Acquired Cystic Disease–Associated Renal Cell Carcinoma: Review of Pathogenesis, Morphology, Ancillary Tests, and Clinical Features

Abstract

Acquired cystic disease-associated renal cell carcinoma (ACD-RCC) is a recently described subtype of RCC found in individuals with ACD of the kidney. Because of underrecognition, information regarding this lesion is sparse but continues to accumulate with each new report. Herein, a thorough literature review amassing the current understanding of this unique neoplasm is presented. Discussion focuses on clinical features, pathogenesis, disease outcome, and relation to the duration of dialysis. The macroscopic and characteristic microscopic features are described with illustrations. Compared with previous opinion, compiled immunohistochemical data may now allow for recognition of a unique immunophenotypic pattern of ACD-RCC. Distinction of ACD-RCC from clear cell and papillary RCCs based on molecular genetic information is deliberated, including a summary of the most frequently detected cytogenetic abnormalities. The key morphologic and immunophenotypic patterns used to distinguish this entity from a comprehensive differential diagnosis are provided.

Rare histological subtypes of renal cell carcinoma in everyday diagnostic practice.

“…Acquired cystic disease (ACD)-associated RCC is a subtype newly included in the 2016 WHO classification. It was first described in 2005 and it was recognized in the 2013 ISUP Vancouver classification of renal neoplasia [9]. This tumour arises in patients with acquired cystic kidney disease due to end-stage renal disease, often in association with long-term haemodialysis or peritoneal dialysis [4,9].…”

Identification of a novel methylation gene sites signature to predict prognosis in kidney renal papillary cell carcinoma by comprehensive profiling

“…There are over ten recognized histological subtypes of RCC but malignancies of chromophobe, kidney renal papillary cell carcinoma (KIRP), and the kidney renal clear cell carcinoma (KIRC) are the three most common subtypes. Among these subtypes, KIRP has the second highest morbidity rate (10%-15% of RCC cases), while KIRC has the highest incidence (75%-80% in RCC [1][2][3][4][5]). About 30% of RCC patients present with distant metastasis at diagnosis and have a poor prognosis.…”

Editorial Comment to Renal tumors in end‐stage renal disease: A comprehensive review

“…Foshat et al . reviewed the pathogenesis, morphology and clinical characteristics of ACD‐associated RCC, and describe dissimilar clinical and pathological features compared with clear cell RCC and papillary RCC . Tsuzuki et al .…”