Bilateral chromophobe renal cell carcinoma in an end-stage renal disease patient on haemodialysis

Yaser Saeedi; Mohammad Hassan Al Hamad; Kais Kotiesh; Rafe Alhayek

doi:10.4103/HMJ.HMJ_17_19

CASE REPORT

Year : 2019 | Volume : 12 | Issue : 4 | Page : 223-225

Bilateral chromophobe renal cell carcinoma in an end-stage renal disease patient on haemodialysis

Yaser Saeedi, Mohammad Hassan Al Hamad, Kais Kotiesh, Rafe Alhayek
Dubai Hospital, Dubai Health Authority, Dubai, UAE

Date of Submission	20-Feb-2019
Date of Acceptance	30-Jun-2019
Date of Web Publication	11-Nov-2019

Correspondence Address:
Mohammad Hassan Al Hamad
Dubai Hospital, Dubai
UAE

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/HMJ.HMJ_17_19

Abstract

There is a well-known association between end-stage renal disease (ESRD) and the development of kidney cancer in the native kidney of patients requiring renal replacement therapy. There is now emerging evidence that lesser degrees of renal insufficiency (chronic kidney disease [CKD]) are also associated with an increased likelihood of cancer in general and kidney cancer in particular. Bilateral renal involvement by primary malignant processes is uncommon, although known to occur with Wilms tumours and in von Hippel–Lindau disease. Our patient is a 65-year-old female CKD, on haemodialysis for 8 years with bilateral renal tumours, underwent laparoscopic bilateral radical nephrectomy with histopathology reported as synchronous chromophobe bilateral renal cell carcinoma (RCC), which is the first case reported in the literature. In a study for 10 years on patients with CKD on haemodialysis, who developed acquired renal cystic disease (ARCD), there was an increase in the prevalence and severity of the ARCD as the dialysis continues over the years. Twenty per cent of patients dialysed for 1–3 years have ARCD, compared with >90% of patients dialysed for 5–10 years. In another study held at the Massachusetts General Hospital. They found that the occurrence of bilateral RCC is rare and found in only 1.8% of 329 patients who were treated RCC in 30 years. In a study published in the European Urology 2011. They also found that there are many favourable clinical, pathological and outcome features in the RCC accompanied with ESRD patients on dialysis which arising in the native kidneys compared with the RCC diagnosed in general population patients.

Keywords: Bilateral acquired renal cystic disease, bilateral nephrectomy, bilateral renal cell carcinoma, chromophobe renal cell carcinoma, chronic kidney disease, cystic, haemodialysis, kidney diseases, renal dialysis, treatment outcome

How to cite this article:
Saeedi Y, Al Hamad MH, Kotiesh K, Alhayek R. Bilateral chromophobe renal cell carcinoma in an end-stage renal disease patient on haemodialysis. Hamdan Med J 2019;12:223-5

How to cite this URL:
Saeedi Y, Al Hamad MH, Kotiesh K, Alhayek R. Bilateral chromophobe renal cell carcinoma in an end-stage renal disease patient on haemodialysis. Hamdan Med J [serial online] 2019 [cited 2020 Oct 31];12:223-5. Available from: http://www.hamdanjournal.org/text.asp?2019/12/4/223/270671

Introduction

The renal cell carcinoma (RCC) is derived from the renal tubular epithelial cells and represents approximately 3.8% of all malignancies in adults.

The incidence of RCC has been growing steadily and ranging from 0.6 to 14.7 for every 100,000 inhabitants.

Patients with end-stage renal disease (ESRD) and acquired cystic kidney (acquired renal cystic disease [ARCD]) disease are at increased risk of developing RCC while undergoing dialysis treatment or after renal transplantation.^[1]

In a multicentre study collected 28,642 patients' data for half a century^[2] reported synchronous bilateral renal cell cancer in 86 patients (0.3%) and metachronous bilateral renal cell cancer in 112 patients (0.39%).

One case reported as a chromophobe renal cell carcinoma (CHRCC) in an ESRD on haemodialysis to date. We present a case of synchronous bilateral renal tumours in an ESRD patient with a rare histopathology finding of CHRCC. To the best of our knowledge, no similar cases were published in the literature.

Case Report

A 65-year-old female diagnosed with chronic kidney disease (CKD) in the year 2007 due to chronic hypertension and diabetes mellitus and was started haemodialysis on the same year.

She was on haemodialysis for 8 years. A routine ultrasound KUB done in May 2015 showed (hypovascular soft-tissue lesion seen in the lower pole of the right kidney, hypoechoic well-defined low vascular lesion seen occupied the left renal fossa).

Computed tomography (CT) chest, abdomen, pelvis with contrast revealed B/L renal masses in 2015, at this time, the patient refused any further surgical intervention and continue follow-up with nephrology team.

