PMID- 20175448
OWN - NLM
STAT- MEDLINE
DCOM- 20100405
LR  - 20190622
IS  - 0065-2598 (Print)
IS  - 0065-2598 (Linking)
VI  - 668
DP  - 2009
TI  - MEN1 clinical background.
PG  - 1-15
AB  - Multiple endocrine neoplasia Type 1 (MEN1) is a rare hereditary tumor syndrome 
      predisposing to tumor development in several endocrine organs. Its major 
      manifestations include hyperparathyroidism, tumors of endocrine pancreas and 
      pituitary. Beside these three, several other endocrine (adrenocortical, foregut 
      carcinoid) and nonendocrine (lipoma, angiofibroma, collagenoma, ependymoma, 
      meningioma) tumors have been described to be associated with this syndrome. Both 
      familial and sporadic forms of the disease are known. The diagnosis of MEN1 can 
      be established if two of the three major manifestations are found in the same 
      patient, whereas the diagnosis of familial MEN1 requires one MEN1 patient and a 
      first degree relative with at least one MEN1 manifestation. MEN1 is transmitted 
      as an autosomal dominant trait with high penetrance, approaching 95-100% by the 
      age of 60. Both benign (parathyroid, anterior pituitary) and malignant 
      (gastrinoma, glucagonoma) lesions may develop in MEN1 patients. Regular 
      surveillance of MEN1 gene mutation carriers is necessary to reveal disease 
      manifestations. Several diagnostic modalities can be used to screen for and to 
      examine MEN1-related tumors. The therapy of MEN1-associated tumors requires 
      specific approach in some cases, as multiple tumors and recurrence is frequently 
      observed.
FAU - Igaz, Peter
AU  - Igaz P
AD  - 2nd Department of Medicine, Faculty of Medicine, Semmelweis University, 1088 
      Budapest, Szentkiralyi u. 46, Hungary. igapet@bel2.sote.hu
LA  - eng
PT  - Journal Article
PL  - United States
TA  - Adv Exp Med Biol
JT  - Advances in experimental medicine and biology
JID - 0121103
SB  - IM
MH  - Adult
MH  - Child
MH  - Child, Preschool
MH  - Diagnosis, Differential
MH  - Genetic Predisposition to Disease
MH  - Genetic Testing
MH  - Humans
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/*genetics/*pathology/*physiopathology/therapy
MH  - Multiple Endocrine Neoplasia Type 2a/genetics/pathology/physiopathology
MH  - Mutation
MH  - Young Adult
EDAT- 2010/02/24 06:00
MHDA- 2010/04/07 06:00
CRDT- 2010/02/24 06:00
PHST- 2010/02/24 06:00 [entrez]
PHST- 2010/02/24 06:00 [pubmed]
PHST- 2010/04/07 06:00 [medline]
AID - 10.1007/978-1-4419-1664-8_1 [doi]
PST - ppublish
SO  - Adv Exp Med Biol. 2009;668:1-15. doi: 10.1007/978-1-4419-1664-8_1.