Cancers (Nov 2021)

A Multicenter Epidemiological Study on Second Malignancy in Non-Syndromic Pheochromocytoma/Paraganglioma Patients in Italy

  • 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

DOI
https://doi.org/10.3390/cancers13225831
Journal volume & issue
Vol. 13, no. 22
p. 5831

Abstract

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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. p 60- vs. 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.

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