PMID- 19294466
OWN - NLM
STAT- MEDLINE
DCOM- 20091020
LR  - 20211020
IS  - 1432-2323 (Electronic)
IS  - 0364-2313 (Linking)
VI  - 33
IP  - 6
DP  - 2009 Jun
TI  - 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).
PG  - 1197-207
LID - 10.1007/s00268-009-9980-y [doi]
AB  - 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.
FAU - Goudet, Pierre
AU  - Goudet P
AD  - Faculte de medecine de Dijon, Centre d'Epidemiologie des Populations, EA4184, 
      Universite de Bourgogne, BP 87900, 21079, Dijon cedex, France. 
      pierre.goudet@chu-dijon.fr
FAU - Murat, Arnaud
AU  - Murat A
FAU - Cardot-Bauters, Catherine
AU  - Cardot-Bauters C
FAU - Emy, Philippe
AU  - Emy P
FAU - Baudin, Eric
AU  - Baudin E
FAU - du Boullay Choplin, Helene
AU  - du Boullay Choplin H
FAU - Chapuis, Yves
AU  - Chapuis Y
FAU - Kraimps, Jean-Louis
AU  - Kraimps JL
FAU - Sadoul, Jean-Louis
AU  - Sadoul JL
FAU - Tabarin, Antoine
AU  - Tabarin A
FAU - Verges, Bruno
AU  - Verges B
FAU - Carnaille, Bruno
AU  - Carnaille B
FAU - Niccoli-Sire, Patricia
AU  - Niccoli-Sire P
FAU - Costa, Annie
AU  - Costa A
FAU - Calender, Alain
AU  - Calender A
CN  - GTE network (Groupe des Tumeurs Endocrines)
LA  - eng
PT  - Comparative Study
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - World J Surg
JT  - World journal of surgery
JID - 7704052
RN  - 0 (Biomarkers, Tumor)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Aged
MH  - Biomarkers, Tumor/blood
MH  - Cohort Studies
MH  - Female
MH  - Guidelines as Topic
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - *Multiple Endocrine Neoplasia Type 1/blood/epidemiology/genetics/pathology
MH  - Registries
MH  - Sex Factors
MH  - *Thymus Neoplasms/blood/epidemiology/genetics/pathology
MH  - Young Adult
EDAT- 2009/03/19 09:00
MHDA- 2009/10/21 06:00
CRDT- 2009/03/19 09:00
PHST- 2009/03/19 09:00 [entrez]
PHST- 2009/03/19 09:00 [pubmed]
PHST- 2009/10/21 06:00 [medline]
AID - 10.1007/s00268-009-9980-y [doi]
PST - ppublish
SO  - World J Surg. 2009 Jun;33(6):1197-207. doi: 10.1007/s00268-009-9980-y.