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Primary tumour resection for synchronously metastatic phaeochromocytoma and paraganglioma: A population-based study
Kan Wu1, Chuan Zhou1, Zhihong Liu1
1Department of Urology, Institute of Urology, West China Hospital, Sichuan University, Sichuan, China.
Context:
Until recently, there are few effective treatment options for patients with synchronous metastatic phaeochromocytoma (PHEO) and paraganglioma (PGL). Surgical resection may improve the survival outcomes of these patients.
Objective:
To assess the role of surgical resection of the primary tumour in patients with synchronous metastatic PHEO and PGL.
Design:
Retrospective analysis of patients with synchronous metastatic PHEO/PGL using the Surveillance, Epidemiology, and End Results database (1988-2016).
Patients:
Patients with synchronous metastatic PHEO/PGL who underwent primary tumour resection.
Measurements:
Overall survival and Cox regression analyses.
Results:
A total of 99 patients with metastatic PHEO and 127 metastatic PGL patients were identified from the SEER database. Compared to metastatic PHEO, metastatic PGL patients had a better overall survival (5-year survival rate: 33.3% vs. 49.0%, p = .001). In metastatic PHEO patients, 53 (53.5%) patients underwent surgery for primary site. Surgically treated patients had an improved survival compared to non-surgery patients (5-year survival rate: 50.9% vs. 29.6%, p = .017). Among metastatic PGL patients, primary tumour resection was performed in 74 (58.3%) patients and had no significant effect on the survival of metastatic PGL. In sub-analyses, surgery only conferred a survival benefit in patients with primary tumours originated from aortic/carotid bodies, rather than other sites or abdominal tumours.
Conclusion:
Our findings suggest that primary tumour resection is associated with improved survival in patients with synchronous metastatic PHEO and those with PGL diseases located in aortic/carotid bodies. In addition, PHEO and PGL should be treated as two distinct clinical entities.
