title: Long-Term Natural Course of Pituitary Tumors in Patients With MEN1: Results From the DutchMEN1 Study Group (DMSG).
abstract: BACKGROUND
  Guidelines advise lifelong radiological followup for asymptomatic pituitary adenomas (PITs) because of the risk for growth and subsequent visual field defects. In the context of multiple endocrine neoplasia type 1 (MEN1) an even more comprehensive screening is advised because PITs are presumed to manifest more aggressive behavior. We studied the long-term course of MEN1-related PITs, which may be used as a model for sporadically occurring PITs.
  
  
  OBJECTIVE
  The aim of our study is to assess the results of systematic pre-symptomatic PIT screening and subsequent long-term followup of PITs with emphasis on nonfunctioning microadenomas diagnosed by screening.
  
  
  METHODS
  A cohort study was performed using the Dutch national MEN1 database, including greater than 90% of the Dutch MEN1 population older than 16 years (n = 323).
  
  
  METHODS
  Screening results, natural course, and effects of treatment of PIT were assessed.
  
  
  RESULTS
  PIT was diagnosed in 123 patients with MEN1 (38.1 %), of whom 66 were diagnosed by MEN1-related screening. Ninety-one percent of the nonfunctioning PIT detected during screening (n = 35), did not require intervention during followup (median, 6.0 y). Three microadenomas showed limited growth but did not progress toward macroadenomas. Both screening-detected and prevalent prolactinomas (n = 52) responded well to treatment with dopamine agonists.
  
  
  CONCLUSIONS
  Systematic presymptomatic screening for PIT in patients with MEN1 predominantly results in detection of nonfunctioning microadenomas. Prolactinoma in patients with MEN1 responded well to medical treatment. Microadenomas grew only occasionally and after many years without clinical consequences. Frequent magnetic resonance imaging followup of nonfunctioning microadenomas in the context of MEN1 and sporadically occurring PITs therefore seems debatable.
authors.affiliation: Department of Endocrine Oncology (J.M.d.L., C.R.C.P., W.P.K., G.D.V.), University Medical Center Utrecht, 3508 GA Utrecht, The Netherlands; Department of Endocrinology and Metabolism (O.M.D., A.M.P.), Leiden University Medical Center, 2300 RC Leiden, The Netherlands; Department of Endocrinology (A.R.H.), Radboud University Medical Center, 6500 HB Nijmegen, The Netherlands Department of Endocrinology (A.N.v.d.H.-S.), University Medical Center Groningen, 9700 RB Groningen, The Netherlands; Department of Internal Medicine, Section Endocrinology (M.L.D.), VU University Medical Center, 1007 MB Amsterdam, The Netherlands; Department of Endocrinology and Metabolism (P.H.B.), Academic Medical Center, 1100 DD Amsterdam Zuidoost, The Netherlands; Department of Internal Medicine, Division of Endocrinology (B.H.), Maastricht University Medical Center, 6202 AZ Maastricht, The Netherlands; and Department of Internal Medicine (W.W.d.H.), Erasmus Medical Center, 3000 CA Rotterdam, The Netherlands.
authors.forename: Joanne M
authors.initials: JM
authors.lastname: de Laat
authors.affiliation: Department of Endocrine Oncology (J.M.d.L., C.R.C.P., W.P.K., G.D.V.), University Medical Center Utrecht, 3508 GA Utrecht, The Netherlands; Department of Endocrinology and Metabolism (O.M.D., A.M.P.), Leiden University Medical Center, 2300 RC Leiden, The Netherlands; Department of Endocrinology (A.R.H.), Radboud University Medical Center, 6500 HB Nijmegen, The Netherlands Department of Endocrinology (A.N.v.d.H.-S.), University Medical Center Groningen, 9700 RB Groningen, The Netherlands; Department of Internal Medicine, Section Endocrinology (M.L.D.), VU University Medical Center, 1007 MB Amsterdam, The Netherlands; Department of Endocrinology and Metabolism (P.H.B.), Academic Medical Center, 1100 DD Amsterdam Zuidoost, The Netherlands; Department of Internal Medicine, Division of Endocrinology (B.H.), Maastricht University Medical Center, 6202 AZ Maastricht, The Netherlands; and Department of Internal Medicine (W.W.d.H.), Erasmus Medical Center, 3000 CA Rotterdam, The Netherlands.
