Abstract 247P
Background
Gastroenteropancreatic neuroendocrine tumours (GEPNETs) are heterogenous group of rare tumours with large spectrum of natural history and biologic behaviour. GEPNETs are classified into least aggressive neuroendocrine tumour (NET) to highly proliferative neuroendocrine carcinoma (NEC). This study aimed to report clinicopathological characteristics, survival outcomes and factors affecting survival of GEPNETs patients treated at National Cancer Institute, Malaysia.
Methods
We identified 99 patients with GEPNETs who had their initial visits between 2013 and 2023. Kaplan-Meier and Cox regression methods were used for statistical analyses.
Results
The median age was 51 years old (range 16 - 80) and male-to-female ratio was 1:1. Primary sites were pancreas (42.4%), small bowel (17.2%), rectum (16.2%), colon (6.1%), appendix (6.1%), stomach (4%), ampullary (4%), and others (4%). At presentation, 53.5% had locoregional disease. Majority presented with abdominal pain (38.4%) or gastrointestinal hemorrhage (15.2%). Pathological distribution demonstrated 80.8% and 19.2% were NET and NEC respectively. Surgery was performed in 51.5% of the cohort (curative; n = 47, palliative; n = 4). As for NET, 70 patients were given somatostatin analogue as first line palliative treatment. For subsequent line of treatment, 20 received Peptide Receptor Radionuclide Therapy (PRRT), 10 received Everolimus and 5 received chemotherapy (Capecitabine; n = 3, Temozolomide; n = 2). As for NEC, 14 patients received chemotherapy (Etoposide/platinum; n = 12, Irinotecan/platinum; n = 2). The median follow-up for entire cohort was 35.2 months (range 0.5 - 180.9). The median overall survival for NET and NEC were 89.7 and 16.8 months respectively. The 5-year survival rates for NET and NEC were 59.9% and 23.4% respectively. On multivariate analysis; age, primary sites, stage at presentation and ki 67% were predictors of survival.
Conclusions
GEPNETs are a diverse group of tumours ranging from long survival of well differentiated NET to the dismal prognosis of high grade NEC.
Clinical trial identification
Editorial acknowledgement
Legal entity responsible for the study
The authors.
Funding
Has not received any funding.
Disclosure
All authors have declared no conflicts of interest.