PMID- 33564173
OWN - NLM
STAT- MEDLINE
DCOM- 20210422
LR  - 20230129
IS  - 1759-5037 (Electronic)
IS  - 1759-5029 (Linking)
VI  - 17
IP  - 4
DP  - 2021 Apr
TI  - Clinical aspects of multiple endocrine neoplasia type 1.
PG  - 207-224
LID - 10.1038/s41574-021-00468-3 [doi]
AB  - Multiple endocrine neoplasia type 1 (MEN1) is a rare syndrome characterized by 
      the co-occurrence of primary hyperparathyroidism, duodenopancreatic 
      neuroendocrine tumours (NETs) and/or pituitary adenomas. MEN1 can predispose 
      patients to other endocrine and non-endocrine tumours, such as cutaneous tumours, 
      central nervous system tumours and breast cancer. Endocrine tumours in patients 
      with MEN1 differ from sporadic tumours in that they have a younger age at onset, 
      present as multiple tumours in the same organ and have a different clinical 
      course. Therefore, patients with overt MEN1 and those who carry a MEN1 mutation 
      should be offered tailored biochemical and imaging screening to detect tumours 
      and evaluate their progression over time. Fortunately, over the past 10 years, 
      knowledge about the clinical phenotype of these tumours has markedly progressed, 
      thanks to the implementation of national registries, particularly in France and 
      the Netherlands. This Review provides an update on the clinical management of 
      MEN1-related tumours. Epidemiology, the clinical picture, diagnostic work-up and 
      the main lines of treatment for MEN1-related tumours are summarized. 
      Controversial therapeutic aspects and issues that still need to be addressed are 
      also discussed. Moreover, special attention is given to MEN1 manifestations in 
      children and adolescents.
FAU - Al-Salameh, Abdallah
AU  - Al-Salameh A
AUID- ORCID: 0000-0002-7951-9926
AD  - Assistance Publique-Hopitaux de Paris, Hopital de Bicetre, Service 
      d'Endocrinologie et des Maladies de la Reproduction, Centre de Reference des 
      Maladies Rares de l'Hypophyse, Le Kremlin-Bicetre, France.
AD  - Service d'Endocrinologie, Maladies Metaboliques et Nutrition, CHU Amiens 
      Picardie, Amiens, France.
FAU - Cadiot, Guillaume
AU  - Cadiot G
AD  - Service d'Hepato-Gastro-Enterologie et de Cancerologie Digestive, Hopital Robert 
      Debre, Reims, France.
FAU - Calender, Alain
AU  - Calender A
AD  - Unite Medicale des Cancers et Maladies Multifactorielles, Service de Genetique, 
      Hospices Civils de Lyon, Lyon, France.
FAU - Goudet, Pierre
AU  - Goudet P
AD  - Service de Chirurgie Endocrinienne, Hopital du Bocage, Dijon, France.
FAU - Chanson, Philippe
AU  - Chanson P
AUID- ORCID: 0000-0001-5096-5722
AD  - Assistance Publique-Hopitaux de Paris, Hopital de Bicetre, Service 
      d'Endocrinologie et des Maladies de la Reproduction, Centre de Reference des 
      Maladies Rares de l'Hypophyse, Le Kremlin-Bicetre, France. 
      philippe.chanson@aphp.fr.
AD  - Universite Paris-Saclay, Inserm, Physiologie et Physiopathologie Endocriniennes, 
      Le Kremlin-Bicetre, France. philippe.chanson@aphp.fr.
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20210209
PL  - England
TA  - Nat Rev Endocrinol
JT  - Nature reviews. Endocrinology
JID - 101500078
RN  - 0 (MEN1 protein, human)
RN  - 0 (Proto-Oncogene Proteins)
SB  - IM
MH  - Humans
MH  - Hyperparathyroidism/complications
MH  - Multiple Endocrine Neoplasia Type 1/*complications/*diagnosis/genetics/therapy
MH  - Neuroendocrine Tumors/complications
MH  - Proto-Oncogene Proteins/genetics
EDAT- 2021/02/11 06:00
MHDA- 2021/04/23 06:00
CRDT- 2021/02/10 05:54
PHST- 2021/01/07 00:00 [accepted]
PHST- 2021/02/11 06:00 [pubmed]
PHST- 2021/04/23 06:00 [medline]
PHST- 2021/02/10 05:54 [entrez]
AID - 10.1038/s41574-021-00468-3 [pii]
AID - 10.1038/s41574-021-00468-3 [doi]
PST - ppublish
SO  - Nat Rev Endocrinol. 2021 Apr;17(4):207-224. doi: 10.1038/s41574-021-00468-3. Epub 
      2021 Feb 9.