Horm Metab Res 2021; 53(05): 319-325
DOI: 10.1055/a-1464-1276
Endocrine Care

Distinct Prognostic Factors in Sporadic and Multiple Endocrine Neoplasia Type 1-Related Pancreatic Neuroendocrine Tumors

Sapir Kon Kfir
1  Department of Internal Medicine D, Sheba Medical Center at Tel Hashomer, Tel Hashomer, Israel
,
Reut Halperin
1  Department of Internal Medicine D, Sheba Medical Center at Tel Hashomer, Tel Hashomer, Israel
2  Division of Endocrinology and Metabolism, Sheba Medical Center at Tel Hashomer, Tel Hashomer, Israel
,
Ruth Percik
2  Division of Endocrinology and Metabolism, Sheba Medical Center at Tel Hashomer, Tel Hashomer, Israel
3  Endo-oncology Clinic, Cancer Center, Sheba Medical Center at Tel Hashomer, Tel Hashomer, Israel
4  Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
,
Inbal Uri
2  Division of Endocrinology and Metabolism, Sheba Medical Center at Tel Hashomer, Tel Hashomer, Israel
3  Endo-oncology Clinic, Cancer Center, Sheba Medical Center at Tel Hashomer, Tel Hashomer, Israel
4  Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
,
Naama Halpern
4  Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
5  GI Unit, Cancer Center, Sheba Medical Center at Tel Hashomer, Tel Hashomer, Israel
,
Gadi Shlomai
1  Department of Internal Medicine D, Sheba Medical Center at Tel Hashomer, Tel Hashomer, Israel
2  Division of Endocrinology and Metabolism, Sheba Medical Center at Tel Hashomer, Tel Hashomer, Israel
4  Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
,
Ido Laish
4  Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
6  Gastroenterology Institute, Sheba Medical Center at Tel Hashomer, Tel Hashomer, Israel
,
Amir Tirosh
2  Division of Endocrinology and Metabolism, Sheba Medical Center at Tel Hashomer, Tel Hashomer, Israel
4  Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
,
2  Division of Endocrinology and Metabolism, Sheba Medical Center at Tel Hashomer, Tel Hashomer, Israel
4  Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
7  Neuroendocrine Tumors Service, Sheba Medical Center at Tel Hashomer, Tel Hashomer, Israel
› Author Affiliations

Abstract

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.

Supplementary Material



Publication History

Received: 24 January 2021

Accepted after revision: 17 March 2021

Publication Date:
20 April 2021 (online)

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