authors.forename: Olaf M
authors.initials: OM
authors.lastname: Dekkers
authors.affiliation: Department of Endocrine Oncology (J.M.d.L., C.R.C.P., W.P.K., G.D.V.), University Medical Center Utrecht, 3508 GA Utrecht, The Netherlands; Department of Endocrinology and Metabolism (O.M.D., A.M.P.), Leiden University Medical Center, 2300 RC Leiden, The Netherlands; Department of Endocrinology (A.R.H.), Radboud University Medical Center, 6500 HB Nijmegen, The Netherlands Department of Endocrinology (A.N.v.d.H.-S.), University Medical Center Groningen, 9700 RB Groningen, The Netherlands; Department of Internal Medicine, Section Endocrinology (M.L.D.), VU University Medical Center, 1007 MB Amsterdam, The Netherlands; Department of Endocrinology and Metabolism (P.H.B.), Academic Medical Center, 1100 DD Amsterdam Zuidoost, The Netherlands; Department of Internal Medicine, Division of Endocrinology (B.H.), Maastricht University Medical Center, 6202 AZ Maastricht, The Netherlands; and Department of Internal Medicine (W.W.d.H.), Erasmus Medical Center, 3000 CA Rotterdam, The Netherlands.
authors.forename: Carolina R C
authors.initials: CR
authors.lastname: Pieterman
authors.affiliation: Department of Endocrine Oncology (J.M.d.L., C.R.C.P., W.P.K., G.D.V.), University Medical Center Utrecht, 3508 GA Utrecht, The Netherlands; Department of Endocrinology and Metabolism (O.M.D., A.M.P.), Leiden University Medical Center, 2300 RC Leiden, The Netherlands; Department of Endocrinology (A.R.H.), Radboud University Medical Center, 6500 HB Nijmegen, The Netherlands Department of Endocrinology (A.N.v.d.H.-S.), University Medical Center Groningen, 9700 RB Groningen, The Netherlands; Department of Internal Medicine, Section Endocrinology (M.L.D.), VU University Medical Center, 1007 MB Amsterdam, The Netherlands; Department of Endocrinology and Metabolism (P.H.B.), Academic Medical Center, 1100 DD Amsterdam Zuidoost, The Netherlands; Department of Internal Medicine, Division of Endocrinology (B.H.), Maastricht University Medical Center, 6202 AZ Maastricht, The Netherlands; and Department of Internal Medicine (W.W.d.H.), Erasmus Medical Center, 3000 CA Rotterdam, The Netherlands.
authors.forename: Wouter P
authors.initials: WP
authors.lastname: Kluijfhout
authors.affiliation: Department of Endocrine Oncology (J.M.d.L., C.R.C.P., W.P.K., G.D.V.), University Medical Center Utrecht, 3508 GA Utrecht, The Netherlands; Department of Endocrinology and Metabolism (O.M.D., A.M.P.), Leiden University Medical Center, 2300 RC Leiden, The Netherlands; Department of Endocrinology (A.R.H.), Radboud University Medical Center, 6500 HB Nijmegen, The Netherlands Department of Endocrinology (A.N.v.d.H.-S.), University Medical Center Groningen, 9700 RB Groningen, The Netherlands; Department of Internal Medicine, Section Endocrinology (M.L.D.), VU University Medical Center, 1007 MB Amsterdam, The Netherlands; Department of Endocrinology and Metabolism (P.H.B.), Academic Medical Center, 1100 DD Amsterdam Zuidoost, The Netherlands; Department of Internal Medicine, Division of Endocrinology (B.H.), Maastricht University Medical Center, 6202 AZ Maastricht, The Netherlands; and Department of Internal Medicine (W.W.d.H.), Erasmus Medical Center, 3000 CA Rotterdam, The Netherlands.
