Letizia Canu, Soraya Puglisi, Paola Berchialla, Giuseppina De Filpo, Francesca Brignardello, Francesca Schiavi, Alfonso Massimiliano Ferrara, Stefania Zovato, Michaela Luconi, Anna Pia, Marialuisa Appetecchia, Emanuela Arvat, Claudio Letizia, Mauro Maccario, Mirko Parasiliti-Caprino, Barbara Altieri, Antongiulio Faggiano, Roberta Modica, Valentina Morelli, Maura Arosio, Uberta Verga, Micaela Pellegrino, Luigi Petramala, Antonio Concistrè, Paola Razzore, Tonino Ercolino, Elena Rapizzi, Mario Maggi, Antonio Stigliano, Jacopo Burrello, Massimo Terzolo, Giuseppe Opocher, Massimo Mannelli, Giuseppe Reimondo

• No studies have carried out an extensive analysis of the possible association between non-syndromic pheochromocytomas and paragangliomas (PPGLs) and other malignancies. To assess >the risk of additional malignancy in PPGL, we retrospectively evaluated 741 patients with PPGLs followed-up in twelve referral centers in Italy. Incidence of second malignant tumors was compared between this cohort and Italian patients with two subsequent malignancies. Among our patients, 95 (12.8%) developed a second malignant tumor, which were mainly prostate,No studies have carried out an extensive analysis of the possible association between non-syndromic pheochromocytomas and paragangliomas (PPGLs) and other malignancies. To assess >the risk of additional malignancy in PPGL, we retrospectively evaluated 741 patients with PPGLs followed-up in twelve referral centers in Italy. Incidence of second malignant tumors was compared between this cohort and Italian patients with two subsequent malignancies. Among our patients, 95 (12.8%) developed a second malignant tumor, which were mainly prostate, colorectal and lung/bronchial cancers in males, breast cancer, differentiated thyroid cancer and melanoma in females. The standardized incidence ratio was 9.59 (95% CI 5.46–15.71) in males and 13.21 (95% CI 7.52–21.63) in females. At multivariable analysis, the risk of developing a second malignant tumor increased with age at diagnosis (HR 2.50, 95% CI 1.15–5.44, p = 0.021 for 50–59 vs. <50-year category; HR 3.46, 95% CI 1.67–7.15, p < 0.001 for >60- vs. <50-year). In patients with available genetic evaluation, a positive genetic test was inversely associated with the risk of developing a second tumor (HR 0.25, 95% CI 0.10–0.63, p = 0.003). In conclusion, PPGLs patients have higher incidence of additional malignant tumors compared to the general population who had a first malignancy, which could have an impact on the surveillance strategy.…