title: Thymic neuroendocrine tumors in multiple endocrine neoplasia type 1: a comparative study on 21 cases among a series of 761 MEN1 from the GTE (Groupe des Tumeurs Endocrines).
abstract: BACKGROUND
  Thymic neuroendocrine tumors (Th-NET) present a poor prognosis for patients with multiple endocrine neoplasia type 1 (MEN1). The purpose of this article was to study the clinical, biological, and pathological features of Th-NET in a large cohort of patients with MEN1.
  
  
  METHODS
  The 761-patient MEN1 cohort from the GTE registry was used (Groupe des Tumeurs Endocrines).
  
  
  RESULTS
  The actuarial probability of occurrence was 2.6% (range, 1.3-5.5%) at aged 40 years. All, except one, Th-NET patients were men. Four patients had no other associated lesions. The youngest patient was aged 16 years. Mean age at the time of diagnosis was 42.7 (range, 16.1-67.5) years. The 10-year probability of survival was 36.1% (range, 11.5-62%). Seven patients (33%) belonged to clustered MEN1 families. The spectrum of associated lesions in patients with Th-NET was not statistically different from the spectrum of the remainder of the cohort. Various endocrine markers were high, but none were sensitive or specific enough to be useful for Th-NET detection. CT-scan and MRI were always positive at the time of diagnosis. No particular mutation was found to be associated with Th-NET. Five cases underwent prophylactic thymectomy without success.
  
  
  CONCLUSIONS
  Several end points may be helpful for future guidelines: (1) earlier detection of Th-NET in MEN1 patients is required; (2) screening of both sexes is necessary; (3) a prospective study comparing MRI vs. CT scan in yearly screening for Th-NET is needed; (4) a reinforced screening program must be established for patients who belong to clustered families; and (5) thymectomies must be performed in specialized centers.
authors.affiliation: Faculté de médecine de Dijon, Centre d'Epidémiologie des Populations, EA4184, Université de Bourgogne, BP 87900, 21079, Dijon cedex, France. pierre.goudet@chu-dijon.fr
authors.forename: Pierre
authors.initials: P
authors.lastname: Goudet
authors.forename: Arnaud
authors.initials: A
authors.lastname: Murat
authors.forename: Catherine
authors.initials: C
authors.lastname: Cardot-Bauters
authors.forename: Philippe
authors.initials: P
authors.lastname: Emy
authors.forename: Eric
authors.initials: E
authors.lastname: Baudin
authors.forename: Hélène
authors.initials: H
authors.lastname: du Boullay Choplin
authors.forename: Yves
authors.initials: Y
authors.lastname: Chapuis
authors.forename: Jean-Louis
authors.initials: JL
authors.lastname: Kraimps
authors.forename: Jean-Louis
authors.initials: JL
authors.lastname: Sadoul
authors.forename: Antoine
authors.initials: A
authors.lastname: Tabarin
authors.forename: Bruno
authors.initials: B
authors.lastname: Vergès
authors.forename: Bruno
authors.initials: B
authors.lastname: Carnaille
authors.forename: Patricia
authors.initials: P
authors.lastname: Niccoli-Sire
authors.forename: Annie
authors.initials: A
authors.lastname: Costa
authors.forename: Alain
authors.initials: A
authors.lastname: Calender
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D014408:Biomarkers, Tumor / Q000097:blood; D015331:Cohort Studies; D005260:Female; D017408:Guidelines as Topic; D006801:Humans; D008297:Male; D008875:Middle Aged; D018761:Multiple Endocrine Neoplasia Type 1 / Q000097:blood / Q000453:epidemiology / Q000235:genetics / Q000473:pathology; D012042:Registries; D012737:Sex Factors; D013953:Thymus Neoplasms / Q000097:blood / Q000453:epidemiology / Q000235:genetics / Q000473:pathology; D055815:Young Adult
chemical_list: D014408:Biomarkers, Tumor