Abstract
1604 Background: The clinical behavior of neuroendocrine tumors (NET) is highly variable, but the only data available in literature on large series are mainly based on retrospective register data collected in 3-5 decades, focusing on short-term follow-up. These are clearly insufficient due to the frequent evolution in classification criteria, diagnostic techniques and the scarcity of clinical data. Methods: Data from a total of 1659 NET patient were collected from 12 Italian referral centers. A specific software was developed for the study. Only patients with histological diagnosis revised according to the WHO Classification in active long term follow-up in the period 1990-2010 were included in the study. Patients with incomplete diagnostic work-up, without clinical data or lost at follow-up were excluded. Cumulative survival were analyzed according to site of origin, histotype, grading, ki67 score, secreting pattern (functioning or not functioning) and staging. Results: At 2 yr cumulative survival do not differ between pancreatic (pNET 94.9%), gastrointestinal (GINET 94.4%) and lung NET (TNET 94.6%), functioning (93.5 %) and non functioning (93.9%), while was significantly different between T1N0M0 (98%), T1N1M0 (92.9%), T1N1M1 (90.2%) and grading (G1 98%, G2 96.8%, G3 84.1%). At 5, 10 and 15 yr, cumulative survival were respectively 90.9, 77.1, 62.7% for pNET, 88.7, 83.5, 62.7% for GINET 90.3, 80.2, 67.9% for TNET. 88.4, 77.0, 59.0% in functioning and 89.8, 79.1, 64.6% in not functioning. 96.1, 89.5 and 77.0% for T1N0M0, 88.2, 83.5, 70.4 for T1N1M0 and 82.0, 55.8 and 43.9 for T1N1M1, 93.1, 82.3, 75.4% for G1, 87.8, 64.8, 48.6% for G2, 81.4, 75.2, 40.1% for G3. Conclusions: Brief (2y) and very long term (15y) survival do not significantly differ between different site of NET origin. Functioning tumors have the same survival rate at 2 y but lower at 15 y. Staging and grading appear as the most significant prognostic factors particularly at 10 and 15 y. Long-term cumulative survival in NET results significantly higher than in historical series, probably due to the anticipation of the diagnosis and the availability of new therapeutic strategies.
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