En Bloc Resection with Major Blood Vessel Reconstruction for Locally Invasive Retroperitoneal Paragangliomas: A 15-Year Experience with Literature Review

Abstract

Background: Retroperitoneal paragangliomas (PGs) are a rare subgroup of neuroendocrine tumors that are commonly located alongside the abdominal aorta. Complete tumor resection is thought to be restricted in patients with major blood vessel involvement. However, no study has specifically focused on aggressive surgical treatment in such patients. We evaluated the value of en bloc resection with major blood vessel reconstruction for locally invasive retroperitoneal PGs.

Methods: Twenty-nine patients with retroperitoneal PGs with major blood vessel involvement were included in this retrospective study. Survival was compared between patients who underwent en bloc resection with major blood vessel reconstruction and those who underwent medical treatment. Prognostic predictors were analyzed in patients who underwent en bloc resection with major blood vessel reconstruction.

Results: All 11 patients who underwent medical treatment obtained a pathological diagnosis by computed tomography-guided percutaneous needle biopsy. All 18 patients who underwent en bloc resection with major blood vessel reconstruction achieved complete tumor resection. Overall survival was higher in patients who underwent complete tumor resection than in those who underwent medical treatment (p < 0.05). No perioperative mortality occurred in patients who underwent complete tumor resection. Patients with no metastasis, no organ invasion, R0 resection, a Ki-67 index of ≤3%, and a tumor diameter of ≤11.7 cm showed better tumor-free survival (p < 0.05).

Conclusions: En bloc resection with major blood vessel reconstruction can be successfully performed and provide satisfactory outcomes for patients with locally invasive retroperitoneal PGs. This technique may become a standard surgical strategy for properly selected patients.