Incidence and survival of pancreatic neuroendocrine tumours in Girona, Spain

Pancreatic neuroendocrine tumours (pNETs) are rare cancers arising from neuroendocrine cells of the pancreas. We evaluated the incidence, demographic and survival outcomes of pNETS in the Girona region. A retrospective review of patients treated between 2001-2018 in our center was undertaken. Clinical data were obtained from the hospital cancer registry and Girona cancer registry. We classified all the neuroendocrine tumors from this period and analyzed according to the 2010 World Health Organisation Classification: NET g1, NET g2 and neuroendocrine carcinoma (NEC). During this period 82 pNETs patients were treated. 34 were women (42%) and 48 were men (58%). The median age at diagnosis was 60 years. 77% of cases were classified into neuroendocrine tumors (NET) g1 and g2, and 23% into neuroendocrine carcinoma (NEC). 14% were functional tumours (5 gastrinomas, 2 insulinomas, 1 vipoma, 1 polypeptide secreting tumor, 2 carcinoid syndrome). Just 29 had metastatic disease at presentation (35%), mostly in the liver (90%). Chromogranin A (CgA) levels at diagnosis were determined in 62% of cases. It was elevated in 65% of the determinations. In terms of treatment, 34 patients were initially treated with radical surgery, chemotherapy was the front line in 18 patients (14 of them were NEC) and somatostatin analogues were used in 16 patients. 45 patients have died (5 patients died for nontumoral causes). Median Overall Survival (mOS) was 59 months. In the univariate analysis, only grade was significantly associated with OS (p=0.018). Sex, functional status, basal CgA and metastatic disease at presentation were not significant, probably because of the low number of cases. In the multivariate analysis, grade was the only factor having a significant impact on OS (HR 3.135, p=0.033). Compared to previously published data, our report showed a smaller rate of metastatic disease at presentation. Survival was poorer, but we included NEC and 5 patients with other causes of death. Management of pNET has changed in the last 10 years and a deeper revision will be performed.