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Predictors of recurrence of pheochromocytoma and paraganglioma: a multicenter study in Piedmont, Italy

Abstract

The available data on the natural history of pheochromocytomas and paragangliomas after radical surgery are heterogeneous and discordant. The aim of our retrospective multicenter study was to find predictors of recurrence in patients with pheochromocytomas and sympathetic paragangliomas submitted to radical surgery in Piedmont (a region in northwest Italy). We collected data from 242 patients diagnosed between 1990 and 2016. Forty-two patients (17.4%) had disease recurrence. Multivariate analysis showed that genetic mutation (HR = 3.62; 95% CI 1.44−9.13; p = 0.006), younger age (HR = 0.97; 95% CI 0.95−0.99; p = 0.031) and larger tumor size (HR = 1.01; 95% CI 1.00−1.02; p = 0.015) were independently associated with a higher recurrence risk of pheochromocytoma and paraganglioma; in pheochromocytomas, genetic mutation (HR = 3.4; 95% CI 1.00−11.48; p = 0.049), younger age (HR = 0.97; 95% CI 0.94−0.99; p = 0.02), higher tumor size (HR = 1.01; 95% CI 1.00−1.03; p = 0.043) and PASS value (HR = 1.16; 95% CI 1.03−1.3; p = 0.011) were associated with recurrence. Moreover, tumor size was the only predictor of metastatic pheochromocytoma and paraganglioma (HR = 4.6; 95% CI 1.4−15.0; p = 0.012); tumor size (HR = 3.93; 95% CI 1.2−16.4; p = 0.026) and PASS value (HR = 1.27; 95% CI 1.06−1.53; p = 0.007) were predictors of metastatic pheochromocytoma. In conclusion, our findings suggest that the recurrence of pheochromocytoma and sympathetic paraganglioma develops more frequently in younger subjects, patients with a family history of chromaffin tissue neoplasms, mutations in susceptibility genes, larger tumors and higher values of PASS. We recommend genetic testing in all patients with PPGL and strict follow-up at least on an annual basis.

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Acknowledgements

For their contributions to the present study, we would like to thank Chiara Bima, Alessia Pozzato, Elena Capello, Erika Rainero and Elena Nasari.

Funding

This study was supported by a grant from the University of Turin. The work of P.M., M.P., B.P., F.V., E.G., E.A. and M.M. has been supported by the “Dipartimenti di Eccellenza 2018–2022” program (project no D15D18000410001). The funding sources had no role in the study design, data collection and analysis, manuscript preparation, or decision to submit the paper for publication.

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Correspondence to Mirko Parasiliti-Caprino.

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Parasiliti-Caprino, M., Lucatello, B., Lopez, C. et al. Predictors of recurrence of pheochromocytoma and paraganglioma: a multicenter study in Piedmont, Italy. Hypertens Res 43, 500–510 (2020). https://doi.org/10.1038/s41440-019-0339-y

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Keywords

  • Endocrine hypertension
  • Chromaffin system
  • Pheochromocytoma
  • Paraganglioma
  • Genetic testing

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