title: Prevalence, characteristics and prognosis of MEN 1-associated glucagonomas, VIPomas, and somatostatinomas: study from the GTE (Groupe des Tumeurs Endocrines) registry.
abstract: 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.
  
  
  OBJECTIVE
  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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Service d'Endocrinologie, Hôpital Robert Debré, Reims.
authors.forename: Nathalie
authors.initials: N
authors.lastname: Lévy-Bohbot
authors.forename: Corinne
authors.initials: C
authors.lastname: Merle
authors.forename: Pierre
authors.initials: P
authors.lastname: Goudet
authors.forename: Brigitte
authors.initials: B
authors.lastname: Delemer
authors.forename: Alain
authors.initials: A
authors.lastname: Calender
authors.forename: Damien
authors.initials: D
authors.lastname: Jolly
authors.forename: Gérard
authors.initials: G
authors.lastname: Thiéfin
authors.forename: Guillaume
authors.initials: G
authors.lastname: Cadiot
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D002675:Child, Preschool; D005260:Female; D005935:Glucagonoma / Q000175:diagnosis / Q000453:epidemiology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D018761:Multiple Endocrine Neoplasia Type 1 / Q000175:diagnosis / Q000453:epidemiology*; D010190:Pancreatic Neoplasms / Q000175:diagnosis / Q000453:epidemiology*; D015995:Prevalence; D011379:Prognosis; D012042:Registries; D013005:Somatostatinoma / Q000175:diagnosis / Q000453:epidemiology*; D003969:Vipoma / Q000453:epidemiology*