PMID- 36325452
OWN - NLM
STAT- MEDLINE
DCOM- 20221104
LR  - 20221105
IS  - 1664-2392 (Print)
IS  - 1664-2392 (Electronic)
IS  - 1664-2392 (Linking)
VI  - 13
DP  - 2022
TI  - Beyond the "3 Ps": A critical appraisal of the non-endocrine manifestations of 
      multiple endocrine neoplasia type 1.
PG  - 1029041
LID - 10.3389/fendo.2022.1029041 [doi]
LID - 1029041
AB  - Multiple endocrine neoplasia type 1 (MEN1), an autosomal-dominantly inherited 
      tumor syndrome, is classically defined by tumors arising from the "3 Ps": 
      Parathyroids, Pituitary, and the endocrine Pancreas. From its earliest 
      descriptions, MEN1 has been associated with other endocrine and non-endocrine 
      neoplastic manifestations. High quality evidence supports a direct association 
      between pathogenic MEN1 variants and neoplasms of the skin (angiofibromas and 
      collagenomas), adipose tissue (lipomas and hibernomas), and smooth muscle 
      (leiomyomas). Although CNS tumors, melanoma, and, most recently, breast cancer 
      have been reported as MEN1 clinical manifestations, the published evidence to 
      date is not yet sufficient to establish causality. Well-designed, multicenter 
      prospective studies will help us to understand better the relationship of these 
      tumors to MEN1, in addition to verifying the true prevalence and penetrance of 
      the well-documented neoplastic associations. Nevertheless, patients affected by 
      MEN1 should be aware of these non-endocrine manifestations, and providers should 
      be encouraged always to think beyond the "3 Ps" when treating an MEN1 patient.
CI  - Copyright (c) 2022 Waguespack.
FAU - Waguespack, Steven G
AU  - Waguespack SG
AD  - Department of Endocrine Neoplasia and Hormonal Disorders and the Children's 
      Cancer Hospital, The University of Texas MD Anderson Cancer Center, Houston, TX, 
      United States.
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20221017
PL  - Switzerland
TA  - Front Endocrinol (Lausanne)
JT  - Frontiers in endocrinology
JID - 101555782
SB  - IM
MH  - Humans
MH  - *Multiple Endocrine Neoplasia Type 1/complications/genetics/pathology
MH  - Prospective Studies
MH  - *Angiofibroma/pathology
MH  - *Islets of Langerhans/pathology
MH  - Multicenter Studies as Topic
PMC - PMC9618614
OTO - NOTNLM
OT  - MEN1
OT  - angiofibroma
OT  - breast cancer
OT  - collagenoma
OT  - ependymoma
OT  - leiomyoma
OT  - lipoma
OT  - meningioma
COIS- The author declares that the research was conducted in the absence of any 
      commercial or financial relationships that could be construed as a potential 
      conflict of interest.
EDAT- 2022/11/04 06:00
MHDA- 2022/11/05 06:00
PMCR- 2022/01/01
CRDT- 2022/11/03 02:48
PHST- 2022/08/26 00:00 [received]
PHST- 2022/09/26 00:00 [accepted]
PHST- 2022/11/03 02:48 [entrez]
PHST- 2022/11/04 06:00 [pubmed]
PHST- 2022/11/05 06:00 [medline]
PHST- 2022/01/01 00:00 [pmc-release]
AID - 10.3389/fendo.2022.1029041 [doi]
PST - epublish
SO  - Front Endocrinol (Lausanne). 2022 Oct 17;13:1029041. doi: 
      10.3389/fendo.2022.1029041. eCollection 2022.