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.
A multicenter epidemiological study on second malignancy in non-syndromic pheochromocytoma/paraganglioma patients in Italy / Canu, L.; Puglisi, S.; Berchialla, P.; De Filpo, G.; Brignardello, F.; Schiavi, F.; Ferrara, A. M.; Zovato, S.; Luconi, M.; Pia, A.; Appetecchia, M.; Arvat, E.; Letizia, C.; Maccario, M.; Parasiliti-Caprino, M.; Altieri, B.; Faggiano, A.; Modica, R.; Morelli, V.; Arosio, M.; Verga, U.; Pellegrino, M.; Petramala, L.; Concistre, A.; Razzore, P.; Ercolino, T.; Rapizzi, E.; Maggi, M.; Stigliano, A.; Burrello, J.; Terzolo, M.; Opocher, G.; Mannelli, M.; Reimondo, G.. - In: CANCERS. - ISSN 2072-6694. - 13:22(2021), pp. 1-15. [10.3390/cancers13225831]
A multicenter epidemiological study on second malignancy in non-syndromic pheochromocytoma/paraganglioma patients in Italy
Letizia C.;Faggiano A.;Petramala L.;Concistre A.;Stigliano A.;
2021
Abstract
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.File | Dimensione | Formato | |
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