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A classification prognostic score to predict OS in stage IV well-differentiated neuroendocrine tumors

2018; Bioscientifica; Volume: 25; Issue: 6 Linguagem: Inglês

10.1530/erc-17-0489

1479-6821

Sara Pusceddu, Francesco Barretta, Annalisa Trama, Laura Botta, Massimo Milione, Roberto Buzzoni, Filippo de Braud, Vincenzo Mazzaferro, Ugo Pastorino, Ettore Seregni, Luigi Mariani, Gemma Gatta, Maria Di Bartolomeo, Daniela Femia, Natalie Prinzi, Jorgelina Coppa, Francesco Panzuto, Lorenzo Antonuzzo, Emilio Bajetta, Maria Pia Brizzi, Davide Campana, Laura Catena, Harry Comber, Fiona Dwane, Nicola Fazio, Antongiulio Faggiano, Dario Giuffrida, K. Henau, Toni Ibrahim, Riccardo Marconcini, Sara Massironi, Maja Primic Žakelj, Francesca Spada, Salvatore Tafuto, E. Van Eycken, Jan Maaten Van der Zwan, Tina Žagar, Luca Giacomelli, Rosalba Miceli, _ _, _ _, Francesca Aroldi, Alberto Bongiovanni, Rossana Berardi, Nicole Brighi, Sara Cingarlini, Carolina Cauchi, Federica Cavalcoli, Carlo Carnaghi, Francesca Corti, Marilina Duro, Maria Vittoria Davì, Chiara De Divitiis, Paola Ermacora, Anna La Salvia, Gabriele Luppi, Giuseppe Lo Russo, Federico Nichetti, Alessandra Raimondi, Vittorio Perfetti, Paola Razzore, Maria Rinzivillo, Sabine Siesling, Martina Torchio, Boukje A. C. van Dijk, Otto Visser, Claudio Vernieri,

Neuroblastoma Research and Treatments

No validated prognostic tool is available for predicting overall survival (OS) of patients with well-differentiated neuroendocrine tumors (WDNETs). This study, conducted in three independent cohorts of patients from five different European countries, aimed to develop and validate a classification prognostic score for OS in patients with stage IV WDNETs. We retrospectively collected data on 1387 patients: (i) patients treated at the Istituto Nazionale Tumori (Milan, Italy; n = 515); (ii) European cohort of rare NET patients included in the European RARECAREnet database (n = 457); (iii) Italian multicentric cohort of pancreatic NET (pNETs) patients treated at 24 Italian institutions (n = 415). The score was developed using data from patients included in cohort (i) (training set); external validation was performed by applying the score to the data of the two independent cohorts (ii) and (iii) evaluating both calibration and discriminative ability (Harrell C statistic). We used data on age, primary tumor site, metastasis (synchronous vs metachronous), Ki-67, functional status and primary surgery to build the score, which was developed for classifying patients into three groups with differential 10-year OS: (I) favorable risk group: 10-year OS ≥70%; (II) intermediate risk group: 30% ≤ 10-year OS < 70%; (III) poor risk group: 10-year OS <30%. The Harrell C statistic was 0.661 in the training set, and 0.626 and 0.601 in the RARECAREnet and Italian multicentric validation sets, respectively. In conclusion, based on the analysis of three 'field-practice' cohorts collected in different settings, we defined and validated a prognostic score to classify patients into three groups with different long-term prognoses.