Clinicopathologic characterization and prognostic factor in extraintestinal neuroendocrine tumors.
e15131 Background: The frequency, prognostic factors and long-term survival of E-NET are poorly known. Methods: A retrospective analysis of clinical, histological characteristics and survival of patients with E-NET were done. Survival analyses were assessed by the Kaplan-Meier method and prognostic factors of overall survival were tested by uni/multivariate analysis. Results: Between 1997-2010, 241 patients were identified with NET. 31 Patients were E-NET (12.8%): median age was 55 years (16-79) and 55% were females. Primary location was lung, 58%, prostate 13%, breast 10%, urinary bladder 7%, and others 12%. At diagnosis 87% patients had symptoms and 52% were localized. A functioning tumor was in 7 patients (6 ACTH). Initial treatment was surgery 61%, chemotherapy 26% (23% had objective response) and palliative care 13%. After complete resection 4/14 patients recurred. With follow-up of 15months, the cancer-specific mortality was 55%. Overall 1- and 5-year survival were 72% and 35% respectively, which were lower than survival for GEP-NET (p=0.001, Figure 1). 5 year survival differed significantly according to age at diagnosis (47% ≤50 vs 27% >50 years); location (60% lung vs 10% non-lung); extension (58% localized vs 15% metastatic/locally advanced); stage at diagnosis (59% stage I-II vs 13% III-IV); histology (52% NET/carcinoid vs 14% NEC); degree of differentiation (44% well/moderately vs 0% poorly differentiated); initial treatment (55% surgery, 25% palliative care and 0% chemotherapy); and recurrence (100% absent vs 25% present). In univariate analysis, the negative prognostic factors were age >50 years, no lung site, symptomatic, metastatic/locally advanced disease, extrahepatic metastases, NEC, poor differentiation and recurrence. In multivariate analysis, only age >50 years was an independent predictor of survival (p=0.027). Conclusions: In our experience, E-NET represent 13% of all the NET. Present symptoms with a fewer frequency, are less functional, have more frequently metastases, and a worst prognosis (5-year survival 34 vs 72%). The most important prognostic factor for overall survival was age >50 years.