PMID- 31263451
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20200225
IS  - 1664-2392 (Print)
IS  - 1664-2392 (Electronic)
IS  - 1664-2392 (Linking)
VI  - 10
DP  - 2019
TI  - Multiple Endocrine Neoplasia Type 1 (MEN1): An Update and the Significance of 
      Early Genetic and Clinical Diagnosis.
PG  - 339
LID - 10.3389/fendo.2019.00339 [doi]
LID - 339
AB  - Multiple endocrine neoplasia type 1 (MEN1) is a rare hereditary tumor syndrome 
      inherited in an autosomal dominant manner and characterized by a predisposition 
      to a multitude of endocrine neoplasms primarily of parathyroid, enteropancreatic, 
      and anterior pituitary origin, as well as nonendocrine neoplasms. Other endocrine 
      tumors in MEN1 include foregut carcinoid tumors, adrenocortical tumors, and 
      rarely pheochromocytoma. Nonendocrine manifestations include meningiomas and 
      ependymomas, lipomas, angiofibromas, collagenomas, and leiomyomas. MEN1 is caused 
      by inactivating mutations of the tumor suppressor gene MEN1 which encodes the 
      protein menin. This syndrome can affect all age groups, with 17% of patients 
      developing MEN1-associated tumors before 21 years of age. Despite advances in the 
      diagnosis and treatment of MEN1-associated tumors, patients with MEN1 continue to 
      have decreased life expectancy primarily due to malignant neuroendocrine tumors. 
      The most recent clinical practice guidelines for MEN1, published in 2012, 
      highlight the need for early genetic and clinical diagnosis of MEN1 and recommend 
      an intensive surveillance approach for both patients with this syndrome and 
      asymptomatic carriers starting at the age of 5 years with the goal of timely 
      detection and management of MEN1-associated neoplasms and ultimately decreased 
      disease-specific morbidity and mortality. Unfortunately, there is no clear 
      genotype-phenotype correlation and individual mutation-dependent surveillance is 
      not possible currently.
FAU - Kamilaris, Crystal D C
AU  - Kamilaris CDC
AD  - Section on Endocrinology and Genetics and Endocrinology Inter-institute Training 
      Program, Eunice Kennedy Shriver National Institute of Child Health and Human 
      Development, National Institutes of Health, Bethesda, MD, United States.
FAU - Stratakis, Constantine A
AU  - Stratakis CA
AD  - Section on Endocrinology and Genetics and Endocrinology Inter-institute Training 
      Program, Eunice Kennedy Shriver National Institute of Child Health and Human 
      Development, National Institutes of Health, Bethesda, MD, United States.
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20190611
PL  - Switzerland
TA  - Front Endocrinol (Lausanne)
JT  - Frontiers in endocrinology
JID - 101555782
PMC - PMC6584804
OTO - NOTNLM
OT  - MEN1
OT  - adrenocortical tumor
OT  - carcinoid
OT  - enteropancreatic tumor
OT  - hyperparathyroidism
OT  - pituitary adenoma
EDAT- 2019/07/03 06:00
MHDA- 2019/07/03 06:01
PMCR- 2019/01/01
CRDT- 2019/07/03 06:00
PHST- 2018/10/24 00:00 [received]
PHST- 2019/05/10 00:00 [accepted]
PHST- 2019/07/03 06:00 [entrez]
PHST- 2019/07/03 06:00 [pubmed]
PHST- 2019/07/03 06:01 [medline]
PHST- 2019/01/01 00:00 [pmc-release]
AID - 10.3389/fendo.2019.00339 [doi]
PST - epublish
SO  - Front Endocrinol (Lausanne). 2019 Jun 11;10:339. doi: 10.3389/fendo.2019.00339. 
      eCollection 2019.