PMID- 12491633
OWN - NLM
STAT- MEDLINE
DCOM- 20030108
LR  - 20191106
IS  - 0003-3944 (Print)
IS  - 0003-3944 (Linking)
VI  - 127
IP  - 8
DP  - 2002 Oct
TI  - [Multiple endocrine neoplasia type I or Werner syndrome. What is important to 
      know about surgery of a rate disease].
PG  - 591-9
AB  - Multiple endocrine neoplasia type 1 (MEN1) is a rare but misleading disease. The 
      diagnosis is evocated when two main lesions are present (parathyroid, endocrine 
      pancreas, pituary gland) but also when a family tree shows recurrent lesions. 
      Other lesions must be taken into account (adrenal glands, neuroendocrine thymic 
      or bronchic lesions, cutaneous lesions, lipomas, nervous central system tumors). 
      Any surgical cure without knowing the MEN1 background leads to failure. Specific 
      treatment of each lesion is reviewed. Genetic diagnosis is possible but the 
      mutation is not found in all cases. Nevertheless, when the mutation is known in a 
      family, a negative genetic test allows to exclude the disease. Prognosis is 
      related to hepatic metastases and to thymic neuroendocrine tumors which are rare 
      (2.1%) but aggressive. As a general rule, any apparently isolated endocrine 
      lesion such hyperparathyroidism must prompt the surgeon to look for another 
      endocrine lesion and to look for an abnormal family tree with recurent 
      monoglandular or pluriglandular lesions.
FAU - Goudet, P
AU  - Goudet P
AD  - Service de chirurgie viscerale et endocrinienne (Pr. Patrick Cougard), centre 
      hospitalier universitaire de Dijon, hopital general, 3, rue du Faubourg-Raines, 
      21033 Dijon, France. pierre.goudet@chu-dijon.fr
FAU - Calender, A
AU  - Calender A
FAU - Cougard, P
AU  - Cougard P
FAU - Murat, A
AU  - Murat A
FAU - Henry, J F
AU  - Henry JF
FAU - Kraimps, J L
AU  - Kraimps JL
FAU - Cadiot, G
AU  - Cadiot G
FAU - Peix, J L
AU  - Peix JL
FAU - Sarfati, E
AU  - Sarfati E
FAU - Mignon, M
AU  - Mignon M
FAU - Proye, C
AU  - Proye C
CN  - Groupe d'Etude des neoplasies Endocrinienne multiples
LA  - fre
PT  - English Abstract
PT  - Journal Article
PT  - Review
TT  - La neoplasie endocrinienne multiple de type 1 ou syndrome de Wermer. Ce qu'il 
      faut savoir en chirurgie d'une maladie rare.
PL  - France
TA  - Ann Chir
JT  - Annales de chirurgie
JID - 0140722
SB  - IM
MH  - Diagnosis, Differential
MH  - *Genetic Testing
MH  - Humans
MH  - Hyperparathyroidism/etiology
MH  - Liver Neoplasms/secondary
MH  - Multiple Endocrine Neoplasia Type 1/diagnosis/pathology/*surgery
MH  - Pancreatic Neoplasms/diagnosis/pathology/*surgery
MH  - Parathyroid Neoplasms/diagnosis/pathology/*surgery
MH  - Pedigree
MH  - Pituitary Neoplasms/diagnosis/pathology/*surgery
MH  - Prognosis
RF  - 15
EDAT- 2002/12/21 04:00
MHDA- 2003/01/09 04:00
CRDT- 2002/12/21 04:00
PHST- 2002/12/21 04:00 [pubmed]
PHST- 2003/01/09 04:00 [medline]
PHST- 2002/12/21 04:00 [entrez]
AID - S0003-3944(02)00848-9 [pii]
AID - 10.1016/s0003-3944(02)00848-9 [doi]
PST - ppublish
SO  - Ann Chir. 2002 Oct;127(8):591-9. doi: 10.1016/s0003-3944(02)00848-9.