CT features of acquired cystic kidney disease-associated renal cell carcinoma
Number:27ESURABS0007
Type:Scientific Electronic Presentation
Authors: Robert Berkenblit, Zina Ricci, Devaraju Kanmaniraja, Judy Sarungbam
Keywords:CT, acquired cystic kidney disease, renal cell carcinoma
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Objectives

Acquired cystic kidney disease (ACKD) is defined as multiple bilateral renal cysts (three or more per kidney) in a patient with end-stage renal disease (ESRD) who does not have hereditary renal cystic disease. ACKD also develops in patients with chronic renal failure prior to dialysis as well as in chronically rejecting renal transplants.  It is three times more common in males than females and rises in incidence with the number of years after dialysis.  90% of patients with ESRD on dialysis for more than five years develop it and nearly 100% of patients develop it after 10 years of dialysis.  Patients with ACKD have a more than 100-fold increased risk of developing RCC than the general population with a reported overall incidence of renal malignancies ranging from 2-7%.

Acquired cystic kidney disease-associated RCC (ACKD-RCC) is a recently recognized subtype of...

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Material and methods

Our institutional review board approved this retrospective study. The need for informed consent was waived. A search of our institution’s pathology database from 2011 to 2020 was performed to identify patients with histologic proof of ACKD-RCC.  The abdominal CT scans of all patients with ACKD-RCC were evaluated by a combination of two out of the three attending radiologists involved in this project, all of whom are fellowship-trained in abdominal imaging and have 9-23 years of experience in abdominal imaging. Two patients underwent non-contrast CT and 22 patients underwent CT scan prior to and after administration of intravenous contrast. The following CT features of the lesions were assessed: maximum lesion diameter in the axial plane, lesion nature (solid, cystic or mixed), lesion border (round, lobulated or crescentic), presence and type of calcifications, lesion...

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Results

Our search revealed 30 lesions in 25 patients with histologic proof of ACKD-RCC.  One of these patients did not have a CT exam and therefore was excluded from the study; hence 29 lesions in 24 patients were evaluated.  The cases ranged in age from 32-68 years with an average of 55 years.  There were 14 males and 10 females.  The 24 patients included 16 Black, 7 Hispanic and 1 White patient.  Eight patients also had a history of renal transplant. One patient had bilateral tumors. Three patients had multiple tumors in one kidney (all three had two tumors each in their right kidney). The lesions ranged from 1.2 to 5.0 cm.  There were 17 solid lesions and 12 cystic or partly cystic lesions.  Lesion borders included: 21 round, 7 lobular and 1 crescentic.  Five lesions had gross calcifications on non-enhanced CT while 24 did not.  Four lesions...

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Figure 1: 42-year-old male with a 2 cm right lower pole partially exophytic round lesion (white arrow) on axial non-enhanced CT with heterogeneous diffuse calcifications causing a hyperdense appearance. Contrast-enhanced CT (not shown) revealed internal enhancement. This was proven to be an ACKD-RCC at surgery.
Figure 2: 67-year-old male with a 5 cm mildly hyperdense round left upper pole renal lesion (white arrow) on non-enhanced CT (a) that demonstrated heterogeneous internal enhancement on contrast-enhanced CT (b). Surgical clips from prior left adrenalectomy are noted. This was proven to be an ACKD-RCC at surgery.

Conclusions

In 2006, Tickoo et al reported a pathology series of 66 ESRD patients who underwent nephrectomy for detected tumors, 52 of whom had ACKD.  Two major groups of RCC were detected: (1) RCCs similar to those seen in the sporadic setting including papillary (18%), clear-cell (15%) and chromophobe (8%) RCCs, and (2) two RCC subtypes unique to ESRD, “acquired cystic disease-associated RCC” (36%) and “clear cell-papillary RCC of the end-stage kidneys” (23%). Acquired cystic disease-associated RCC, now also coined ACKD-RCC, was the single most common neoplasm detected in ESRD patients and was unique to patients with ACDK, not occurring in patients with non-cystic ESRD. Later, in 2020, Shah et al reported a pathology series of 59 patients with ESRD who underwent nephrectomy and ACKD-RCC was once again found to be the most common tumor detected with 54% being...

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