PMID- 33878789
OWN - NLM
STAT- MEDLINE
DCOM- 20211217
LR  - 20211217
IS  - 1439-4286 (Electronic)
IS  - 0018-5043 (Linking)
VI  - 53
IP  - 5
DP  - 2021 May
TI  - Distinct Prognostic Factors in Sporadic and Multiple Endocrine Neoplasia Type 
      1-Related Pancreatic Neuroendocrine Tumors.
PG  - 319-325
LID - 10.1055/a-1464-1276 [doi]
AB  - Pancreatic neuroendocrine tumors (PNET) may develop sporadically or in the 
      context of hereditary syndromes. In patients with multiple endocrine neoplasia 
      type 1 (MEN1), PNET is the leading cause of death. Our aim was to compare the 
      mortality risk in sporadic and MEN1-related PNETs and identify high-risk 
      populations. A retrospective Surveillance, Epidemiology, and End Results database 
      analysis of patients with PNET was used. Patients with MEN1 were defined by 
      syn/metachronous pituitary adenoma. Clinical data were retrieved, and all-cause 
      mortality (ACM) risk was compared in univariate and multivariable analyses. The 
      cohort included 569 patients (46.6% males) with sporadic (n=542) and MEN1-related 
      (n=27) PNETs. Age at diagnosis of MEN1-related PNET was significantly younger 
      than with sporadic PNETs (mean age 49.2+/-16.7 vs. 61.6+/-12.7 years, respectively; p 
      < 0.001). Survival analysis showed a trend for a better outcome in patients with 
      MEN1-related vs. sporadic PNET (Log-rank, p=0.09) and in subgroup analysis for 
      patients with advanced disease (p=0.08). Furthermore, among patients followed 
      expectantly, those with MEN1-related PNET had lower ACM risk than their sporadic 
      counterparts (p=0.08). Multivariable analysis demonstrated lower ACM risk in 
      patients diagnosed with MEN1 (hazard ratio 0.35, 95% confidence interval 
      0.11-1.2, p=0.09), further supporting the trend detected in the univariate 
      analysis. In conclusion, our study demonstrates the distinct clinical profile of 
      patients with MEN1-related PNET compared to sporadic disease and emphasizes the 
      expertise required to accurately manage patients with PNET in this rare context. 
      The cautious decision-making required before embarking on surgical intervention 
      is further emphasized in this robust analysis of a large cancer database.
CI  - Thieme. All rights reserved.
FAU - Kfir, Sapir Kon
AU  - Kfir SK
AD  - Department of Internal Medicine D, Sheba Medical Center at Tel Hashomer, Tel 
      Hashomer, Israel.
FAU - Halperin, Reut
AU  - Halperin R
AD  - Department of Internal Medicine D, Sheba Medical Center at Tel Hashomer, Tel 
      Hashomer, Israel.
AD  - Division of Endocrinology and Metabolism, Sheba Medical Center at Tel Hashomer, 
      Tel Hashomer, Israel.
FAU - Percik, Ruth
AU  - Percik R
AD  - Division of Endocrinology and Metabolism, Sheba Medical Center at Tel Hashomer, 
      Tel Hashomer, Israel.
AD  - Endo-oncology Clinic, Cancer Center, Sheba Medical Center at Tel Hashomer, Tel 
      Hashomer, Israel.
AD  - Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
FAU - Uri, Inbal
AU  - Uri I
AD  - Division of Endocrinology and Metabolism, Sheba Medical Center at Tel Hashomer, 
      Tel Hashomer, Israel.
AD  - Endo-oncology Clinic, Cancer Center, Sheba Medical Center at Tel Hashomer, Tel 
      Hashomer, Israel.
AD  - Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
FAU - Halpern, Naama
AU  - Halpern N
AD  - Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
AD  - GI Unit, Cancer Center, Sheba Medical Center at Tel Hashomer, Tel Hashomer, 
      Israel.
FAU - Shlomai, Gadi
AU  - Shlomai G
AD  - Department of Internal Medicine D, Sheba Medical Center at Tel Hashomer, Tel 
      Hashomer, Israel.
AD  - Division of Endocrinology and Metabolism, Sheba Medical Center at Tel Hashomer, 
      Tel Hashomer, Israel.
AD  - Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
FAU - Laish, Ido
AU  - Laish I
AD  - Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
AD  - Gastroenterology Institute, Sheba Medical Center at Tel Hashomer, Tel Hashomer, 
      Israel.
FAU - Tirosh, Amir
AU  - Tirosh A
AD  - Division of Endocrinology and Metabolism, Sheba Medical Center at Tel Hashomer, 
      Tel Hashomer, Israel.
AD  - Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
FAU - Tirosh, Amit
AU  - Tirosh A
AUID- ORCID: 0000-0003-3794-9634
AD  - Division of Endocrinology and Metabolism, Sheba Medical Center at Tel Hashomer, 
      Tel Hashomer, Israel.
AD  - Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
AD  - Neuroendocrine Tumors Service, Sheba Medical Center at Tel Hashomer, Tel 
      Hashomer, Israel.
LA  - eng
PT  - Journal Article
DEP - 20210420
PL  - Germany
TA  - Horm Metab Res
JT  - Hormone and metabolic research = Hormon- und Stoffwechselforschung = Hormones et 
      metabolisme
JID - 0177722
SB  - IM
MH  - Adult
MH  - Aged
MH  - Female
MH  - Humans
MH  - Kaplan-Meier Estimate
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/diagnosis/*mortality
MH  - Neuroendocrine Tumors/diagnosis/*mortality
MH  - Pancreatic Neoplasms/diagnosis/*mortality
MH  - Prognosis
MH  - Retrospective Studies
MH  - Young Adult
COIS- The authors declare that they have no conflict of interest.
EDAT- 2021/04/21 06:00
MHDA- 2021/12/18 06:00
CRDT- 2021/04/20 20:25
PHST- 2021/04/21 06:00 [pubmed]
PHST- 2021/12/18 06:00 [medline]
PHST- 2021/04/20 20:25 [entrez]
AID - 10.1055/a-1464-1276 [doi]
PST - ppublish
SO  - Horm Metab Res. 2021 May;53(5):319-325. doi: 10.1055/a-1464-1276. Epub 2021 Apr 
      20.