authors.forename: Ad R
authors.initials: AR
authors.lastname: Hermus
authors.affiliation: Department of Endocrine Oncology (J.M.d.L., C.R.C.P., W.P.K., G.D.V.), University Medical Center Utrecht, 3508 GA Utrecht, The Netherlands; Department of Endocrinology and Metabolism (O.M.D., A.M.P.), Leiden University Medical Center, 2300 RC Leiden, The Netherlands; Department of Endocrinology (A.R.H.), Radboud University Medical Center, 6500 HB Nijmegen, The Netherlands Department of Endocrinology (A.N.v.d.H.-S.), University Medical Center Groningen, 9700 RB Groningen, The Netherlands; Department of Internal Medicine, Section Endocrinology (M.L.D.), VU University Medical Center, 1007 MB Amsterdam, The Netherlands; Department of Endocrinology and Metabolism (P.H.B.), Academic Medical Center, 1100 DD Amsterdam Zuidoost, The Netherlands; Department of Internal Medicine, Division of Endocrinology (B.H.), Maastricht University Medical Center, 6202 AZ Maastricht, The Netherlands; and Department of Internal Medicine (W.W.d.H.), Erasmus Medical Center, 3000 CA Rotterdam, The Netherlands.
authors.forename: Alberto M
authors.initials: AM
authors.lastname: Pereira
authors.affiliation: Department of Endocrine Oncology (J.M.d.L., C.R.C.P., W.P.K., G.D.V.), University Medical Center Utrecht, 3508 GA Utrecht, The Netherlands; Department of Endocrinology and Metabolism (O.M.D., A.M.P.), Leiden University Medical Center, 2300 RC Leiden, The Netherlands; Department of Endocrinology (A.R.H.), Radboud University Medical Center, 6500 HB Nijmegen, The Netherlands Department of Endocrinology (A.N.v.d.H.-S.), University Medical Center Groningen, 9700 RB Groningen, The Netherlands; Department of Internal Medicine, Section Endocrinology (M.L.D.), VU University Medical Center, 1007 MB Amsterdam, The Netherlands; Department of Endocrinology and Metabolism (P.H.B.), Academic Medical Center, 1100 DD Amsterdam Zuidoost, The Netherlands; Department of Internal Medicine, Division of Endocrinology (B.H.), Maastricht University Medical Center, 6202 AZ Maastricht, The Netherlands; and Department of Internal Medicine (W.W.d.H.), Erasmus Medical Center, 3000 CA Rotterdam, The Netherlands.
authors.forename: Anouk N
authors.initials: AN
authors.lastname: van der Horst-Schrivers
authors.affiliation: Department of Endocrine Oncology (J.M.d.L., C.R.C.P., W.P.K., G.D.V.), University Medical Center Utrecht, 3508 GA Utrecht, The Netherlands; Department of Endocrinology and Metabolism (O.M.D., A.M.P.), Leiden University Medical Center, 2300 RC Leiden, The Netherlands; Department of Endocrinology (A.R.H.), Radboud University Medical Center, 6500 HB Nijmegen, The Netherlands Department of Endocrinology (A.N.v.d.H.-S.), University Medical Center Groningen, 9700 RB Groningen, The Netherlands; Department of Internal Medicine, Section Endocrinology (M.L.D.), VU University Medical Center, 1007 MB Amsterdam, The Netherlands; Department of Endocrinology and Metabolism (P.H.B.), Academic Medical Center, 1100 DD Amsterdam Zuidoost, The Netherlands; Department of Internal Medicine, Division of Endocrinology (B.H.), Maastricht University Medical Center, 6202 AZ Maastricht, The Netherlands; and Department of Internal Medicine (W.W.d.H.), Erasmus Medical Center, 3000 CA Rotterdam, The Netherlands.
authors.forename: Madeleine L
authors.initials: ML
authors.lastname: Drent
authors.affiliation: Department of Endocrine Oncology (J.M.d.L., C.R.C.P., W.P.K., G.D.V.), University Medical Center Utrecht, 3508 GA Utrecht, The Netherlands; Department of Endocrinology and Metabolism (O.M.D., A.M.P.), Leiden University Medical Center, 2300 RC Leiden, The Netherlands; Department of Endocrinology (A.R.H.), Radboud University Medical Center, 6500 HB Nijmegen, The Netherlands Department of Endocrinology (A.N.v.d.H.-S.), University Medical Center Groningen, 9700 RB Groningen, The Netherlands; Department of Internal Medicine, Section Endocrinology (M.L.D.), VU University Medical Center, 1007 MB Amsterdam, The Netherlands; Department of Endocrinology and Metabolism (P.H.B.), Academic Medical Center, 1100 DD Amsterdam Zuidoost, The Netherlands; Department of Internal Medicine, Division of Endocrinology (B.H.), Maastricht University Medical Center, 6202 AZ Maastricht, The Netherlands; and Department of Internal Medicine (W.W.d.H.), Erasmus Medical Center, 3000 CA Rotterdam, The Netherlands.
