Clinical Profile, Outcomes, and Predictors of Malignant Pheochromocytoma and Paraganglioma: Insights From a Single-Center Cohort

Abstract

Background

Malignant pheochromocytoma and paraganglioma (mPPGL) are not so common and the factors predicting the metastatic behavior are not well understood.

Methods

This retrospective cohort study included 218 patients with PPGL managed at our center over a period of 11 years (2013–2024). The clinical profile and treatment outcomes of patients with mPPGL were studied and were compared with non-metastatic PPGL(non-mPPGL).

Results

Thirty-six patients had mPPGL and the median age at diagnosis of metastases was 39.5 years(range 10–62). Twenty-one(58.3%) patients had synchronous metastases and 15(41.7%) developed metachronous metastases after a median duration of 76 months(range 13–270 months) from the diagnosis of the primary tumor. Metastases were detected in 100% of patients who had 18FDG(4/4) and 68 Ga DOTATATE(4/4) PET-CT, 97.2% (35/36) with CT/MRI and 79.3%(23/29) with 131I MIBG scan. Surgery was the primary treatment in 78%, and 131I MIBG therapy was administered to 19(52.8%) patients. Eleven patients succumbed due to metastatic disease and among them nine died within a year of diagnosis of metastases, the median survival at last follow-up was 31.5 months (range 3–96). On comparing mPPGL(n = 36) and non-mPPGL(n = 182), we found that patients with mPPGL had larger tumors (8.8 ± 5.2 vs. 6.3 ± 3.3, p = 0.001), had less frequent adrenergic symptoms and more often had extra-adrenal tumors.

Conclusion

Malignant PPGLs had a variable clinical course and were amenable to multimodal therapeutic strategies with a favorable outcome in about 67% of the patients. Although mPPGL had larger tumor diameter compared to non-mPPGL, no particular size cut-off could accurately predict metastases. Adjuvant 131I MIBG therapy is a useful treatment option in resource limited settings.