitary MRI scans is insufficient to monitor for the development of pituitary adenomas.
Methods
Review of relevant literature and chart review.
Results
A 25 year old man with known MEN1 manifested by hyperparathyroidism and a gastrin-producing neuroendocrine tumor was found to have a prolactin [PRL] level of 20.0 ng/mL [1.6–16 ng/mL] but a normal pituitary MRI scan. The impression then was that he had prolactinoma too small to be visualized on the MRI. Over the next 3.5 years his PRL levels remained in this mildly elevated range but he then presented with severe headaches and visual field defects. An MRI showed a 3.1 × 1.7 × 1.9 cm pituitary adenoma with compression of the optic chiasm and invasion of the left cavernous sinus. Surgery revealed a gonadotroph adenoma and he subsequently required gamma knife radiotherapy for residual tumor. Postoperative PRL levels were normal.
Conclusions
Small, intrasellar microadenomas may be associated with elevated PRL levels due to possible direct hormone production [prolactinoma] or possibly to interference with portal vessel blood flow. In monitoring hyperprolactinemic MEN1 patients for the development of pituitary adenomas, measurement of PRL levels is insufficient and periodic MRI scans are necessary at a more frequent interval than every 3–5 years. This may also pertain to patients with “idiopathic” hyperprolactinemia.
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References
Thakker RV (2010) Multiple endocrine neoplasia type 1 (MEN1). Best Pract Res Clin Endocrinol Metab 24:355–370
Burgess J, Shepherd J, Parameswaran V, Hoffman L, Greenaway T (1996) Spectrum of pituitary disease in multiple endocrine neoplasia type 1 [MEN 1]: clinical, biochemical, and radiological features of pituitary disease in a large MEN 1 kindred. J Clin Endocrinol Metab 81:2642–2646
O’Brien T, O’Riordan D, Gharib H, Scheithauer BW, Ebersold M, van Heerden JA (1996) Results of treatment of pituitary disease in multiple endocrine neoplasia, type 1. Neurosurgery 39:273–279
Verges B, Boureille F, Goudet P, Murat A, Beckers A, Sassolas G, Cougard P, Chambe B, Montvernay C, Calender A, Members of the Groupe d’Etude des Neopalsmies Endocriniennes Multiples (2002) Pituitary disease in MEN type 1 [MEN1]: data from the France-Belgium MEN1 multicenter study. J Clin Endocrinol Metab 87:457–465
Hao W, Skarulis MC, Simonds WF, Weinstein LS, Agarwal SK, Mateo C, James-Newton L, Hobbs GR, Gibril F, Jensen RT, Marx SJ (2004) Multiple endocrine neoplasia type 1 variant with frequent prolactinoma and rare gastrinoma. J Clin Endocrinol Metab 89:3776–3784
Vierimaa O, Ebeling TML, Kytölä S, Bloigu R, Eloranta E, Salmi J, Korpi-Hyöväti E, Niskanen L, Orvola A, Elovaara E, Gynther A, Sane T, Välimäki M, Ignatius J, Leisti J, Salmela PI (2007) Multiple endocrine neoplasia type 1 in Northern Finland; clinical features and genotype-phenotype correlation. Eur J Endocrinol 157:285–294
De Laat JM, Dekkers OM, Pieterman CRC, Kluifjhout WP, Hermus AR, Pereira AM, van den Horst-Schrivers AN, Drent ML, Bisschop PH, Havekes B, de Herder WW, Valk GD (2015) Long-term natural course of pituitary tumors in patients with MEN1: results from the DutchMEN1 Study Group [DMSG]. J Clin Endocrinol Metab 100:3288–3296
Beckers A, Betea D, Socin HV, Stevenaert A (2003) The treatment of sporadic versus MEN1-related pituitary adenomas. J Intern Med 253:599–605
Syro LV, Scheithauer BW, Kovacs K, Toledo RA, Londoño FJ, Ortiz LD, Rotondo F, Horvath E, Uribe H (2012) Pituitary tumors in patients with MEN1 syndrome. Clinics 67(S1):43–48
Thakker RV, Newey PJ, Walls GV, Bilezikian J, Dralle H, Ebeling PR, Melmed S, Sakurai A, Tonelli F, Brandi ML (2012) Clinical practice guidelines for multiple endocrine neoplasia type 1 (MEN1). J Clin Endocrinol Metab 97:2990–3011
Gillam MP, Molitch ME (2010) Prolactin. In: Melmed S (ed) The Pituitary, 3rd edn. Elsevier Inc., San Diego, pp 119–166
Karavitaki N, Thanabalasingham G, Shore HC, Trifanescu R, Ansorge O, Meston N, Turner HE, Wass JAH (2006) Do the limits of serum prolactin in disconnection hyperprolactinaemia need re-definition? A study of 226 patients with histologically verified non-functioning pituitary macroadenoma. Clin Endocrinol 65:524–529
Serri O, Robert F, Pelletier G, Beauregard H, Hardy J (1987) Hyperprolactinemia associated with clinically silent adenomas: endocrinologic and pathologic studies; a report of two cases. Fertil Steril 47:792–796
Losa M, Mortini P, Giovanelli M (2004) A non-functioning pituitary adenoma initially mimicking a microprolactinoma: the case for long-term follow-up of patients with mild hyperprolactinemia? J Endocrinol Invest 27:367–370
Arafah BM, Prunty D, Ybarra J, Hlavin ML, Selman WR (2000) The dominant role of increased intrasellar pressure in the pathogenesis of hypopituitarism, hyperprolactinemia, and headaches in patients with pituitary adenomas. J Clin Endocrinol Metab 85:1789–1793
Pontiroli AE, Falsetti L (1984) Development of pituitary adenoma in women with hyperprolactinaemia: clinical, endocrine, and radiological characteristics. B.M.J 288:515–518
Schlechte J, Dolan K, Sherman B, Chapler F, Luciano A (1989) The natural history of untreated hyperprolactinemia: a prospective analysis. J Clin Endocrinol Metab 68:412–418
Sluijmer AV, Lappöhn RE (1992) Clinical history and outcome of 59 patients with idiopathic hyperprolactinemia. Fertil Steril 58:72–77
Melmed S, Casanueva FF, Hoffman AR, Kleinberg DL, Montori VM, Schlechte JA, Wass JAH (2011) Diagnosis and treatment of hyperprolactinemia: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab 96:273–288
Casanueva FF, Molitch ME, Schlechte JA, Abs R, Bonert V, Bronstein MD, Brue T, Cappabianca P, Colao A, Fahlbusch R, Fideleff H, Hadani M, Kelly P, Kleinberg D, Laws E, Marek J, Scanlon M, Sobrinho L, Wass J, Giustina A (2006) Guidelines of the Pituitary Society for the diagnosis and management of prolactinomas. Clin Endocrinol 65:265–273
Chuang E, Molitch M (2005) Need for periodic pituitary imaging in patients with MEN I syndrome. Presented at the 9th international pituitary congress, San Diego, June, 2005, Abstract #15. Pituitary 7 4
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AL wrote the first draft of this report and the other coauthors reviewed and revised the manuscript.
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Livshits, A., Kravarusic, J., Chuang, E. et al. Pituitary tumors in MEN1: do not be misled by borderline elevated prolactin levels. Pituitary 19, 601–604 (2016). https://doi.org/10.1007/s11102-016-0752-z
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DOI: https://doi.org/10.1007/s11102-016-0752-z