PMID- 32229700
OWN - NLM
STAT- MEDLINE
DCOM- 20210823
LR  - 20210823
IS  - 1479-6821 (Electronic)
IS  - 1351-0088 (Print)
IS  - 1351-0088 (Linking)
VI  - 27
IP  - 6
DP  - 2020 Jun
TI  - Prognostic factors for the outcome of nonfunctioning pancreatic neuroendocrine 
      tumors in MEN1: a systematic review of literature.
PG  - R145-R161
LID - ERC-19-0372 [pii]
LID - 10.1530/ERC-19-0372 [doi]
AB  - Metastatic duodenopancreatic neuro-endocrine tumors (dpNETs) are the most 
      important disease-related cause of death in patients with multiple endocrine 
      neoplasia type 1 (MEN1). Nonfunctioning pNETs (NF-pNETs) are highly prevalent in 
      MEN1 and clinically heterogeneous. Therefore, management is controversial. Data 
      on prognostic factors for risk stratification are limited. This systematic review 
      aims to establish the current state of evidence regarding prognostic factors in 
      MEN1-related NF-pNETs. We systematically searched four databases for studies 
      assessing prognostic value of any factor on NF-pNET progression, development of 
      distant metastases, and/or overall survival. In- and exclusion, critical 
      appraisal and data-extraction were performed independently by two authors 
      according to pre-defined criteria. Thirteen studies (370 unique patients) were 
      included. Prognostic factors investigated were tumor size, timing of surgical 
      resection, WHO grade, methylation, p27/p18 expression by immunohistochemistry 
      (IHC), ARX/PDX1 IHC and alternative lengthening of telomeres. Results were 
      complemented with evidence from studies in MEN1-related pNET for which data could 
      not be separately extracted for NF-pNET and data from sporadic NF-pNET. We found 
      that the most important prognostic factors used in clinical decision making in 
      MEN1-related NF-pNETs are tumor size and grade. NF-pNETs <2 cm may be managed 
      with watchful waiting, while surgical resection is advised for NF-pNETs >/=2 cm. 
      Grade 2 NF-pNETs should be considered high risk. The most promising and 
      MEN1-relevant avenues of prognostic research are multi-analyte circulating 
      biomarkers, tissue-based molecular factors and imaging-based prognostication. 
      Multi-institutional collaboration between clinical, translation and basic 
      scientists with uniform data and biospecimen collection in prospective cohorts 
      should advance the field.
FAU - Sadowski, S M
AU  - Sadowski SM
AD  - Endocrine Surgery, Surgical Oncology Program, National Cancer Institute, NIH, 
      Bethesda, Maryland, USA.
FAU - Pieterman, C R C
AU  - Pieterman CRC
AD  - Department of Surgical Oncology, Section of Surgical Endocrinology, University of 
      Texas MD Anderson Cancer Center, Houston, Texas, USA.
AD  - Department of Endocrine Oncology, University Medical Center Utrecht, Utrecht, The 
      Netherlands.
FAU - Perrier, N D
AU  - Perrier ND
AD  - Department of Surgical Oncology, Section of Surgical Endocrinology, University of 
      Texas MD Anderson Cancer Center, Houston, Texas, USA.
FAU - Triponez, F
AU  - Triponez F
AD  - Thoracic and Endocrine Surgery and Faculty of Medicine, University Hospitals of 
      Geneva, Geneva, Switzerland.
FAU - Valk, G D
AU  - Valk GD
AD  - Department of Endocrine Oncology, University Medical Center Utrecht, Utrecht, The 
      Netherlands.
LA  - eng
GR  - ZIA BC011899/ImNIH/Intramural NIH HHS/United States
PT  - Journal Article
PT  - Research Support, N.I.H., Intramural
PT  - Systematic Review
PL  - England
TA  - Endocr Relat Cancer
JT  - Endocrine-related cancer
JID - 9436481
SB  - IM
MH  - Female
MH  - Humans
MH  - Male
MH  - Multiple Endocrine Neoplasia Type 1/*genetics
MH  - Prognosis
MH  - Treatment Outcome
PMC - PMC7304521
MID - NIHMS1585339
OTO - NOTNLM
OT  - MEN1
OT  - metastases
OT  - nonfunctioning pancreatic neuroendocrine tumors
OT  - prognostic factors
OT  - survival
OT  - systematic review
EDAT- 2020/04/02 06:00
MHDA- 2021/08/24 06:00
PMCR- 2021/06/01
CRDT- 2020/04/02 06:00
PHST- 2020/03/18 00:00 [received]
PHST- 2020/03/31 00:00 [accepted]
PHST- 2020/04/02 06:00 [pubmed]
PHST- 2021/08/24 06:00 [medline]
PHST- 2020/04/02 06:00 [entrez]
PHST- 2021/06/01 00:00 [pmc-release]
AID - ERC-19-0372 [pii]
AID - 10.1530/ERC-19-0372 [doi]
PST - ppublish
SO  - Endocr Relat Cancer. 2020 Jun;27(6):R145-R161. doi: 10.1530/ERC-19-0372.