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International Journal of Case Reports and Images (IJCRI) Renal carcinoma complicating autosomal dominant polycystic kidney disease

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  • Hospital General Universitario de Ciudad Real. Servicio de Salud de Castilla-La Mancha

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International Journal of Case Reports and Images (IJCRI) is an international, peer reviewed, monthly, open access, online journal, publishing high-quality, articles in all areas of basic medical sciences and clinical specialties. Aim of IJCRI is to encourage the publication of new information by providing a platform for reporting of unique, unusual and rare cases which enhance understanding of disease process, its diagnosis, management and clinico-pathologic correlations. ABSTRACT Abstract is not required for Clinical Images
Content may be subject to copyright.
CLINICAL IMAGES OPEN ACCESS | PEER REVIEWED
www.edoriumjournals.com
International Journal of Case Reports and Images (IJCRI)
International Journal of Case Reports and Images (IJCRI) is
an international, peer reviewed, monthly, open access, online
journal, publishing high-quality, articles in all areas of basic
medical sciences and clinical specialties.
Aim of IJCRI is to encourage the publication of new information
by providing a platform for reporting of unique, unusual and
rare cases which enhance understanding of disease process,
its diagnosis, management and clinico-pathologic correlations.
IJCRI publishes Review Articles, Case Series, Case Reports,
Case in Images, Clinical Images and Letters to Editor.
Website: www.ijcasereportsandimages.com
Renal carcinoma complicating autosomal dominant polycystic
kidney disease
Francisco Rivera, Celia López Redondo
ABSTRACT
Abstract is not required for Clinical Images
(This page in not part of the published article.)
International Journal of Case Reports and Images, Vol. 6 No. 2, February 2015. ISSN – [0976-3198]
Int J Case Rep Images 2015;6(2):115–117.
www.ijcasereportsandimages.com
Rivera et al. 115
CLINICAL IMAGES OPEN ACCESS
Renal carcinoma complicating autosomal dominant
polycystic kidney disease
Francisco Rivera, Celia López Redondo
CASE REPORT
On February 2013, a 76-year-old male was sent
to our nephrology unit to evaluate renal disease. He
was asymptomatic and had been diagnosed with
hypertension, overweight, benign prostatic hypertrophy
and chronic obstructive pulmonary disease several years
ago. His one daughter was also diagnosed with a renal
cyst. His serum creatinine was 1.6 mg/dL, eGFR 42 mL/
min, albumin/creatinine ratio in spot urine sample 2.8
mg/g and normal urinary sediment. Ultrasonography
showed a slight enlargement of both kidneys and the
presence of multiple bilateral cysts, predominantly with
cortical distribution, classified as Bosniak I. No complex
cysts that required monitoring or solid lesions were found
(Figure 1). Therefore, he was diagnosed with Autosomal
Dominant Polycystic Kidney Disease (ADPKD) and
follow-up was drawn. He was successfully treated with
losartan 50 mg/day. On July 2014, a new ultrasound
control revealed the appearance of an echogenic nodule
on the upper pole of the right kidney with vascularization
in Doppler mode (Figure 2). The study was completed by
computed tomography scan that confirmed the presence
of a solid nodule of 23 mm on the right kidney with
early contrast enhancement after i.v. iodine contrast
administration. These findings strongly suggested the
existence of superimposed renal cell carcinoma (RCC)
(Figure 3). Considering his age and co-morbidities
conservative treatment was planned.
Francisco Rivera1, Celia López Redondo2
Affiliations: 1Department of Nephrology, Hospital General
Universitario de Ciudad Real, Spain; 2Department of
Radiology, Hospital Santa Bárbara, Puertollano, Ciudad
Real, Spain.
Corresponding Author: Francisco Rivera, Nephrology Unit,
Hospital General Universitario de Ciudad Real, Spain;
Email: friverahdez@senefro.org
Received: 08 November 2014
Accepted: 02 December 2014
Published: 01 February 2015
CLINICAL IMAGES OPEN ACCESS | PEER REVIEWED
Figure 1: Ultrasound image of right kidney showing multiple
cysts with cortical distribution, classified as Bosniak I.
Figure 2: Ultrasound image of the upper pole of right kidney.
(A) B-mode image with an echogenic nodule, with rounded
morphology (white arrow), (B) Doppler mode reveals the
presence of vascularization in this nodule (white arrow).
DISCUSSION
The association between RCC and ADPKD has
been described although this association has raised
some controversy. While several case reports of RCC
complicating ADPKD have been described and it has been
found premalignant epithelial cells in the cyst epithelia,
epidemiologic and autopsy studies have not shown a
significant higher incidence of RCC in ADPKD [1, 2].
International Journal of Case Reports and Images, Vol. 6 No. 2, February 2015. ISSN – [0976-3198]
Int J Case Rep Images 2015;6(2):115–117.
www.ijcasereportsandimages.com
Rivera et al. 116
On the other hand, more recent studies have described
that kidney-related prevalence of RCC in patients with
ADPKD and advanced chronic renal failure treated by
hemodialysis or renal transplantation was high [3, 4].