authors.forename: Peter H
authors.initials: PH
authors.lastname: Bisschop
authors.affiliation: Department of Endocrine Oncology (J.M.d.L., C.R.C.P., W.P.K., G.D.V.), University Medical Center Utrecht, 3508 GA Utrecht, The Netherlands; Department of Endocrinology and Metabolism (O.M.D., A.M.P.), Leiden University Medical Center, 2300 RC Leiden, The Netherlands; Department of Endocrinology (A.R.H.), Radboud University Medical Center, 6500 HB Nijmegen, The Netherlands Department of Endocrinology (A.N.v.d.H.-S.), University Medical Center Groningen, 9700 RB Groningen, The Netherlands; Department of Internal Medicine, Section Endocrinology (M.L.D.), VU University Medical Center, 1007 MB Amsterdam, The Netherlands; Department of Endocrinology and Metabolism (P.H.B.), Academic Medical Center, 1100 DD Amsterdam Zuidoost, The Netherlands; Department of Internal Medicine, Division of Endocrinology (B.H.), Maastricht University Medical Center, 6202 AZ Maastricht, The Netherlands; and Department of Internal Medicine (W.W.d.H.), Erasmus Medical Center, 3000 CA Rotterdam, The Netherlands.
authors.forename: Bas
authors.initials: B
authors.lastname: Havekes
authors.affiliation: Department of Endocrine Oncology (J.M.d.L., C.R.C.P., W.P.K., G.D.V.), University Medical Center Utrecht, 3508 GA Utrecht, The Netherlands; Department of Endocrinology and Metabolism (O.M.D., A.M.P.), Leiden University Medical Center, 2300 RC Leiden, The Netherlands; Department of Endocrinology (A.R.H.), Radboud University Medical Center, 6500 HB Nijmegen, The Netherlands Department of Endocrinology (A.N.v.d.H.-S.), University Medical Center Groningen, 9700 RB Groningen, The Netherlands; Department of Internal Medicine, Section Endocrinology (M.L.D.), VU University Medical Center, 1007 MB Amsterdam, The Netherlands; Department of Endocrinology and Metabolism (P.H.B.), Academic Medical Center, 1100 DD Amsterdam Zuidoost, The Netherlands; Department of Internal Medicine, Division of Endocrinology (B.H.), Maastricht University Medical Center, 6202 AZ Maastricht, The Netherlands; and Department of Internal Medicine (W.W.d.H.), Erasmus Medical Center, 3000 CA Rotterdam, The Netherlands.
authors.forename: Wouter W
authors.initials: WW
authors.lastname: de Herder
authors.affiliation: Department of Endocrine Oncology (J.M.d.L., C.R.C.P., W.P.K., G.D.V.), University Medical Center Utrecht, 3508 GA Utrecht, The Netherlands; Department of Endocrinology and Metabolism (O.M.D., A.M.P.), Leiden University Medical Center, 2300 RC Leiden, The Netherlands; Department of Endocrinology (A.R.H.), Radboud University Medical Center, 6500 HB Nijmegen, The Netherlands Department of Endocrinology (A.N.v.d.H.-S.), University Medical Center Groningen, 9700 RB Groningen, The Netherlands; Department of Internal Medicine, Section Endocrinology (M.L.D.), VU University Medical Center, 1007 MB Amsterdam, The Netherlands; Department of Endocrinology and Metabolism (P.H.B.), Academic Medical Center, 1100 DD Amsterdam Zuidoost, The Netherlands; Department of Internal Medicine, Division of Endocrinology (B.H.), Maastricht University Medical Center, 6202 AZ Maastricht, The Netherlands; and Department of Internal Medicine (W.W.d.H.), Erasmus Medical Center, 3000 CA Rotterdam, The Netherlands.
authors.forename: Gerlof D
authors.initials: GD
authors.lastname: Valk
mesh_terms: D000236:Adenoma / Q000473:pathology*; D000293:Adolescent; D000328:Adult; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D018761:Multiple Endocrine Neoplasia Type 1 / Q000473:pathology*; D009426:Netherlands; D010911:Pituitary Neoplasms / Q000473:pathology*; D015175:Prolactinoma / Q000473:pathology*; D055815:Young Adult