PMID- 24915123
OWN - NLM
STAT- MEDLINE
DCOM- 20150112
LR  - 20220311
IS  - 1945-7197 (Electronic)
IS  - 0021-972X (Linking)
VI  - 99
IP  - 9
DP  - 2014 Sep
TI  - Natural course and survival of neuroendocrine tumors of thymus and lung in MEN1 
      patients.
PG  - 3325-33
LID - 10.1210/jc.2014-1560 [doi]
AB  - CONTEXT: The natural course and survival of neuroendocrine tumors (NETs) of 
      thymus (Th) and lung in multiple endocrine neoplasia type 1 (MEN1) patients are 
      still unknown. OBJECTIVE: Our objective was to assess prevalence, tumor growth, 
      and survival of Th and lung NETs in an unselected MEN1 population with long-term 
      follow-up. DESIGN: This was an observational study. PATIENTS AND METHODS: A 
      longitudinal study was performed using the Dutch national MEN1 database, 
      including >90% of the Dutch MEN1 population >16 years of age. Patients under care 
      of the Dutch University Medical Centers (1990-2011) (n = 323) were included. MAIN 
      OUTCOME MEASURES: The prevalence and survival of Th and lung NETs were assessed. 
      Linear mixed-models analysis was applied to assess tumor growth with age as a 
      possible confounder and gender, genotype and baseline tumor size as possible 
      effect modifiers. RESULTS: Th NETs occurred in 3.4% of patients, almost 
      exclusively in males with a 10-year survival of 25% (95% confidence interval = 
      8%-80%). A thoracic computed tomography scan was available in 188 patients 
      (58.2%). A lung NET was identified in 42 patients (13.0%) with a 10-year survival 
      of 71.1% (95% confidence interval = 51%-100%). Tumor volume of lung NETs 
      increased 17% per year (P < .001) (tumor doubling time 4.5 years). Tumor doubling 
      time in males was 2.5 vs 5.5 years in females (P = .05). Lung NET growth was not 
      associated with genotype or with baseline tumor size (<1 vs >/=1 cm). CONCLUSION: 
      In MEN1 patients, Th NETs almost exclusively occurred in males and had a very low 
      prevalence and a high mortality. Lung NETs occurred more often than previously 
      thought, had an indolent course, and occurred equally in both sexes. Tumor growth 
      in males was double compared with female patients.
FAU - de Laat, Joanne M
AU  - de Laat JM
AD  - Departments of Internal Medicine (J.M.d.L., C.R.P., M.F.v.d.B., G.D.V.) and 
      Surgery (M.R.V.), University Medical Center Utrecht, 3508 GA Utrecht, The 
      Netherlands; Department of Clinical Epidemiology and Biostatistics (J.W.T.), VU 
      University Medical Center, 1007 MB, Amsterdam, The Netherlands; Department of 
      Endocrinology (A.R.H.), Radboud University Medical Center, 6500 HB Nijmegen, The 
      Netherlands; Departments of Endocrinology and Metabolism and Clinical 
      Epidemiology (O.M.D.), Leiden University Medical Center, 2300 RC Leiden, The 
      Netherlands; Department of Internal Medicine (W.W.d.H.), Erasmus Medical Center, 
      3000 CA Rotterdam, The Netherlands; Department of Endocrinology (A.N.v.d.H.-S.), 
      University Medical Center Groningen, 9700 RB Groningen, The Netherlands; 
      Department of Internal Medicine (M.L.D.), Section of Endocrinology, VU University 
      Medical Center, 6202 AZ Amsterdam, The Netherlands; Department of Endocrinology 
      and Metabolism (P.H.B.), Academic Medical Center, 1100 DD Amsterdam, The 
      Netherlands; and Department of Internal Medicine (B.H.), Division of 
      Endocrinology, Maastricht University Medical Center, 6202 AZ Maastricht, The 
      Netherlands.
FAU - Pieterman, Carolina R
AU  - Pieterman CR
FAU - van den Broek, Medard F
AU  - van den Broek MF
FAU - Twisk, Jos W
AU  - Twisk JW
FAU - Hermus, Ad R
AU  - Hermus AR
FAU - Dekkers, Olaf M
AU  - Dekkers OM
FAU - de Herder, Wouter W
AU  - de Herder WW
FAU - van der Horst-Schrivers, Anouk N
AU  - van der Horst-Schrivers AN
FAU - Drent, Madeleine L
AU  - Drent ML
FAU - Bisschop, Peter H
AU  - Bisschop PH
FAU - Havekes, Bas
AU  - Havekes B
FAU - Vriens, Menno R
AU  - Vriens MR
FAU - Valk, Gerlof D
AU  - Valk GD
LA  - eng
PT  - Journal Article
PT  - Observational Study
PT  - Research Support, Non-U.S. Gov't
DEP - 20140610
PL  - United States
TA  - J Clin Endocrinol Metab
JT  - The Journal of clinical endocrinology and metabolism
JID - 0375362
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Aged
MH  - Aged, 80 and over
MH  - Child
MH  - Disease Progression
MH  - Female
MH  - Follow-Up Studies
MH  - Humans
MH  - Kaplan-Meier Estimate
MH  - Longitudinal Studies
MH  - Lung Neoplasms/*mortality
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/*mortality
MH  - Neuroendocrine Tumors/*mortality
MH  - Prevalence
MH  - Prognosis
MH  - Thymus Neoplasms/*mortality
MH  - Young Adult
EDAT- 2014/06/11 06:00
MHDA- 2015/01/13 06:00
CRDT- 2014/06/11 06:00
PHST- 2014/06/11 06:00 [entrez]
PHST- 2014/06/11 06:00 [pubmed]
PHST- 2015/01/13 06:00 [medline]
AID - 10.1210/jc.2014-1560 [doi]
PST - ppublish
SO  - J Clin Endocrinol Metab. 2014 Sep;99(9):3325-33. doi: 10.1210/jc.2014-1560. Epub 
      2014 Jun 10.