Unexpected Paraganglioma Identified During Bilateral Nephrectomy in Autosomal Dominant Polycystic Kidney Disease: A Case Report

Autosomal dominant polycystic kidney disease (ADPKD) is commonly associated with hypertension, which is typically attributed to renal dysfunction. However, secondary causes of hypertension, particularly pheochromocytomas and paragangliomas (PPGLs), should be considered in cases of severe or difficult-to-control hypertension. We report the case of a 77-year-old woman with ADPKD and end-stage renal disease who underwent bilateral nephrectomy before kidney transplantation. Despite requiring four antihypertensive medications, her hypertension was attributed to renal disease. During left nephrectomy, manipulation of a perirenal mass resulted in a severe hypertensive crisis followed by refractory hypotension. Intraoperative frozen section analysis revealed an unsuspected 6.3-cm paraganglioma, while final pathology further demonstrated a 13-cm perirenal metastasis of the previously known myxoid liposarcoma, and also identified a second 0.4-cm peri-aortic paraganglioma. The patient experienced significant perioperative hemodynamic instability but recovered without organ failure. Postoperative complications included arteriovenous fistula occlusion, which was successfully managed. The discovery of metastatic disease led to reassessment of transplant candidacy. This case highlights the importance of considering PPGLs in ADPKD patients with severe hypertension requiring multiple antihypertensive medications. Preoperative biochemical screening and appropriate perioperative management are crucial for patient safety. The complexity of imaging interpretation in ADPKD patients requires careful multidisciplinary evaluation to exclude neoplastic processes.