Abstract
INTRODUCTION: Acquired cystic kidney disease (ACKD) affects almost half of patients with chronic hemodialysis. ACKD is recognized as a predisposing condition for renal carcinoma, particularly in patients undergoing long-term dialysis. Acquired cystic disease-associated renal cell carcinoma (ACD-RCC) is a subtype of renal cell carcinoma (RCC) occurring solely in patients with ACKD. ACD-RCC was first introduced as a distinct entity in the 2016 World Health Organization (WHO) classification of renal tumors and retained in the 2022 update. However, it remains underdiagnosed due to histologic overlap with other subtypes. We aimed to study the clinicopathological features of the entity. CASE PRESENTATION: We reported the case of a 44-year-old patient who presented a hemorrhagic shock of suspected renal origin. A scan showed no renal tumor. The patient experienced a second episode of hemorrhagic shock. A hemostatic nephrectomy was necessary. Microscopic examination confirmed the diagnosis of ACD-RCC. The patient died three days post-operatively. CLINICAL DISCUSSION: ACD-RCC is a common but under recognized entity with specific clinical, histopathological, and evolutionary features. CONCLUSION: Enhanced recognition of ACD-RCC and its features by pathologists is mandatory to ensure accurate histopathological diagnosis and appropriate clinical management.