These apparent discrepancies could be explained by the
difficulties in the diagnosis of RCC in this setting and
emphasizes the importance of close clinical surveillance
and the interpretation of several radiological studies such
ultrasonography, CT scan and MRI scan [5, 6].
Herein, we report a case of RCC complicating ADPKD
who has several characteristics. Firstly, he did have neither
hematuria nor symptoms of occult neoplasia. Secondly,
renal function was decreased although dialysis was not
needed. Finally, the combination of ultrasonography and
unenhanced and contrast-enhanced CT scan studies were
able to achieve a diagnosis without using RMI, as it has
been recently recommended.
CONCLUSION
Renal cell carcinoma (RCC) can appear as a
complication of autosomal dominant polycystic kidney
disease (ADPKD). The diagnosis of RCC in this setting
needs a thoroughly radiological evaluation, which should
include ultrasonography and computed tomography scan
studies.
How to cite this article
Rivera F, Redondo CL. Renal carcinoma complicating
autosomal dominant polycystic kidney disease. Int J
Case Rep Images 2015;6(2):115–117.
doi:10.5348/ijcri-201504-CL-10059
*********
Author Contributions
Francisco Rivera – Substantial contributions to
conception and design, Acquisition of data, Analysis
and interpretation of data, Drafting the article, Revising
it critically for important intellectual content, Final
approval of the version to be published
Celia López Redondo – Substantial contributions to
conception and design, Acquisition of data, Analysis
and interpretation of data, Drafting the article, Revising
it critically for important intellectual content, Final
approval of the version to be published
Guarantor
The corresponding author is the guarantor of submission.
Conflict of Interest
Authors declare no conflict of interest.
Copyright
© 2015 Francisco Rivera et al. This article is distributed
under the terms of Creative Commons Attribution
License which permits unrestricted use, distribution
and reproduction in any medium provided the original
author(s) and original publisher are properly credited.
Please see the copyright policy on the journal website for
more information.
REFERENCES
1. Kumar S, Cederbaum AI, Pletka PG. Renal cell
carcinoma in polycystic kidneys: case report and
review of literature. J Urol 1980 Nov;124(5):708–9.
2. Keith DS, Torres VE, King BF, Zincki H, Farrow
GM. Renal cell carcinoma in autosomal dominant
polycystic kidney disease. J Am Soc Nephrol 1994
Mar;4(9):1661–9.
3. Hajj P, Ferlicot S, Massoud W, et al. Prevalence of
renal cell carcinoma in patients with autosomal
dominant polycystic kidney disease and chronic renal
failure. Urology 2009 Sep;74(3):631–4.
4. Jilg CA, Drendel V, Bacher J, et al. Autosomal
dominant polycystic kidney disease: Prevalence
of renal neoplasias in surgical kidney specimens.
Nephron Clin Pract 2013;123(1-2):13–21.
5. Gupta S, Seith A, Sud K, et al. CT in the evaluation
of complicated autosomal dominant polycystic kidney
disease. Acta Radiologica 2000 May;41(3):280–4.
6. Ars E, Bernis C, Fraga G, et al. Spanish guidelines for
the management of autosomal dominant polycystic
kidney disease. Nephrol Dial Transplant 2014 Sep;29
Suppl 4:iv95–105.
Figure 3: (A) Computed tomography scan showing without
intravenous contrast: exophytic isodense nodule compared to
the rest of the renal parenchyma (white arrow), (B) Computed
tomography scan in arterial phase after the administration of
intravenous contrast: early enhancement predominates at the
periphery of the nodule (white arrow).
International Journal of Case Reports and Images, Vol. 6 No. 2, February 2015. ISSN – [0976-3198]
Int J Case Rep Images 2015;6(2):115–117.
www.ijcasereportsandimages.com
Rivera et al. 117
ABOUT THE AUTHORS
Article citation: Rivera F, Redondo CL. Renal carcinoma complicating autosomal dominant polycystic kidney
disease. Int J Case Rep Images 2015;6(2):115–117.
Francisco Rivera is Nephrologist at Hospital General Universitario de Ciudad Real (Spain). He
earned undergraduate and postgraduate degrees of Medicine from Universidad Autónoma de Madrid
(Spain). He has published more than 200 research papers in national and international academic
journals and authored 70 chapters. His research interests include autoimmune primary and secondary
renal diseases and chronic renal failure.
Dra Celia López Redondo is Radiologist at Hospital Santa Bárbara in Puertollano (Spain). She
earned undergraduate and postgraduate degrees of Medicine from Universidad de Córdoba (Spain).
She completed her specialty few months ago. She has published some research papers in national and
international academic journals. Her research interests include oncology and abdominal radiology.
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