Indian Journal of Pathology and Microbiology
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Year : 2023  |  Volume : 66  |  Issue : 3  |  Page : 678-680
Catechism (Quiz 20)

Department of Pathology, Tata Memorial Hospital, Homi Bhabha National Institute University, Mumbai, Maharashtra, India

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Date of Web Publication28-Jul-2023

How to cite this article:
Rekhi B. Catechism (Quiz 20). Indian J Pathol Microbiol 2023;66:678-80

How to cite this URL:
Rekhi B. Catechism (Quiz 20). Indian J Pathol Microbiol [serial online] 2023 [cited 2023 Sep 27];66:678-80. Available from:

A 50-year-old postmenopausal female presented with abdominal pain of 1-year duration. Thereafter, she developed abdominal distension, bilateral pedal edema, and dyspnea on exertion.

She was treated elsewhere for abdominal Koch's to which she did not seem to respond and was referred to our cancer center.

Her abdominal examination revealed gross ascites with everted umbilicus. There was no lymphadenopathy.

Her recent ultrasonogram revealed a 10 cm × 5 cm-sized cystic mass with multiple internal thick septations in the pelvis, along with gross ascites with internal echoes, likely neoplastic etiology.

Computed tomography revealed a multiloculated cystic left-sided adnexal lesion measuring nearly 7.9 cm × 5.8 cm, which was not seen separately from the left ovary. There was omental thickening and nodularity in the supracolic mesentery with gross ascites.

She underwent ascitic fluid examination. Smears and cell blocks were prepared. Two images of Papanicolaou- and Giemsa-stained smears [Figure 1]a and [Figure 1b, along with a hematoxylin and eosin stained corresponding cell block preparation [Figure 2].
Figure 1:

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Figure 2:

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   Questions Top

Q1: What is the diagnosis?

Q2: What are the characteristic features/appearances associated with this lesion on smears?

Q3: Which immunostains are useful to confirm the diagnosis?

Q4: What are the diagnostic implications with respect to treatment with this lesion?

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   Answers of Catechism (Quiz 19) Top

Microscopic description: Low-power view of H&E-stained slides shows eosinophilic tumor in sheets/nests with a rim of uninvolved kidney parenchyma. High-power view shows tumor cells with abundant eosinophilic vacuolated cytoplasm, prominent nucleoli arranged in cribriform or sieve-like, papillary, cystic, and tubular patterns.

The differential diagnosis includes clear cell papillary renal cell carcinoma, papillary renal cell carcinoma, acquired cystic disease-associated renal cell carcinoma, chromophobe renal cell carcinoma, and Fumarate hydratase (FH) deficient renal cell carcinoma (RCC).

On immunohistochemical staining, the tumor showed positive staining for AMACR, CD10, and FH and focal positive staining for CAIX and CK7 and negative staining for CD117. Focal positive staining for CK7 rules out papillary RCC (usually strong diffuse CK7 positivity), FH retention rules out FH deficient RCC. Negative staining for CD117 rules out eosinophilic tumors like oncocytoma and chromophobe renal cell carcinoma. These features along with history of ESRD correspond to a diagnosis of acquired cystic disease-associated renal cell carcinoma.

  1. Diagnosis: Acquired cystic disease-associated renal cell carcinoma
  2. Differential diagnosis: Papillary renal cell carcinoma, clear cell papillary renal cell carcinoma, FH deficient renal cell carcinoma

   Discussion Top

Acquired cystic disease-associated renal cell carcinoma (ACD-RCC) is a unique subtype of renal cell carcinoma only found in patients with end-stage renal disease.[1],[2] It is the most common subtype of RCC present in end-stage kidneys (36%) and is present in 46% of kidneys with acquired cystic kidney disease (ACKD).[1],[2] ACD-RCC is more prevalent in men, and length of dialysis is a prominent risk factor, as incidence of ACD-RCC increases significantly with a dialysis duration of 10 years or longer.[3]

