PMID- 15657529
OWN - NLM
STAT- MEDLINE
DCOM- 20050329
LR  - 20220419
IS  - 0399-8320 (Print)
IS  - 0399-8320 (Linking)
VI  - 28
IP  - 11
DP  - 2004 Nov
TI  - Prevalence, characteristics and prognosis of MEN 1-associated glucagonomas, 
      VIPomas, and somatostatinomas: study from the GTE (Groupe des Tumeurs Endocrines) 
      registry.
PG  - 1075-81
AB  - Few studies have concerned the rare functioning endocrine pancreatic tumors 
      associated with multiple endocrine neoplasia type 1 (MEN 1). When sporadic, these 
      tumors have a poor prognosis. AIM: To analyze the frequency, characteristics and 
      prognosis of MEN 1-associated glucagonomas, VIPomas and somatostatinomas recorded 
      in the GTE (Groupe des Tumeurs Endocrines) registry. METHODS: Records of the 
      patients whose GTE registry codes included glucagonoma, VIPoma or somatostatinoma 
      were reviewed. The diagnosis was confirmed when there were clinical signs of a 
      functioning tumor and/or when blood levels of the peptide were higher than twice 
      the upper limit of normal. RESULTS: Among 580 patients with MEN 1, 
      duodeno-pancreatic involvement was present in 307 (52.9%). Five (1.6%) had a 
      glucagonoma, 3 (0.98%) a VIPoma and 2 (0.65%) a somatostatinoma. A clinical 
      syndrome was present in 1 patient with glucagonoma, in the 3 with VIPomas and in 
      1 with somatostatinoma. Tumor size was greater than 3 cm more often for these 
      rare tumours (67%) than in patients with other type of duodeno-pancreatic 
      involvement (28%) (P=0.02) and visceral metastases were more frequent (40% vs 
      15%; P=0.056). Ten-year survival of patients with glucagonomas, VIPomas or 
      somatostatinomas (53.8%; CI95%: 15.5-92.1) was poorer than that of patients with 
      insulinomas (91.4%; CI95%: 83.399.5; P=0.01) or gastrinomas (81.7%; CI95%: 
      74.9-88.5; P=0.20) and close to that of patients with non-functioning tumors 
      (62.2%, CI95%: 41.0-83.9; NS). CONCLUSION: Glucagonomas, VIPomas and 
      somatostatinomas, especially the functioning type, are very rare in patients with 
      MEN 1. Prognosis is poor, probably because of large tumor size and high rate of 
      metastasis. Survival is similar to that in patients with non-functioning tumors.
FAU - Levy-Bohbot, Nathalie
AU  - Levy-Bohbot N
AD  - Service d'Endocrinologie, Hopital Robert Debre, Reims.
FAU - Merle, Corinne
AU  - Merle C
FAU - Goudet, Pierre
AU  - Goudet P
FAU - Delemer, Brigitte
AU  - Delemer B
FAU - Calender, Alain
AU  - Calender A
FAU - Jolly, Damien
AU  - Jolly D
FAU - Thiefin, Gerard
AU  - Thiefin G
FAU - Cadiot, Guillaume
AU  - Cadiot G
CN  - Groupe des Tumeurs Endocrines
LA  - eng
PT  - Comparative Study
PT  - Journal Article
PL  - France
TA  - Gastroenterol Clin Biol
JT  - Gastroenterologie clinique et biologique
JID - 7704825
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Aged
MH  - Aged, 80 and over
MH  - Child
MH  - Child, Preschool
MH  - Female
MH  - Glucagonoma/diagnosis/*epidemiology
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/diagnosis/*epidemiology
MH  - Pancreatic Neoplasms/diagnosis/*epidemiology
MH  - Prevalence
MH  - Prognosis
MH  - Registries
MH  - Somatostatinoma/diagnosis/*epidemiology
MH  - Vipoma/*epidemiology
EDAT- 2005/01/20 09:00
MHDA- 2005/03/30 09:00
CRDT- 2005/01/20 09:00
PHST- 2005/01/20 09:00 [pubmed]
PHST- 2005/03/30 09:00 [medline]
PHST- 2005/01/20 09:00 [entrez]
AID - s0399-8320(04)95184-6 [pii]
AID - 10.1016/s0399-8320(04)95184-6 [doi]
PST - ppublish
SO  - Gastroenterol Clin Biol. 2004 Nov;28(11):1075-81. doi: 
      10.1016/s0399-8320(04)95184-6.