PMID- 21061174
OWN - NLM
STAT- MEDLINE
DCOM- 20110602
LR  - 20211020
IS  - 1573-7292 (Electronic)
IS  - 1389-9600 (Linking)
VI  - 10
IP  - 1
DP  - 2011 Mar
TI  - Care for patients with multiple endocrine neoplasia type 1: the current evidence 
      base.
PG  - 157-71
LID - 10.1007/s10689-010-9398-6 [doi]
AB  - Multiple endocrine neoplasia type 1 (MEN1) is a rare disease caused by mutations 
      in the MEN1 gene on chromosome 11. It is characterized by the occurrence of 
      primary hyperparathyroidism (pHPT), duodenopancreatic neuroendocrine tumours 
      (pNET), pituitary tumours (PIT), adrenal adenomas (ADR) and neuroendocrine 
      tumours (NET) of the stomach, bronchus and thymus. MEN1 is a syndrome with high 
      penetrance and high morbidity. Malignant NETs are the most important cause of 
      MEN1-related death. Since 1997 the diagnosis can be made by genetic screening. 
      MEN1 is a complex syndrome and the endocrine manifestations cannot be viewed upon 
      as coinciding sporadic tumours. Differences in epidemiology and pathology between 
      MEN1-related tumours and their sporadic counterparts show that a unique approach 
      is needed. Therefore the care for MEN1 patients should be provided by a centre of 
      expertise. Early genetic diagnosis and periodic screening are important pillars 
      of care. For primary hyperparathyroidism surgery is the most important treatment 
      modality, with a subtotal parathyroid gland resection as the procedure of choice. 
      In neuroendocrine tumours surgery also is the most important treatment modality. 
      Selective tumour enucleation has no place in the surgical treatment of 
      MEN1-related pNETs; the exact procedure depends on the functionality of the 
      tumour. In MEN1-associated pituitary and adrenal adenomas, watchful waiting and 
      medical therapy play more important roles. In the twenty-first century new 
      developments will impact the care for MEN1 patients. These developments should be 
      critically evaluated in clinical research with the ultimate goal of optimizing 
      the care for MEN1 patients on an evidence base.
FAU - Pieterman, C R C
AU  - Pieterman CR
AD  - Department of Internal Medicine, University Medical Centre Utrecht, Utrecht, The 
      Netherlands.
FAU - Vriens, M R
AU  - Vriens MR
FAU - Dreijerink, K M A
AU  - Dreijerink KM
FAU - van der Luijt, R B
AU  - van der Luijt RB
FAU - Valk, G D
AU  - Valk GD
LA  - eng
PT  - Journal Article
PT  - Review
PL  - Netherlands
TA  - Fam Cancer
JT  - Familial cancer
JID - 100898211
SB  - IM
MH  - Humans
MH  - Multiple Endocrine Neoplasia Type 1/*diagnosis/*therapy
MH  - *Patient Care
EDAT- 2010/11/10 06:00
MHDA- 2011/06/03 06:00
CRDT- 2010/11/10 06:00
PHST- 2010/11/10 06:00 [entrez]
PHST- 2010/11/10 06:00 [pubmed]
PHST- 2011/06/03 06:00 [medline]
AID - 10.1007/s10689-010-9398-6 [doi]
PST - ppublish
SO  - Fam Cancer. 2011 Mar;10(1):157-71. doi: 10.1007/s10689-010-9398-6.