Locally advanced pheochromocytoma/paraganglioma exhibit high metastatic recurrence and disease specific mortality rates: long-term follow-up of 283 patients
Main finding · Overall recurrenceResult — go to the resultHR for overall recurrence 2.4 95% CI 1.4–5.0Authors' conclusion
Patients with LAP vs. nLAP have higher metastatic recurrences and worse DSS. Tumor emboli and extra-adrenal extension indicated lower DSS, warranting a close follow-up.
Study context
- Population
- Patients with pheochromocytoma/paraganglioma at a referral center, 1981–2024; followed 11.3 ± 8.8 (mean ± SD) years
- Participants
- 283 patients
- Exposure
- Locally advanced vs non-locally advanced PPGL
- LAPn = 79
- nLAPn = 204reference
- Outcome
- Overall, metastatic and nonmetastatic recurrence, overall survival (OAS) and disease-specific survival (DSS)
Methodological detailsprospectively supplemented 2020-2024, referral center
- Prospectively supplemented 2020-2024
- Referral center
Main finding
Overall recurrence · follow-up 11.3 ± 8.8 (mean ± SD) years
HR for overall recurrence
2.4
95% CI 1.4–5.0
LAPvs nLAP
HR for overall recurrence 2.4 (95% CI 1.4–5.0)
As reported in the abstract
Observational data: associations, not effects.
Further findings
Predictors of outcomes
- Extra-adrenal location predicted metastatic and nonmetastatic recurrence, not OAS or disease-specific survival (DSS)
- Tumor size predicted metastatic recurrence and DSS
- Cluster 1 and 2 pathogenic variants predicted overall and nonmetastatic recurrence, not OAS or DSS
LAP subtypes (tumor emboli; extra-adrenal extension) as predictors
- Overall recurrence, HR (tumor emboli; extra-adrenal extension)
- 4.5, 1.3-14.2 and 5.0, 1.4-13.7
- Metastatic recurrence, HR (tumor emboli; extra-adrenal extension)
- 24.6, 6.4-91.8 and 6.5, 1.6-23.4
- OAS, HR (tumor emboli only)
- 21.2, 2.8-108
- DSS, HR (tumor emboli; extra-adrenal extension)
- 22.6, 3.5-183 and 13.1, 1.7-120
- LAP with vessel invasion: nonmetastatic recurrence, HR
- 3.6, 1.2-10.0
Original publication
Locally advanced pheochromocytoma/paraganglioma exhibit high metastatic recurrence and disease specific mortality rates: long-term follow-up of 283 patients. Eur J Endocrinol. Published 20 May 2025. doi:10.
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