CASE OF THE WEEK
Editor: Mahmut Akgul (akgulm@amc.edu)
2022-21/May 30
Contributors: Walaa Hammad, Richard Pacheco, Rupinder Brar, Mahmut Akgul
A woman in her 60s with end-stage renal disease (ESRD) receiving hemodialysis (HD) was evaluated for right lower quadrant pain. Computed tomography (CT) demonstrated a 3.9 cm complex renal cyst, enlarged from 2.1 cm on previous imaging. There was concern for malignancy and she subsequently underwent left radical nephrectomy. The specimen was bisected to reveal multiple masses in the upper and lower poles. These masses demonstrated variegated, orange-yellow to tan-red cut surfaces with well-delineated borders. The remaining parenchyma demonstrated multiple cysts measuring 4.5 cm in greatest dimension. (Microscopic images are from the largest mass, showing similar features with the other masses).
Quiz
a) Clear cell renal cell carcinoma (CCRCC)
b) Papillary renal cell carcinoma (PRCC)
c) Acquired cystic kidney disease associated renal cell carcinoma (ACKD-RCC)
d) Chromophobe renal cell carcinoma (ChRCC)
1. c
1. Acquired cystic kidney disease associated renal cell carcinoma.
Hematoxylin and eosin stained slides of the masses showed renal parenchyma largely replaced by cystic change. Hemorrage with hemosiderin laden macrophages are common. Proliferation of tumor cells with microcystic, sheet-like, tubulopapillary, and cribriform growth patterns are noted. The individual tumor cells have granular eosinophilic and clear cytoplasm with large nuclei and conspicuous nucleoli; and showing syncytial type cohesion. Immunohistochemical assays show tumor cells staining positive for CD10 (not shown), AMACR, CA9 (focal, only in relation with cystic change) and negative for KRT7 (CK7). The morphologic and immunohistochemical profile supports a diagnosis of ACKD-RCC, although CCRCC and PRCC were considered.
Various renal tumors have been reported in patients with chronic kidney disease. These patients are more likely to develop RCC and the risk only increases with hemodialysis. ACD-RCC was first described in a 2006 study of 66 tumor-bearing kidneys from patient with ESRD. The authors noted that patients receiving hemodialysis developed ACD-RCC. Age of presentation is variable, ranging from 28 to 87 years, and is more common in men. Diagnosis can be challenging due to overlapping morphologic and immunohistochemical features with other RCCs. However, history of ESRD requiring hemodialysis, cribriform architecture, and occasional intratumor oxalate deposits can help distinguish this tumor from other entities. ACKD-RCC is predominantly an indolent tumor, although rare cases of metastasis and sarcomatoid differentiation are reported.
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Walaa Hammad, Richard Pacheco, Rupinder Brar, Mahmut Akgul
Department of Pathology and Laboratory Medicine, Albany Medical Center, Albany, NY, USA
kidney
Acquired cystic disease-associated renal cell carcinoma; hemodialysis; end stage renal disease