TY  - JOUR
A1  - Canu, Letizia
A1  - Puglisi, Soraya
A1  - Berchialla, Paola
A1  - De Filpo, Giuseppina
A1  - Brignardello, Francesca
A1  - Schiavi, Francesca
A1  - Ferrara, Alfonso Massimiliano
A1  - Zovato, Stefania
A1  - Luconi, Michaela
A1  - Pia, Anna
A1  - Appetecchia, Marialuisa
A1  - Arvat, Emanuela
A1  - Letizia, Claudio
A1  - Maccario, Mauro
A1  - Parasiliti-Caprino, Mirko
A1  - Altieri, Barbara
A1  - Faggiano, Antongiulio
A1  - Modica, Roberta
A1  - Morelli, Valentina
A1  - Arosio, Maura
A1  - Verga, Uberta
A1  - Pellegrino, Micaela
A1  - Petramala, Luigi
A1  - Concistrè, Antonio
A1  - Razzore, Paola
A1  - Ercolino, Tonino
A1  - Rapizzi, Elena
A1  - Maggi, Mario
A1  - Stigliano, Antonio
A1  - Burrello, Jacopo
A1  - Terzolo, Massimo
A1  - Opocher, Giuseppe
A1  - Mannelli, Massimo
A1  - Reimondo, Giuseppe
T1  - A multicenter epidemiological study on second malignancy in non-syndromic pheochromocytoma/paraganglioma patients in Italy
JF  - Cancers
N2  - 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.
KW  - pheochromocytoma
KW  - paraganglioma
KW  - epidemiology
KW  - genetic analysis
KW  - mortality
KW  - surveillance
Y1  - 2021
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-250148
SN  - 2072-6694
VL  - 13
IS  - 22
ER  -