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

Puglisi S.
Co-first
;
Berchialla P.;Brignardello F.;Pia A.;Arvat E.;Maccario M.;Parasiliti-Caprino M.;Modica R.;Burrello J.;Terzolo M.;Reimondo G.
2021-01-01

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.
2021
13
22
5831
5846
Epidemiology; Genetic analysis; Mortality surveillance; Paraganglioma; Pheochromocytoma
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.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2318/1821176
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