Introduction: Clinicopathological characterisation ofneuroendocrine neoplasms could provide improved prognostic information even at the time of the diagnosis.
Objectives: The aim of this study was to characterise the clinicopathological features of a large cohort of patients with neuroendocrine neoplasms (NENs).
Patients and methods: The study included 210 patients (95 men and 115 women) with histologically confirmed and verified neuroendocrine neoplasms in our single tertiary referral endocrine unit. Descriptive statistics were performed providing summaries of selected clinical and pathological parameters at the time of diagnosis. Factors contributing to survival were analysed. A p value of less than 0.05 was considered statistically significant.
Results: The mean age at diagnosis was 54.7±16.3 years for men and 52.3±15.1 years for women. The most common primary tumour sites were the pancreas (n=74, 35.2%), the ileocecal region (n=34; 16.2%) and the lungs (n=29; 13.8%), respectively. In 12.6% of patients, the primary tumour site remained unidentified. The mean follow-up time of patients was 4.6 years. Original histopathological reports were available in 128 patients (60.9%) who were classified according to the 2010 WHO criteria. The most common grade was grade 2 (n=55; 43%), while grade 1 and grade 3 were diagnosed in 52 (40.6%) and 21 (16.4%) patients, respectively. Sufficient clinical data for staging were available in 169 patients, who were classified according to the 2009 American Joint Committee on Cancer/Union for International Cancer Control (AJCC/UICC) recommendations. The majority of patients had been presented with advanced, metastatic disease in stage IV (n=122; 72.2%). 33 patients were diagnosed in stage I, while stages II and III constituted in 7-7 patients. 5-year survival was poorer in patients with grade 3 and in stage IV tumours. According to univariate Cox regression model male gender, tumours with grade 3 and stage IV and increased 5-HIAA urinary excretion was associated with poorer prognosis. The most frequent hormone-producing tumours were insulin-, gastrin- and ACTH-producing tumours as well as tumours causing carcinoid syndrome.
Conclusion: The overrepresentation of patients with tumours in a higher stage and grade reflects the specified tertiary feature of our centre. Our results confirm previous data that higher tumour grade and stage, the presence of metastases at diagnosis are the most important factors influencing the prognosis. Patients presented with carcinoid syndrome also have a poorer overall survival.
18 - 21 May 2019
European Society of Endocrinology