Renal Cell Carcinoma in End-Stage Renal Disease: A Review and Update

Abstract

Renal cell carcinoma (RCC) occurring in the setting of end-stage renal disease (ESRD) shows unique clinicopathological characteristics. The two most frequent types of ESRD-associated RCC are acquired cystic kidney disease-associated renal cell carcinoma (ACKD-RCC) and clear-cell papillary renal cell carcinoma (ccpRCC). Other types of RCC also occur in ESRD, albeit with different frequencies from the non-ESRD general population. The histological features of RCC do not vary in the setting of ESRD vs. non-ESRD, yet other findings, such as multifocality and multiple tumor types, are more frequent in ESRD. Studies have generated novel and important knowledge of the etiology, epidemiology, diagnosis, treatment, immunophenotype, and molecular characteristics of ESRD-associated RCC. Knowledge of these data is important for both pathologists and other physicians who may encounter ESRD patients with RCC. This review presents a comprehensive summary and update of the literature on RCC in ESRD, with a focus on the two most frequent types, ACKD-RCC and ccpRCC.

Keywords: acquired cystic kidney disease; acquired cystic kidney disease-associated renal cell carcinoma; clear-cell papillary renal cell carcinoma; end-stage renal disease; end-stage renal disease-associated renal cell carcinoma; immunohistochemistry; molecular pathology; pathology; renal cell carcinoma.

Figure 1

Summary of pathogenesis of RCC in ESRD.

Figure 2

Concurrent ACKD-RCC and ccpRCC in a single kidney. Two tumors occurred simultaneously in this nephrectomy, a ccpRCC (right side of picture, abutting renal capsule) and a smaller ACKD-RCC (central part of picture).

Figure 3

Gross and histological features of ACKD-RCC. (a) ACKD-RCC arising in two foci (arrows) of a kidney with multiple background cysts. (b) Papillary architecture in ACKD-RCC. (c) Calcium oxalate crystals in ACKD-RCC under polarized light. (d) Nests and cords of eosinophilic cells in ACKD-RCC with interspersed calcium oxalate. (e) Characteristic “sieve-like” spaces. (f) Area of an ACKD-RCC, including cells with clear cytoplasm. (Note: (a) gross image, (b–f) photomicrographs of hematoxylin and eosin stained sections, (b–e) 100× magnification, (f) 200× magnification).

Figure 4

Histological features of ccpRCC. (a) Tubular architecture in ccpRCC. (b) Areas with tubular and solid or nested architecture of clear cells. (c) Clear cells with linear arrangement of nuclei and inverse polarization, which have been described as resembling secretory endometrial glands. (d) Cystic septum in ccpRCC with short papillary projections. (Note: Hematoxylin and eosin stained sections, (a,b,d) 100× magnification, (c) 600× magnification).