Pituitary adenoma in patients with multiple endocrine neoplasia type 1: a cohort study

in European Journal of Endocrinology
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  • 1 CHU de Nantes PHU2 Institut du Thorax et du Système Nerveux, Service d’Endocrinologie, Diabétologie et Nutrition, Nantes, France
  • | 2 Centre Hospitalier de La Roche sur Yon, Department of Endocrinology and Diabetology, La Roche sur Yon, France
  • | 3 INSERM CIC 1413, Clinique des Données, CHU de Nantes, Nantes, France
  • | 4 University Hospital Dijon, Endocrine Surgery, Hôpital du Bocage, Dijon, France
  • | 5 Department of Endocrinology, Hopital de la Conception, Marseille, France
  • | 6 Aix-Marseille Université, Assistance-Publique-Hôpitaux de Marseille, Centre de Référence des Maladies Rares d’Origine Hypophysaire, Department of endocrinology, Marseille, France
  • | 7 CHU Lille, Service d’Endocrinologie, Lille, France
  • | 8 Assistance Publique-Hôpitaux de Paris, Endocrinology, Le Kremlin-Bicêtre, Île-de-France, France
  • | 9 Department of Endocrinology, Hôpital Haut Lévêque-CHU de Bordeaux, Pessac, France
  • | 10 Hopital Cochin, Digestive and Endocrine Surgery, Paris, France
  • | 11 Hopital Saint-Antoine, Endocrine Unit, Paris, France
  • | 12 Department of Gastroenterology and Pancreatology, Université Paris Diderot, Sorbonne Paris Cité, Beaujon Hospital, Paris Clichy, France
  • | 13 Hospices Civils de Lyon, Fédération d’Endocrinologie Groupement Hospitalier Est, Lyon, France
  • | 14 Centre Hospitalier Universitaire de Rennes, Hôpital Sud, Service d’Endocrinologie-Diabétologie-Nutrition, Rennes, France
  • | 15 CHU Toulouse, Endocrinology and Metabolic diseases, Service d’Endocrinologie, Toulouse, France
  • | 16 Hôpital de Hautepierre, Service de Medecine Interne et Nutrition, Strasbourg Cedex, France
  • | 17 CHU de Liège, Service d’Endocrinologie, Domaine Univeritaire du Sart-Tilman, Liège, Belgium
  • | 18 Hôpital Robert-Debré, CHU de Reims, Service Endocrinologie, Reims, France
  • | 19 University Hospital of Montpellier, Endocrinology, Montpellier, France
  • | 20 CHU Dijon, Hôpital du Bocage, Endocrinologie, Diabétologie, Dijon, France
  • | 21 UNIV Nantes, CNRS, INSERM, CHU Nantes, l’Institut du Thorax, Nantes, France

Correspondence should be addressed to M Le Bras Email maelle.lebras@chu-nantes.fr

(B Cariou and S Hadjadj contributed equally to this work)

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Objective

Pituitary adenoma (PA) is one of the three major components of multiple endocrine neoplasia type 1 (MEN1). Recent studies have suggested that MEN1-associated PAs are less aggressive than initially estimated. We propose an analysis of the outcome of PAs with a standard of care treatment in a nationwide cohort of MEN1 patients.

Design

Retrospective observational nationwide cohort study using the MEN1 patient registry from the French Group of Endocrine Tumours (GTE).

Methods

The GTE database population consists of 1435 patients with MEN1. This analysis focused on 551 patients recruited after 2000 with at least 3 years of follow-up. The study outcome was tumour progression of PA defined by an increase in Hardy classification (HC) during follow-up according to referring physician regular reports.

Results

Among 551 MEN1 patients (index and related), 202 (36.7%) had PA, with 114 (56.4%) diagnosed by MEN1-related screening. PAs were defined according to HC as microadenoma (grade I) in 117 cases (57.9%), macroadenoma in 59 (29.2%) with 20 HC grade II and 39 HC grades III–IV and unspecified in 26 (12.8%). They were prolactinomas in 92 cases (45.5%) and non-secreting in 73 (36.1%). After a median follow-up of 3 years among the 137 patients with HC grades I–II, 4 patients (2.9%) presented tumour progression.

