PMID- 33905872
OWN - NLM
STAT- MEDLINE
DCOM- 20220308
LR  - 20220308
IS  - 1096-3650 (Electronic)
IS  - 1044-579X (Linking)
VI  - 79
DP  - 2022 Feb
TI  - Multiple endocrine neoplasia type 1 (MEN-1) and neuroendocrine neoplasms (NENs).
PG  - 141-162
LID - S1044-579X(21)00112-7 [pii]
LID - 10.1016/j.semcancer.2021.04.011 [doi]
AB  - Neuroendocrine neoplasms (NENs) are relatively rare neoplasms with 6.4-times 
      increasing age-adjusted annual incidence during the last four decades. NENs arise 
      from neuroendocrine cells, which release hormones in response to neuronal stimuli 
      and they are distributed into organs and tissues. The presentation and biological 
      behaviour of the NENs are highly heterogeneous, depending on the organ. The 
      increased incidence is mainly due to increased awareness and improved detection 
      methods both in the majority of sporadic NENs (non-inherited), but also the 
      inherited groups of neoplasms appearing in at least ten genetic syndromes. The 
      most important one is multiple endocrine neoplasia type 1 (MEN-1), caused by 
      mutations in the tumour suppressor gene MEN1. MEN-1 has been associated with 
      different tumour manifestations of NENs e.g. pancreas, gastrointestinal tract, 
      lungs, thymus and pituitary. Pancreatic NENs tend to be less aggressive when 
      arising in the setting of MEN-1 compared to sporadic pancreatic NENs. There have 
      been very important improvements over the past years in both genotyping, genetic 
      counselling and family screening, introduction and validation of various relevant 
      biomarkers, as well as newer imaging modalities. Alongside this development, both 
      medical, surgical and radionuclide treatments have also advanced and improved 
      morbidity, quality of life and mortality in many of these patients. Despite this 
      progress, there is still space for improving insight into the genetic and 
      epigenetic factors in relation to the biological mechanisms determining NENs as 
      part of MEN-1. This review gives a comprehensive update of current evidence for 
      co-occurrence, diagnosis and treatment of MEN-1 and neuroendocrine neoplasms and 
      highlight the important progress now finding its way to international guidelines 
      in order to improve the global management of these patients.
CI  - Copyright (c) 2021. Published by Elsevier Ltd.
FAU - Effraimidis, Grigoris
AU  - Effraimidis G
AD  - ENETS Neuroendocrine Tumor Centre of Excellence, Rigshospitalet, Copenhagen 
      University Hospital, Denmark; Department of Medical Endocrinology and Metabolism, 
      Rigshospitalet, Copenhagen University Hospital, Denmark.
FAU - Knigge, Ulrich
AU  - Knigge U
AD  - ENETS Neuroendocrine Tumor Centre of Excellence, Rigshospitalet, Copenhagen 
      University Hospital, Denmark; Department of Medical Endocrinology and Metabolism, 
      Rigshospitalet, Copenhagen University Hospital, Denmark; Department of Surgery 
      and Transplantation, Rigshospitalet, Copenhagen University Hospital, Denmark.
FAU - Rossing, Maria
AU  - Rossing M
AD  - Centre for Genomic Medicine, Rigshospitalet, Copenhagen University Hospital, 
      Denmark.
FAU - Oturai, Peter
AU  - Oturai P
AD  - Department of Clinical Physiology, Nuclear Medicine & PET, Rigshospitalet, 
      Copenhagen University Hospital, Denmark.
FAU - Rasmussen, Ase Krogh
AU  - Rasmussen AK
AD  - ENETS Neuroendocrine Tumor Centre of Excellence, Rigshospitalet, Copenhagen 
      University Hospital, Denmark; Department of Medical Endocrinology and Metabolism, 
      Rigshospitalet, Copenhagen University Hospital, Denmark.
FAU - Feldt-Rasmussen, Ulla
AU  - Feldt-Rasmussen U
AD  - ENETS Neuroendocrine Tumor Centre of Excellence, Rigshospitalet, Copenhagen 
      University Hospital, Denmark; Department of Medical Endocrinology and Metabolism, 
      Rigshospitalet, Copenhagen University Hospital, Denmark; Institute of Clinical 
      Medicine, Faculty of Health Sciences, Copenhagen University, Denmark. Electronic 
      address: ufeldt@rh.dk.
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Review
DEP - 20210424
PL  - England
TA  - Semin Cancer Biol
JT  - Seminars in cancer biology
JID - 9010218
RN  - 0 (Biomarkers, Tumor)
RN  - 0 (MEN1 protein, human)
RN  - 0 (Proto-Oncogene Proteins)
SB  - IM
MH  - Biomarkers, Tumor/genetics
MH  - Diagnostic Techniques, Radioisotope
MH  - Gastrinoma/pathology
MH  - Gastrointestinal Neoplasms/pathology
MH  - Genetic Predisposition to Disease/genetics
MH  - Humans
MH  - Lung Neoplasms/pathology
MH  - Multiple Endocrine Neoplasia Type 1/diagnosis/*genetics/*therapy
MH  - Neuroendocrine Tumors/diagnosis/*genetics/*therapy
MH  - Pancreatic Neoplasms/pathology
MH  - Proto-Oncogene Proteins/*genetics
OTO - NOTNLM
OT  - Biomarkers
OT  - Imaging
OT  - Multiple endocrine neoplasia
OT  - Neuroendocrine tumours
OT  - Treatment
EDAT- 2021/04/28 06:00
MHDA- 2022/03/09 06:00
CRDT- 2021/04/27 20:12
PHST- 2020/08/20 00:00 [received]
PHST- 2021/01/03 00:00 [revised]
PHST- 2021/04/16 00:00 [accepted]
PHST- 2021/04/28 06:00 [pubmed]
PHST- 2022/03/09 06:00 [medline]
PHST- 2021/04/27 20:12 [entrez]
AID - S1044-579X(21)00112-7 [pii]
AID - 10.1016/j.semcancer.2021.04.011 [doi]
PST - ppublish
SO  - Semin Cancer Biol. 2022 Feb;79:141-162. doi: 10.1016/j.semcancer.2021.04.011. 
      Epub 2021 Apr 24.