European journal of endocrinology · 2025
Locally advanced pheochromocytoma/paraganglioma exhibit high metastatic recurrence and disease specific mortality rates: long-term follow-up of 283 patients.
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Raber Wolfgang, Scheuba Andreas, Marculescu Rodrig, Esterbauer Harald, Rohrbeck Johannes
Аннотация
IMPORTANCE: Data on locally advanced (LAP) pheochromocytoma/paraganglioma (PPGL), based on capsular, vascular or periadrenal fat invasion, tumor emboli and extra-adrenal extension, are scarce.
OBJECTIVE: To compare outcomes of patients with LAP and without (nLAP).
DESIGN: Retrospective cohort study, 1981-2024, prospectively supplemented 2020-2024.
SETTING: Referral center.
OUTCOMES: Overall, metastatic and nonmetastatic recurrence, overall (OAS) and disease-specific survival (DSS).
RESULTS: Of 283 patients followed for 11.3 ± 8.8 (mean ± SD) years, 79 (27.9%) had LAP. Compared to patients with nLAP (n = 204), patients with LAP had more overall (n = 17 vs. 31, hazard ratio 2.4, 95% CI 1.4-5.0) and metastatic (11 vs. 9, HR 6.8, 2.2-20.6) and similar (6 vs. 22, HR 1.2, 0.5-3.0) nonmetastatic recurrences. OAS was comparable (12 vs. 42 nonsurvivors, HR 1.2, 0.6-2.3), but mortality from metastatic disease was higher with LAP (2 vs. 4 deaths, HR 12.2, 1.8-82.8). Extra-adrenal tumor location was predictive of metastatic and nonmetastatic recurrence but not of OAS or DSS, tumor size of metastatic recurrence and of DSS, cluster 1 and 2 pathogenic variants of overall and nonmetastatic recurrence but not of OAS or DSS. LAP with tumor emboli and extra-adrenal extension predicted overall (HR 4.5, 1.3-14.2 and 5.0, 1.4-13.7) and metastatic recurrence (HR 24.6, 6.4-91.8 and 6.5, 1.6-23.4), OAS (HR 21.2, 2.8-108, tumor emboli only) and DSS (HR 22.6, 3.5-183 and 13.1, 1.7-120), LAP with vessel invasion nonmetastatic recurrence (HR 3.6, 1.2-10.0).
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.
OBJECTIVE: To compare outcomes of patients with LAP and without (nLAP).
DESIGN: Retrospective cohort study, 1981-2024, prospectively supplemented 2020-2024.
SETTING: Referral center.
OUTCOMES: Overall, metastatic and nonmetastatic recurrence, overall (OAS) and disease-specific survival (DSS).
RESULTS: Of 283 patients followed for 11.3 ± 8.8 (mean ± SD) years, 79 (27.9%) had LAP. Compared to patients with nLAP (n = 204), patients with LAP had more overall (n = 17 vs. 31, hazard ratio 2.4, 95% CI 1.4-5.0) and metastatic (11 vs. 9, HR 6.8, 2.2-20.6) and similar (6 vs. 22, HR 1.2, 0.5-3.0) nonmetastatic recurrences. OAS was comparable (12 vs. 42 nonsurvivors, HR 1.2, 0.6-2.3), but mortality from metastatic disease was higher with LAP (2 vs. 4 deaths, HR 12.2, 1.8-82.8). Extra-adrenal tumor location was predictive of metastatic and nonmetastatic recurrence but not of OAS or DSS, tumor size of metastatic recurrence and of DSS, cluster 1 and 2 pathogenic variants of overall and nonmetastatic recurrence but not of OAS or DSS. LAP with tumor emboli and extra-adrenal extension predicted overall (HR 4.5, 1.3-14.2 and 5.0, 1.4-13.7) and metastatic recurrence (HR 24.6, 6.4-91.8 and 6.5, 1.6-23.4), OAS (HR 21.2, 2.8-108, tumor emboli only) and DSS (HR 22.6, 3.5-183 and 13.1, 1.7-120), LAP with vessel invasion nonmetastatic recurrence (HR 3.6, 1.2-10.0).
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.