Conclusion

PAs in patients with MEN1 are less aggressive than previously thought. Tumour progression is rare with a standard of care monitoring and treatment, especially in related patients who mostly present non-secreting microadenoma. MRI monitoring for asymptomatic MEN1 patients should be reduced accordingly.

 

     European Society of Endocrinology

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  • 1

    Brandi ML, Gagel RF, Angeli A, Bilezikian JP, Beck-Peccoz P, Bordi C, Conte-Devolx B, Falchetti A, Gheri RG & Libroia A et al.Guidelines for diagnosis and therapy of MEN type 1 and type 2. Journal of Clinical Endocrinology and Metabolism 2001 86 56585671. (https://doi.org/10.1210/jcem.86.12.8070)

    • Search Google Scholar
    • Export Citation
  • 2

    Ballard HS, Frame B, Hartsock RJ. Familial multiple endocrine adenoma-peptic ulcer complex. Medicine 1964 43 281283; discussion 283. (https://doi.org/10.1097/00005792-196407000-00003)

    • Search Google Scholar
    • Export Citation
  • 3

    Stratakis CA, Schussheim DH, Freedman SM, Keil MF, Pack SD, Agarwal SK, Skarulis MC, Weil RJ, Lubensky IA & Zhuang Z et al.Pituitary macroadenoma in a 5-year-old: an early expression of multiple endocrine neoplasia Type 1. Journal of Clinical Endocrinology and Metabolism 2000 85 47764780. (https://doi.org/10.1210/jcem.85.12.7064)

    • Search Google Scholar
    • Export Citation
  • 4

    Vannucci L, Marini F, Giusti F, Ciuffi S, Tonelli F, Brandi ML. MEN1 in children and adolescents: data from patients of a regional referral center for hereditary endocrine tumors. Endocrine 2018 59 438448. (https://doi.org/10.1007/s12020-017-1322-5)

    • Search Google Scholar
    • Export Citation
  • 5

    Vergès B, Boureille F, Goudet P, Murat A, Beckers A, Sassolas G, Cougard P, Chambe B, Montvernay C, Calender A. Pituitary disease in MEN type 1 (MEN1): data from the France-Belgium MEN1 multicenter study. Journal of Clinical Endocrinology and Metabolism 2002 87 457465. (https://doi.org/10.1210/jcem.87.2.8145)

    • Search Google Scholar
    • Export Citation
  • 6

    Trouillas J, Labat-Moleur F, Sturm N, Kujas M, Heymann MF, Figarella-Branger D, Patey M, Mazucca M, Decullier E & Vergès B et al.Pituitary tumors and hyperplasia in multiple endocrine neoplasia type 1 syndrome (MEN1): a case-control study in a series of 77 patients versus 2509 non-MEN1 patients. American Journal of Surgical Pathology 2008 32 534543. (https://doi.org/10.1097/PAS.0b013e31815ade45)

    • Search Google Scholar
    • Export Citation
  • 7

    Freda PU, Beckers AM, Katznelson L, Molitch ME, Montori VM, Post KD, Lee Vance ML & Endocrine Society. Pituitary incidentaloma: an endocrine society clinical practice guideline. Journal of Clinical Endocrinology and Metabolism 2011 96 894904. (https://doi.org/10.1210/jc.2010-1048)

    • Search Google Scholar
    • Export Citation
  • 8

    Thakker RV, Newey PJ, Walls GV, Bilezikian J, Dralle H, Ebeling PR, Melmed S, Sakurai A, Tonelli F & Brandi ML et al.Clinical practice guidelines for multiple endocrine neoplasia type 1 (MEN1). Journal of Clinical Endocrinology and Metabolism 2012 97 29903011. (https://doi.org/10.1210/jc.2012-1230)

    • Search Google Scholar
    • Export Citation
  • 9

    Goudet P, Dalac A, Bras Le M, Cardot-Bauters C, Niccoli P, Lévy-Bohbot N, Boullay Du H, Bertagna X, Ruszniewski P & Borson-Chazot F et al.MEN1 disease occurring before 21 years old: a 160-patient cohort study from the Groupe d’étude des Tumeurs Endocrines. Journal of Clinical Endocrinology and Metabolism 2015 100 15681577. (https://doi.org/10.1210/jc.2014-3659)

