Long-term outcomes in MEN-1 patients with pancreatic neuroendocrine neoplasms: an Israeli specialist center experience

Autor: Oleinikov, Kira, Uri, Inbal, Jacob, Harold, Epshtein, Julia, Benson, Ariel, Ben-Haim, Simona, Atlan, Karine, Tal, Ilanit, Meirovitz, Amichay, Maimon, Ofra, Lev-Cohain, Naama, Mazeh, Haggi, Glaser, Benjamin, Gross, David J., Grozinsky-Glasberg, Simona
Zdroj: Endocrine; April 2020, Vol. 68 Issue: 1 p222-229, 8p
Abstrakt: Purpose: The decreased life expectancy of MEN-1 patients is mainly related to pancreatic neuroendocrine tumors (pNETs). At best, limited data is available on the natural history of MEN-1-associated pNETs, as these tumors are rare and have a wide range of biologic behavior. Our study aims to explore the clinical course of patients with MEN-1-associated pNETs and the long-term outcomes. Methods: This longitudinal study was conducted on the MEN-1 cohort treated at our referral center over a 22-year period (1996–2018). Relevant clinical data were retrospectively analysed. Results: Among the 33 MEN-1 patients included in our study, pNETs were identified in 21 subjects with a penetrance of 48% by the age of 50. Non-functioning and functioning pNETs were diagnosed in sixteen (76%) and five (24%) patients, respectively. Two-thirds of the patients had multifocal tumors. The median number of pancreatic macroscopic lesions per individual was 4.0 ± 3.9 (range 1–8) with a mean size of 1.3 ± 2.1 cm (range 0.5–10). The metastatic rate according to the dominant pNET lesion reached 100%, 62% and 6% for tumors sized > 4 cm, 2.1–4 cm, and 1–2 cm, respectively. Over the study period, one or more therapeutic interventions for pNETs were required in 20 out of the 21 patients. pNET-related metastatic complication was the main cause of death within this MEN-1 cohort. The overall survival rate for the pNETs patients was 86% during a mean follow-up period of 8.0 ± 4.6 years. Conclusions: In our MEN-1 cohort, non-functioning pNETs were the most frequent type of pancreaticoduodenal tumor, and the tumor size correlated with the risks of metastasis and death. Increased awareness, early diagnosis, and a multidisciplinary approach may improve the associated morbidity and mortality in these patients.
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