Epidemiology and Treatment Patterns among Patients with Pancreatic Neuroendocrine Tumours: Results from Latvian GEP-NET Study Group

Abstract

Introduction: Pancreatic neuroendocrine tumours (pNET) comprise a rare, heterogeneous subgroup of neoplasms with relatively favourable prognosis in comparison to other malignancies of the pancreas. Thereby, efficacy of treatment of pNET requires experience and multidisciplinary approach. The aim of this study was to analyse the incidence, management and survival outcomes of pNET in a tertiary referral hospitals in Latvia. Method(s): Patients with confirmed pNET at Riga East Clinical University Hospital and Pauls Stradins Clinical University Hospital between 2006 and 2019 were retrospectively analysed. Data were collected and included by trained study personnel into EUROCRINE, an open-label international Endocrine Surgical registry. Result(s): In total, 83 patients were included. The median age at diagnosis was 62 (IQR 70-53) years, 68% (n = 56) were females. The age-adjusted incidence rate of pNET increased from 0.05 to 0.73 per 100 000 persons between 2006 and 2019. The most common location was head (40.9%), followed by tail (36.1%) and body (23%) of the pancreas. In 77% (n = 64) tumours were hormonally non-functional. Among those with functioning pNET, 58% (n = 11) presented with hypoglycaemia and were diagnosed with insulinoma, 32% (n = 6) were presented with symptoms related to classical carcinoid syndrome. pNET were all sporadic, 34% (n = 28) initially presented with distant metastases. According to Ki-67 distribution, G1 tumours were 36% (n = 30), G2 41% (n = 34), G3 6% (n = 5), in 17% (n = 14) correct grading was difficult to obtain. The majority of patients 75% (n = 62) received an operation as a first-line therapy, 3% (n = 2) of patients underwent simultaneous resection of liver metastases. For the resected patients the overall morbidity rate was 17.7% (n = 11). For two patients with pNET < 2 cm watch and wait policy was used. Only 19 patients with advanced disease were treated with systemic therapy: including 11 with SSA, six with chemotherapy, two with PRRT. The median follow-up period of the entire cohort was 34 (IQR 64-13) months. The OS rate at 1- and 3-year was 90.2% and 88.6%, respectively. The overall morbidity rate for surgically treated patients was 17.7% (n = 11), with a mortality rate 3.2% (n = 2). Meanwhile, recurrence rate was 8.1% (n = 5) in patients with resected tumours. Conclusion(s): Increased incidence of pNET in Latvia during the 14-year period is observed. pNET represent a heterogenous group of tumours with relatively good long term survival rates.Copyright © 2021