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Diagnosis: Renal Cell Carcinoma With Intratumoral Calcium Oxalate Crystal Deposition in a Patient With Acquired Cystic Disease of the Kidney

The main complication of acquired cystic kidney disease (ACKD) is frequent development of renal tumors, including renal cell carcinoma (RCC). Intratumoral deposition of calcium oxalate (CaOx) is a distinct feature of ACKD-associated RCCs.

Clear cell RCC is the most common histological type in ESRD patients regardless of hemodialysis duration. RCCs tend to show sarcomatoid changes during long‐term hemodialysis, especially when it exceeds 20 years. Collectively, these findings suggest that hemodialysis‐associated toxicities, such as those causing continuous long‐term oxidative stress, elicit numerous genetic changes that promote RCC development, including sarcomatoid transformation, in ESRD patients.

RCCs in ESRD patients were first described in 1977, the histological features of the six RCCs in that study appeared to resemble those of clear cell and papillary RCCs. ACKD‐associated RCC was categorized as a new entity by the WHO in 2016. ACKD‐associated RCCs usually occur within a cyst in a background of multiple cysts. They are sometimes multifocal and bilateral, and usually well circumscribed, except in cases with sarcomatoid components. The cut surface is typically tan to yellow or brown with dry features, and sometimes hemorrhagic and necrotic.

Most ACKD‐associated RCCs have multiple histological patterns (alveolar, tubular, papillary, cystic and solid) to some degree. They characteristically have a cribriform or sieve‐like architecture. The tumor cells usually have abundant eosinophilic cytoplasm and round to oval nuclei with prominent nucleoli. Mitotic figures are rarely seen. The presence of calcium oxalate crystals showing multicolored birefringence under polarized light is one of the most distinguishing histological features of ACKD‐associated RCC. Calcium oxalate crystals are often observed in the stroma as well as the tumor, and interestingly, are sometimes associated with multinucleated giant cells resulting from foreign body reactions. Their recognition is critical for diagnosing ACKD‐associated RCC. Immunohistochemically, ACKD‐associated RCC tumor cells express RCC marker, CD10, AMACR, glutathione S‐transferase‐alpha and BerEP4, but not cytokeratin 7, E‐cadherin, high‐molecular‐weight cytokeratin or MOC31. Cytogenetic studies show a gain of chromosomes 3, 7, 12, 16, 17, 20 and Y. The cysts are lined by atypical cells with eosinophilic cytoplasm, and have flat, papillary or cribriform proliferation patterns and cytogenetic abnormalities, including a gain of chromosomes 3, 7, 12, 16, 17, 20 and Y. Although the atypical cell proliferation might indicate pre‐malignancy, as suggested by some studies, there is no direct evidence of ACKD‐associated RCCs arising from cysts. (Most of the previous text was taken from: Tsuzuki T, et al. Renal tumors in end-stage renal disease: A comprehensive review. Int J Urol. 2018;25(9):780-786. [PubMed link]).

See the chapter: Renal Neoplasms of our Tutorial (only Spanish version).

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References

  • Tsuzuki T, Iwata H, Murase Y, Takahara T, Ohashi A. Renal tumors in end-stage renal disease: A comprehensive review. Int J Urol. 2018;25(9):780-786. [PubMed link]
  • Sun Y, Argani P, Tickoo SK, Epstein JI. Acquired Cystic Disease-associated Renal Cell Carcinoma (ACKD-RCC)-like Cysts. Am J Surg Pathol. 2018;42(10):1396-1401. [Pub-Med link]
  • Bhatnagar R, Alexiev BA. Renal-cell carcinomas in end-stage kidneys: a clinicopathological study with emphasis on clear-cell papillary renal-cell carcinoma and acquired cystic kidney disease-associated carcinoma. Int J Surg Pathol. 2012;20(1):19-28. [PubMed link]
  • Enoki Y, Katoh G, Okabe H, Yanagisawa A. Clinicopathological features and CD57 expression in renal cell carcinoma in acquired cystic disease of the kidneys: with special emphasis on a relation to the duration of haemodialysis, the degree of calcium oxalate deposition, histological type, and possible tumorigenesis. Histopathology. 2010;56(3):384-94. [PubMed link]
  • Sule N, Yakupoglu U, Shen SS, Krishnan B, Yang G, Lerner S, Sheikh-Hamad D, Truong LD. Calcium oxalate deposition in renal cell carcinoma associated with acquired cystic kidney disease: a comprehensive study. Am J Surg Pathol. 2005;29(4):443-51. [PubMed link].

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