Objective: Gastroenteropancreatic neuroendocrine tumors (GEP-NETs) are relatively infrequent. We present a descriptive study of GEP-NETs of a reference Spanish hospital.
Patients and methods: Forty-six patients were diagnosed of GEP-NETs during the period 19932011. Data regarding demographic, clinical, analytical, anatomopathological and diagnostic variables were collected. Results are expressed as mean±SD.
Results: Twenty-seven (58.7%) were men. The mean age at diagnosis was 44.5 (15.5) years. 82.6% were sporadic, multiple endocrine neoplasia type 1 was diagnosed in 15.2% and neurofibromatosis type 1 in 2.2%. Localization was 48.9% pancreatic, 40% gastrointestinal tract, 6.7% bronchial and 4.4% thymic. Incidental diagnosis occurred in 11.4% of the cases, 62.9% presented hormone hypersecretion symptoms. In those with clinical symptoms, the mean time until diagnosis was of 7.4 (12.5) months with a median of 3 months.
At diagnosis 69% of the cases presented distant metastases. The most common site of metastases was liver (100%). The most commonly carried out imaging studies for the primary tumor and metastases was computed tomography (CT) scan 89.1 and 100% respectively. Overall, about 97.7% of the patients underwent surgery most of them with curative intent. 33.8% underwent hepatic transplant. 55.5% were treated with somatostatin analogues, 19.4% with chemotherapy, 7.5% with lutetium and 2.5% with itrium.
After a mean follow-up of 7.2 (5.0) years for pancreatic NET and 8.8 (5.3) years for the intestinal NET, the survival was 81 and 66.7% respectively.
Conclusions: The NETs of pancreatic location are the most common in our series. Metastatic disease is frequent during follow-up and in most NETs is already present at diagnosis. Surgery is the first-line treatment for the NETs regardless of their location. Up to one third of the patients received a hepatic transplant, being this method a real option for those patients with metastases confined to the liver.
27 Apr - 01 May 2013
European Society of Endocrinology