    • Search Google Scholar
    • Export Citation
  • 10

    Laat De JM, Dekkers OM, Pieterman CRC, Kluijfhout WP, Hermus AR, Pereira AM, Horst-Schrivers Van Der AN, Drent ML, Bisschop PH & Havekes B et al.Long-term natural course of pituitary tumors in patients with MEN1: results from the Dutch MEN1 study group (DMSG). Journal of Clinical Endocrinology and Metabolism 2015 100 32883296. (https://doi.org/10.1210/JC.2015-2015)

    • Search Google Scholar
    • Export Citation
  • 11

    Cuny T, Pertuit M, Sahnoun-Fathallah M, Daly A, Occhi G, Odou MF, Tabarin A, Nunes ML, Delemer B & Rohmer V et al.Genetic analysis in young patients with sporadic pituitary macroadenomas: besides AIP don’t forget MEN1 genetic analysis. European Journal of Endocrinology 2013 168 533541. (https://doi.org/10.1530/EJE-12-0763)

    • Search Google Scholar
    • Export Citation
  • 12

    Hardy J, Vezina JL. Transsphenoidal neurosurgery of intracranial neoplasm. Advances in Neurology 1976 15 261273.

  • 13

    Syro LV, Scheithauer BW, Kovacs K, Toledo RA, Londoño FJ, Ortiz LD, Rotondo F, Horvath E, Uribe H. Pituitary tumors in patients with MEN1 syndrome. Clinics 2012 67 (Supplement 1) 4348. (https://doi.org/10.6061/clinics/2012(Sup0109)

    • Search Google Scholar
    • Export Citation
  • 14

    Farid NR, Buehler S, Russell NA, Maroun FB, Allerdice P, Smyth HS. Prolactinomas in familial multiple endocrine neoplasia syndrome type I. Relationship to HLA and carcinoid tumors. American Journal of Medicine 1980 69 874880. (https://doi.org/10.1016/S0002-9343(8080013-1)

    • Search Google Scholar
    • Export Citation
  • 15

    Burgess JR, Shepherd JJ, Parameswaran V, Hoffman L, Greenaway TM. Prolactinomas in a large kindred with multiple endocrine neoplasia type 1: clinical features and inheritance pattern. Journal of Clinical Endocrinology and Metabolism 1996 81 18411845. (https://doi.org/10.1210/jcem.81.5.8626844)

    • Search Google Scholar
    • Export Citation
  • 16

    Lourenço DM, Toledo RA, Mackowiak II, Coutinho FL, Cavalcanti MG, Correia-Deur JEM, Montenegro F, Siqueira SAC, Margarido LC & Machado MC et al.Multiple endocrine neoplasia type 1 in Brazil: MEN1 founding mutation, clinical features, and bone mineral density profile. European Journal of Endocrinology 2008 159 259274. (https://doi.org/10.1530/EJE-08-0153)

    • Search Google Scholar
    • Export Citation
  • 17

    Leeuwaarde Van RS, Pieterman CRC, Bleiker EMA, Dekkers OM, Horst-Schrivers Van Der AN, Hermus AR, Herder De WW, Drent ML, Bisschop PH & Havekes B et al.High fear of disease occurrence is associated with low quality of life in patients with multiple endocrine neoplasia Type 1: results from the Dutch MEN1 study group. Journal of Clinical Endocrinology and Metabolism 2018 103 23542361 (https://doi.org/10.1210/jc.2018-00259)

    • Search Google Scholar
    • Export Citation
  • 18

    Asgharian B, Chen YJ, Patronas NJ, Peghini PL, Reynolds JC, Vortmeyer A, Zhuang Z, Venzon DJ, Gibril F, Jensen RT. Meningiomas may be a component tumor of multiple endocrine neoplasia Type 1. Clinical Cancer Research 2004 10 869880. (https://doi.org/10.1158/1078-0432.CCR-0938-3)

    • Search Google Scholar
    • Export Citation
  • 19

    Zhu H, Miao Y, Shen Y, Guo J, Xie W, Zhao S, Dong W, Zhang Y, Li C. Germline mutations in MEN1 are associated with the tumorigenesis of pituitary adenoma associated with meningioma. Oncology Letters 2020 20 561568. (https://doi.org/10.3892/ol.2020.11601)

    • Search Google Scholar
    • Export Citation