Primary tumour resection for synchronously metastatic phaeochromocytoma and paraganglioma: A population‐based study.

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. [ABSTRACT FROM AUTHOR]