On January 2017, a CT chest, abdomen and pelvis with contrast repeated and revealed mild progressive course as regards the size of the previously seen bilateral lower polar heterogeneously enhancing well-defined mass lesions presenting central hypodensity. They measure 3.7 and 3.4 on the right and left side, respectively (compared to 3.4 cm and 2.9 cm in the previous study).

The patient and her family agreed to undergo for the surgery and subsequently she underwent laparoscopic bilateral radical nephrectomy on March 2017.

CT abdomen with contrast showed bilateral lower polar heterogeneously enhancing well-defined mass lesions presenting central hypodensity. They measure 3.7 and 3.4 on the right and left side, respectively.

Histopathology results found as bilateral CHRCC.

Left kidney: The histomorphological features and immunoprofile are in keeping with CHRCC. No sarcomatoid features.

Right kidney: The histomorphological features and immunoprofile are in keeping with CHRCC. No sarcomatoid features.

A unifocal 3.8 cm × 3.6 cm lower pole Grade 3 Fuhrman with tumour necrosis focally present.

The patient shifted to the surgical ward, where she recovered gradually and discharged in good condition except from her haemodialysis.

Moreover, she is still following with nephrology team and urology with no recurrence of her RCC.

Discussion

The patient was asymptomatic, and during a routine ultrasound study, we found bilateral renal masses.

Laparoscopic bilateral radical nephrectomy performed, and there was no lymph nodes involvement or distant metastasis.

Now, the patient is on regular follow-up for 27 months with no evidence of recurrence or metastasis.

Apart from her dialysis follow-up, her performance status (PS) was very good.

CHRCC counts 3%–5% of all renal malignant tumours; it comes in a familial type accompanied with Birt–Hogg–Dubé syndrome, folliculin gene mutation (17p11) and loss of multiple chromosomes (1, 2, 6, 10, 13, 17, 21, Y).

In the gross appearance, usually, it is well-circumscribed, homogeneous, tan or light brown cut surface.

Originates from intercalated cells of collecting duct.

In general, good prognosis, although sarcomatoid variant associated with poor prognosis.

Papillary RCC is known of its tendency towards multicentricity which is around 40% in many series and occurs more commonly in patients with end-stage renal failure and ARCD.^[3]

ARCD characterised by the development of multiple cysts in the kidneys of patients with ESRD, in the absence of congenital cystic disease [Figure 1].^[3],[4] A study by the United States Renal Data System found a link between kidney cancer and ESRD. Study results showed that an increased number of kidney-cancer patients later developed ESRD during their treatment; similarly, patients with either CKD or ESRD showed an increased number of patients who developed renal cancer after long-term haemodialysis.^[1]

Figure 1: CT abdomen with contrast showed bilateral lower polar heterogeneously enhancing well-defined mass lesions presenting central hypodensity. They measure 3.7 and 3.4 on the right and left side respectively

Click here to view

In a comparison, a retrospective study ^[5] included 1250 RCC patients [Table 1]:

Table 1: RCC in ESRD patients and in general population patients; comparative study

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The results were as follows:

In univariate analysis, histology subtype, symptoms at diagnosis, poor PS, advanced TNM stage; high Fuhrman grade, large tumour size and non-ESRD diagnosis context were adverse predictors for survival. However, only PS, TNM stage and Fuhrman grade remained independent Cancer-specific survival (CSS) predictors in multivariate analysis. The limitation of this study related to the retrospective design.

Scientists notice the correlation between RCC and acquired cystic kidney disease (ACKD) arising in ESRD patients on haemodialysis since >50 years.

The first correlation was noticed in the autopsy findings.

Dunnill et al. were first described this association between ARCD and RCC in ESRD patients with haemodialysis.^[6]

They performed an autopsy study of 40 patients, where they found 46% had ACKD and 15% had a renal tumour.

In a larger autopsy study series performed on 155 haemodialysis patients, Miller et al. subsequently reported that ACKD noted in 58%, renal adenoma in 16% and RCC in 2% of patients.^[7]

Later, many studies focused and correlated between the prevalence, risk factors and natural history of both ARCD and RCC in ESRD patients (both before and after renal transplantation).^{[8],[9],[10],[11]}

The disadvantage of most of these studies that they used the ultrasound study as a screening utility; which has underestimated the true prevalence of RCC due to the limitations of the US resolution.

Another autopsy study performed on 14 patients with ACKD found that 6 of them had RCC.

Conclusion

RCC post-haemodialysis in the ESRD is usually papillary cell carcinoma.^[1],[3]

In our case, the RCC was bilateral and chromophobe cell carcinoma.

Patients with ESRD are at increased risk of developing RCC in their native kidneys.

ACKD resulting in long-standing haemodialysis represents the predisposing factor for developing RCC.^[4]

To the best of our knowledge, this is the first case reported in the literature of bilateral CHRCC in an ESRD patient on haemodialysis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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