The pathogenesis of ACD-RCC is unclear, though current theories include multifactorial explanations, such as immunosuppression, oncogenic viruses, dialysis, and end-stage renal disease.[2] It is hypothesized that mutagenic or oxidative stress-inducing compounds accumulate in the cystic fluid, and the exposure of intracystic cells to carcinogens may induce carcinogenesis, potentially explaining the higher incidence of ACD-RCC in ESRD patients with long-term dialysis. Papillary adenoma and associated cysts have also been considered as putative precursors of ACD-RCC.[3]

Grossly, the tumors are well circumscribed and appear to arise in a cyst and generally completely fill the cystic space. The cells lining the cysts are typically morphologically similar to those in the rest of the tumor, with granular eosinophilic cytoplasm, round to oval nuclei, and prominent nucleoli. A sieve-like appearance, intra-tumoral oxalate crystal deposits, and prominent nucleoli are also characteristic features.[1],[4] ACD-RCC displays a variety of architectural patterns, presenting with solid, acinar, cystic, and papillary growth in various combinations, making it difficult to accurately diagnose. However, the presence of the cribriform, sieve-like architecture, and oxalate deposits are diagnostic of ACD-RCC.
Figure 1: H&E-stained slides show eosinophilic tumor with cystic areas. The tumor cells with abundant eosinophilic vacuolated cytoplasm, prominent nucleoli arranged in cribriform or sieve-like, papillary, cystic and tubular patterns. The background uninvolved renal parenchyma shows features of chronic kidney disease

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Figure 2: The tumor shows focal positive stainig for CK7 (a), diffuse positive staining for AMAXR (b), CD10 (c), focal positive staining for CAIX (d), negative staining for CD117 (e) and diffuse strong retention of FH (f)

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A specific immunohistochemical profile is not necessary for diagnosis and there is large variability in findings. AMACR diffusely stains positive, and CD10, PAX8, RCC, CD57, and GST-α positivity has been found. Variable negativity has been seen for CK7, including focal positivity. Molecular studies have shown gains and losses on varying chromosomes, most consistently a gain of chromosome 3.[1],[3]

The current literature suggests ACD-RCC is more aggressive than other renal cell carcinoma subtypes seen in end-stage renal disease due to the prevalence in high-grade tumors (WHO/ISUP grade 3/4). [2,3] ACD-RCC has a lower risk for metastasis and more favorable prognosis compared to other RCCs not associated with ACKD, most likely due to early incidental diagnosis during follow-ups in patients with chronic renal disease.[1]

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   References Top

Srigley JR, Delahunt B, Eble JN, Egevad L, Epstein JI, Grignon D, et al. The international society of urological pathology (ISUP) vancouver classification of renal neoplasia. Am J Surg Pathol 2013;37:1469-89.  Back to cited text no. 1
Song Y, Zheng J, Guo S, Fan L. An intracapsular nephrectomy for the acquired cystic disease-associated renal cell carcinoma in renal transplant allograft: A clinical case report. Medicine (Baltimore) 2021;100:e25858.  Back to cited text no. 2
Kondo T, Sasa N, Yamada H, Takagi T, Iizuka J, Kobayashi H, et al. Acquired cystic disease-associated renal cell carcinoma is the most common subtype in long-term dialyzed patients: Central pathology results according to the 2016 WHO classification in a multi-institutional study. Pathol Int 2018;68:543-9.  Back to cited text no. 3
Sun Y, Argani P, Tickoo SK, Epstein JI. Acquired cystic disease-associated renal cell carcinoma (ACKD-RCC)-like cysts. Am J Surg Pathol 2018;42:1396-401.  Back to cited text no. 4

Correspondence Address:
Bharat Rekhi
Room No. 818, Department of Surgical Pathology, 8th Floor, Annex Building, Tata Memorial Hospital, Dr. E.B. Road, Parel, Mumbai - 400 012, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.382466

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