Nephrectomy-facilitated resection of a retroperitoneal paraganglioma encasing the renal artery and adherent to the IVC: a case report

Introduction: Functional pheochromocytoma and paraganglioma (PPGL) with major vessel involvement require complex surgical management. Presentation of case: A 64-year-old man with hypertension, diabetes mellitus, and chronic kidney disease (CKD) stage 3 presented with a functional retroperitoneal paraganglioma encasing the right renal artery (split function, 17%) and adherent to the suprarenal inferior vena cava (IVC). Plasma metanephrine was 3959.5 pg/mL; normetanephrine was 1784.7 pg/mL. A three-step approach (hilar nephrectomy, IVC rotation along the caval adventitial plane, and tumor resection) achieved complete resection without prosthetic reconstruction. Operative time was 182 minutes; estimated blood loss was 100 mL. Serum creatinine improved despite right nephrectomy. At 1 month, blood pressure was normal off antihypertensives, catecholamines were in the reference range, computed tomography showed no recurrence, and renal function improved (CKD G2). Biochemical remission was confirmed at discharge (metanephrine, 45.6 pg/mL; normetanephrine, 80.5 pg/mL) and maintained at 1 month; “remission” is preferred to “cure” because PPGL recurrence can occur years later. Discussion: Fourteen reports of PPGL with major vascular involvement have been published to date, all of which involved prosthetic or primary vascular reconstruction. Three earlier reports describe nephrectomy for renal-hilum PPGL without great-vessel reconstruction, but none involved an IVC component. Three preoperative criteria are proposed to guide the selection of nephrectomy-facilitated resection when the encased kidney has severely diminished function. Conclusion: Nephrectomy-facilitated resection may be a less morbid alternative to prosthetic reconstruction (with risks of graft infection, thrombosis, and lifelong anticoagulation) in selected patients with PPGL and renal artery encasement. Selection criteria require external validation.