PMID- 33839123
OWN - NLM
STAT- MEDLINE
DCOM- 20220203
LR  - 20220203
IS  - 2213-3941 (Electronic)
IS  - 0003-4266 (Linking)
VI  - 82
IP  - 2
DP  - 2021 Apr
TI  - New therapies for patients with multiple endocrine neoplasia type 1.
PG  - 112-120
LID - S0003-4266(21)00030-5 [pii]
LID - 10.1016/j.ando.2021.03.005 [doi]
AB  - In 1953, for the first time, Paul Wermer described a family presenting endocrine 
      gland neoplasms over several generations. The transmission was autosomal dominant 
      and the penetrance was high. Forty years later in 1997, the multiple endocrine 
      neoplasia type 1 (MEN1) gene was sequenced, thus enabling diagnosis and early 
      optimal treatment. Patients carrying the MEN1 gene present endocrine but also 
      non-endocrine tumors. Parathyroid, pancreatic and pituitary impairment are the 
      three main types of endocrine involvement. The present article details 
      therapeutic management of hyperparathyroidism, neuroendocrine pancreatic tumors 
      and pituitary adenomas in patients carrying the MEN1 gene. Significant 
      therapeutic progress has in fact been made in the last few years. As concerns the 
      parathyroid glands, screening of family members and regular monitoring of 
      affected subjects now raise the question of early management of parathyroid 
      lesions and optimal timing of parathyroid surgery. As concerns the 
      duodenum-pancreas, proton-pump inhibitors are able to control gastrin-secreting 
      syndrome, reducing mortality in MEN1 patients. Mortality in MEN1 patients is no 
      longer mainly secondary to uncontrolled hormonal secretion but to metastatic 
      (mainly pancreatic) disease progression. Tumor risk requires regular monitoring 
      of morphological assessment, leading to iterative pancreatic surgery in a large 
      number of patients. Finally, pituitary adenomas in MEN1 patients are 
      traditionally described as aggressive, invasive and resistant to medical 
      treatment. However, regular pituitary screening showed them to be in fact 
      infra-centimetric and non-secreting in the majority of patients. Consequently, it 
      is necessary to regularly monitor MEN1 patients, with regular clinical, 
      biological and morphological work-up. Several studies showed that this regular 
      monitoring impairs quality of life. Building a relationship of trust between 
      patients and care provider is therefore essential. It enables the patient to be 
      referred for psychological or psychiatric care in difficult times, providing 
      long-term support and preventing any breakdown in continuity of care.
CI  - Copyright (c) 2021 Elsevier Masson SAS. All rights reserved.
FAU - Geslot, Aurore
AU  - Geslot A
AD  - Service d'endocrinologie, hopital Larrey, 24, chemin de Pouvourville, 31029 
      Toulouse cedex 9, France.
FAU - Vialon, Magaly
AU  - Vialon M
AD  - Service d'endocrinologie, hopital Larrey, 24, chemin de Pouvourville, 31029 
      Toulouse cedex 9, France.
FAU - Caron, Philippe
AU  - Caron P
AD  - Service d'endocrinologie, hopital Larrey, 24, chemin de Pouvourville, 31029 
      Toulouse cedex 9, France.
FAU - Grunenwald, Solange
AU  - Grunenwald S
AD  - Service d'endocrinologie, hopital Larrey, 24, chemin de Pouvourville, 31029 
      Toulouse cedex 9, France.
FAU - Vezzosi, Delphine
AU  - Vezzosi D
AD  - Institut CardioMet, Toulouse, France; Service d'endocrinologie, hopital Larrey, 
      24, chemin de Pouvourville, 31029 Toulouse cedex 9, France. Electronic address: 
      vezzosi.d@chu-toulouse.fr.
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20210408
PL  - France
TA  - Ann Endocrinol (Paris)
JT  - Annales d'endocrinologie
JID - 0116744
SB  - IM
MH  - Breast Neoplasms/genetics/therapy
MH  - Female
MH  - Gastrinoma/genetics/therapy
MH  - Genetic Predisposition to Disease
MH  - Humans
MH  - Hyperparathyroidism/genetics/therapy
MH  - Insulinoma/genetics/therapy
MH  - Male
MH  - Multiple Endocrine Neoplasia Type 1/*genetics/*therapy
MH  - Neuroendocrine Tumors/genetics/therapy
MH  - Pancreatic Neoplasms/genetics/therapy
MH  - Parathyroidectomy
MH  - Pituitary Neoplasms/genetics/therapy
MH  - Thymus Neoplasms/genetics/therapy
OTO - NOTNLM
OT  - Adenome hypophysaire
OT  - Duodenopancreatic neuroendocrine tumor
OT  - Hyperparathyroidie primaire
OT  - Multiple endocrine neoplasia type 1
OT  - Neoplasie endocrinienne multiple de type 1
OT  - Pituitary adenoma
OT  - Primary hyperparathyroidism
OT  - Tumeur neuroendocrine duodeno-pancreatique
EDAT- 2021/04/12 06:00
MHDA- 2022/02/04 06:00
CRDT- 2021/04/11 20:30
PHST- 2021/02/14 00:00 [received]
PHST- 2021/03/18 00:00 [revised]
PHST- 2021/03/26 00:00 [accepted]
PHST- 2021/04/12 06:00 [pubmed]
PHST- 2022/02/04 06:00 [medline]
PHST- 2021/04/11 20:30 [entrez]
AID - S0003-4266(21)00030-5 [pii]
AID - 10.1016/j.ando.2021.03.005 [doi]
PST - ppublish
SO  - Ann Endocrinol (Paris). 2021 Apr;82(2):112-120. doi: 10.1016/j.ando.2021.03.005. 
      Epub 2021 Apr 8.