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


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

Overview publication

TitlePredictors of recurrence of pheochromocytoma and paraganglioma: a multicenter study in Piedmont, Italy
DateOctober 4th, 2020
Issue nameHypertension Research
Issue numberVolume 43, pages 500–510(2020)
AuthorsMirko Parasiliti-Caprino, Barbara Lucatello, Chiara Lopez, Jacopo Burrello, Francesca Maletta, Marinella Mistrangelo, Enrica Migliore, Francesco Tassone, Antonio La Grotta, Anna Pia, Giuseppe Reimondo, Roberta Giordano, Giuseppe Giraudo, Alessandro Piovesan, Giovannino Ciccone, Dèsirèe Deandreis, Paolo Limone, Fabio Orlandi, Giorgio Borretta, Marco Volante, Paolo Mulatero, Mauro Papotti, Gianluca Aimaretti, Massimo Terzolo, Mario Morino, Barbara Pasini, Franco Veglio, Ezio Ghigo & Emanuela Arvat & Mauro Maccario
Read Read publication