Risk Factors for Disease Progression in Advanced Jejunoileal Neuroendocrine TumorsPanzuto F. · Campana D. · Fazio N. · Brizzi M.P. · Boninsegna L. · Nori F. · Di Meglio G. · Capurso G. · Scarpa A. · Dogliotti L. · De Braud F. · Tomassetti P. · Delle Fave G. · Falconi M.
aDigestive and Liver Disease Unit, ‘Sapienza’ University of Rome, Rome, bDepartment of Clinical Medicine, S. Orsola-Malpighi Hospital, University of Bologna, Bologna, cDepartment of Medicine, Medical Oncology Division, European Institute of Oncology, Milan, dMedical Oncology, A.O.U. San Luigi, Orbassano, University of Turin, Turin, eSurgery B, and fARC-NET Center for Applied Research on Cancer and Department of Pathology and Diagnostics, University of Verona, Verona, and gDepartment of Surgery, Sacro Cuore Don Calabria Hospital, Negrar, Italy
Background: Knowledge of clinical course in advanced jejunoileal neuroendocrine tumors (NETs) is poor. Aim: To investigate progression-free survival (PFS), overall survival (OS), and possible predictors for disease progression (DP) in advanced jejunoileal NETs. Patients and Methods: We carried out a multicenter, retrospective analysis of incoming patients with sporadic advanced jejunoileal NETs. PFS and OS were assessed by Kaplan-Meier analysis. Risk factors for progression were analyzed by the Cox proportional hazards method. Results: Of the 114 patients enrolled, 46.5% had functioning tumors, 93.9% had stage IV disease, and 57.3 and 42.7% were G1 and G2 tumors, respectively. During a median follow-up of 48 months (interquartile range 29–84 months), DP occurred in 61.4% of patients, after 19 months (interquartile range 10–41 months) from diagnosis. Median PFS was 36 months. The 2-year and 5-year PFS were 59 and 33%, respectively, while 5-year OS was 77.5%. Ki67 was the sole strong independent risk factor for unfavorable outcome according to multivariate analysis, being significantly associated with both PFS and OS. Conclusions: DP occurred in the majority of patients with advanced jejunoileal NETs, with median PFS being 36 months. Ki67 was a significant predictor of DP and should be considered in determining appropriate treatments and planning follow-up for these patients.