Canu Letizia, Puglisi Soraya, Berchialla Paola, De Filpo Giuseppina, Brignardello Francesca, Schiavi Francesca, Ferrara Alfonso Massimiliano, Zovato Stefania, Luconi Michaela, Pia Anna, Appetecchia Marialuisa, Arvat Emanuela, Letizia Claudio, Maccario Mauro, Parasiliti-Caprino Mirko, Altieri Barbara, Faggiano Antongiulio, Modica Roberta, Morelli Valentina, Arosio Maura, Verga Uberta, Pellegrino Micaela, Petramala Luigi, Concistrè Antonio, Razzore Paola, Ercolino Tonino, Rapizzi Elena, Maggi Mario, Stigliano Antonio, Burrello Jacopo, Terzolo Massimo, Opocher Giuseppe, Mannelli Massimo, Reimondo Giuseppe
Department of Experimental and Clinical Biomedical Sciences "Mario Serio", University of Florence, 50139 Florence, Italy.
Centro di Ricerca e Innovazione sulle Patologie Surrenaliche, AOU Careggi, 50134 Florence, Italy.
Cancers (Basel). 2021 Nov 20;13(22):5831. doi: 10.3390/cancers13225831.
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, = 0.021 for 50-59 vs. <50-year category; HR 3.46, 95% CI 1.67-7.15, < 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, = 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.
尚无研究对非综合征性嗜铬细胞瘤和副神经节瘤（PPGLs）与其他恶性肿瘤之间可能的关联进行广泛分析。为评估PPGL患者发生其他恶性肿瘤的风险，我们对意大利12个转诊中心随访的741例PPGL患者进行了回顾性评估。将该队列患者与患有两种后续恶性肿瘤的意大利患者的第二恶性肿瘤发病率进行了比较。在我们的患者中，95例（12.8%）发生了第二恶性肿瘤，男性主要为前列腺癌、结直肠癌和肺癌/支气管癌，女性为乳腺癌、分化型甲状腺癌和黑色素瘤。男性的标准化发病率比为9.59（95%CI 5.46 - 15.71），女性为13.21（95%CI 7.52 - 21.63）。在多变量分析中，诊断时年龄越大，发生第二恶性肿瘤的风险越高（50 - 59岁组与<50岁组相比，HR 2.50，95%CI 1.15 - 5.44，P = 0.021；>60岁组与<50岁组相比，HR 3.46，95%CI 1.67 - 7.15，P < 0.001）。在进行了基因评估的患者中，基因检测呈阳性与发生第二种肿瘤的风险呈负相关（HR 0.25，95%CI 0.10 - 0.63，P = 0.003）。总之，与首次发生恶性肿瘤的普通人群相比，PPGL患者发生其他恶性肿瘤的发病率更高，这可能会对监测策略产生影响。
