Endocrine Abstracts

Introduction: GEP-NENs represent a heterogeneous group of neoplasms with increasing incidence in the last years. Pathogenesis and risk factors of sporadic GEP-NENs are not clear and still debated. The aim of this study is to evaluate the main risk factors for sporadic GEP-NENs in patients followed by the NETTARE Unit (NeuroEndocrine TAsk force of of ‘Sapienza’ University of Rome).

Methods: We performed a case-control study including 32 consecutive sporadic GEP-NENs and 109 age- and sex-matched controls, affected by benign thyroid or adrenal non-functioning nodules. We collected data on demographic aspects, clinical features and potential risk factors (family history of cancer, smoking, alcohol, body mass index, age of menopause, use and duration of contraceptive, hormonal replacement therapy, personal history of cancer, diabetes mellitus, inflammatory bowel disease, celiac disease, autoimmune atrophic gastritis and pancreatitis). The two groups were compared using χ² test for comparison of proportions for categorical variables, Student’s t-test for normally distributed continuous variables and Mann-Witney test for non-normally distributed continuous variables.

Results: Among the 32 GEP-NENs patients mean age was 58.5±11.8 years, with F:M ratio of 1.3 (18 females and 14 males). The most frequent tumor site was pancreas (65.6%), followed by ileum (28.1%). Most patients had a well-differentiated NEN, 78.1% G1 neuroendocrine tumors (NET), 15.6% G2 (NET), while 6.2% had a neuroendocrine carcinoma (NEC). Only the 28.1% had locally advanced or metastatic disease at diagnosis (TNM stage III or IV). A family history of cancer was more frequent in GEP-NENs group than in the control one (90.6% vs 55.0%; P<0.001). Particularly, the percentage of GEP-NENs patients with a family history of non-endocrine GEP cancer was significantly higher than controls (34.4% vs 13.8%, P=0.008). Lung (21.9%), colon-rectum (21.9%) and breast (12.5%) were the most common non-endocrine cancer sites in the family history of GEP-NENs patients. No other risk factors were found significantly different between the two groups.

Conclusions: This case-control study suggests that a family history of cancer, particularly of non-endocrine GEP tumor, is associated with GEP-NENs in the offspring. If confirmed in larger cohorts, this finding could have a significant impact on the early screening and prevention strategies for GEP-NEN.