PMID- 8949733
OWN - NLM
STAT- MEDLINE
DCOM- 19970116
LR  - 20161209
IS  - 0755-4982 (Print)
IS  - 0755-4982 (Linking)
VI  - 25
IP  - 27
DP  - 1996 Sep 21
TI  - [Characteristic pathological associations in multiple endocrine neoplasia type 
      1].
PG  - 1229-33
AB  - OBJECTIVES: Multiple endocrine neoplasia type 1 (MEN 1) is an inherited disorder 
      characterised by slow progressing tumors of the parathyroids, of the endocrine 
      pancreas and of the anterior pituitary. A genetic locus predisposing to this 
      disease has been localised on chromosome 11. Predictive diagnosis of carriers of 
      the defective gene is possible in families using genetic markers at this locus. 
      However, this analysis presupposes a precise identification of affected subjects. 
      Moreover, expression of the disease may vary from one family to the other. The 
      aim of the present study was to define the typical clinical features of the 
      syndrom. METHODS: We assessed retrospectively 26 cases of MEN 1 identified during 
      20 years in the same medico-surgical center. Among 11 men and 15 women, all those 
      who had a genealogical investigation had a positive family history of MEN 1. 
      RESULTS: Bifocal and trifocal tumors were the main patterns of associations, and 
      were diagnosed at a mean age of 48.6 years. Parathyroid involvement was most 
      frequent and earliest (96% of cases). The second most frequent was pancreatic 
      involvement (69.2% of cases) predominantly manifesting with gastrinomas (N = 13). 
      Multifocal tumors were usually diagnosed before or within 5 years following 
      diagnosis of the first tumor. Among pituitary tumors one case of meningioma was 
      observed, a feature not reported previously. An asymptomatic adrenal involvement 
      was observed in about 1/3 of cases. Other silent tumors (euthyroid nodules, 
      lipomas) were also noted. CONCLUSION: These data suggest that the clinical 
      presentation and course of MEN 1 is homogeneous and are in agreement with the 
      hypothesis of a recessive tumor-suppressor gene expressed in specific endocrine 
      cell lines, suggesting that careful family studies should be conducted when a 
      case of MEN 1 is diagnosed to facilitate early carrier detection among relatives.
FAU - Chigot, J P
AU  - Chigot JP
AD  - Service de Chirurgie viscerale, Groupe Hospitalier Pitie-Salpetriere, Paris.
FAU - Bendib, S
AU  - Bendib S
FAU - Turpin, G
AU  - Turpin G
FAU - Benlian, P
AU  - Benlian P
LA  - fre
PT  - English Abstract
PT  - Journal Article
PT  - Review
TT  - Associations pathologiques caracteristiques dans la neoplasie endocrinienne 
      multiple de type 1.
PL  - France
TA  - Presse Med
JT  - Presse medicale (Paris, France : 1983)
JID - 8302490
SB  - IM
MH  - Adult
MH  - Aged
MH  - Female
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/diagnosis/*genetics
MH  - Pedigree
MH  - Retrospective Studies
RF  - 13
EDAT- 1996/09/21 00:00
MHDA- 1996/09/21 00:01
CRDT- 1996/09/21 00:00
PHST- 1996/09/21 00:00 [pubmed]
PHST- 1996/09/21 00:01 [medline]
PHST- 1996/09/21 00:00 [entrez]
PST - ppublish
SO  - Presse Med. 1996 Sep 21;25(27):1229-33.