PMID- 29383428
OWN - NLM
STAT- MEDLINE
DCOM- 20180822
LR  - 20181202
IS  - 1432-2323 (Electronic)
IS  - 0364-2313 (Linking)
VI  - 42
IP  - 4
DP  - 2018 Apr
TI  - From Initial Description by Wermer to Present-Day MEN1: What have We Learned?
PG  - 1031-1035
LID - 10.1007/s00268-017-4435-3 [doi]
AB  - INTRODUCTION: Pancreas, parathyroid, and pituitary, are referred to as the "3 Ps" 
      of MEN1. The time has come to move beyond those Ps and begin to discuss (1) 
      prediction, (2) pausing progression, and (3) prevention of MEN1. METHODS: In 
      preparation for the International Association of Endocrine Surgeons State of the 
      Art address, updates and uncertainties of MEN were reviewed. This included a 
      detailed examination of the MEN1 gene and the library of implicated mutations, 
      exon sequencing databases and cell cycle pathways. Therapeutic options including 
      radiofrequency ablation, systemic therapy, peptide receptor radionuclide therapy, 
      immune checkpoint inhibitor mechanisms and preimplantation genetic testing were 
      described. RESULTS: Several key points included mutations in exon 2 are suspected 
      of being associated with a higher rate of distant metastases, a higher rate of 
      PNET development, and more aggressive disease. The suggestion that missense 
      mutations involving loss of interaction with CHES1 (associated with DNA repair) 
      correlates with more aggressive disease and is more closely associated with death 
      related to PNET than to death from other causes was mentioned. For advanced NETs, 
      optimism for agents under study include lanreotide, a long-acting somatostatin 
      analog, and everolimus (Afinitor), a mammalian target of rapamycin (mTOR) 
      inhibitor. The NETest shows the potential value of being a multidimensional tumor 
      marker for response to therapy. Preimplantation genetic diagnosis (PGD) is 
      applicable. CONCLUSION: Adjunct modalities and determination of the effect of 
      therapy for MEN1 is needed. Prediction through early detection of aggressive 
      disease is an idea worth spreading. We are called us to engage with our patients 
      about prevention, the only true cure.
FAU - Perrier, Nancy D
AU  - Perrier ND
AD  - Department of Surgical Oncology, Unit 1484, The University of Texas MD Anderson 
      Cancer Center, 1515 Holcombe Boulevard, Houston, TX, 77030, USA. 
      nperrier@mdanderson.org.
LA  - eng
PT  - Journal Article
PT  - Review
PL  - United States
TA  - World J Surg
JT  - World journal of surgery
JID - 7704052
RN  - 0 (Biomarkers, Tumor)
RN  - 0 (MEN1 protein, human)
RN  - 0 (Proto-Oncogene Proteins)
SB  - IM
MH  - Biomarkers, Tumor/genetics
MH  - Combined Modality Therapy
MH  - Genetic Testing
MH  - Humans
MH  - *Multiple Endocrine Neoplasia Type 1/diagnosis/genetics/therapy
MH  - Mutation
MH  - Pancreatic Neoplasms/diagnosis/genetics/therapy
MH  - Parathyroid Neoplasms/diagnosis/genetics/therapy
MH  - Pituitary Neoplasms/diagnosis/genetics/therapy
MH  - Prognosis
MH  - Proto-Oncogene Proteins/genetics
EDAT- 2018/02/01 06:00
MHDA- 2018/08/23 06:00
CRDT- 2018/02/01 06:00
PHST- 2018/02/01 06:00 [pubmed]
PHST- 2018/08/23 06:00 [medline]
PHST- 2018/02/01 06:00 [entrez]
AID - 10.1007/s00268-017-4435-3 [pii]
AID - 10.1007/s00268-017-4435-3 [doi]
PST - ppublish
SO  - World J Surg. 2018 Apr;42(4):1031-1035. doi: 10.1007/s00268-017-4435-3.