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RENAL CELL CARCINOMA IN THE AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE
TIHANA ŠIMUNDIĆ
; Osijek University Hospital Centre, J. J. Strossmayer University, Faculty of Medicine, Department of Internal Medicine, Department of Nephrology, Osijek, Croatia
JOSIP PASINI
; Zagreb University Hospital Centre, Department of Urology,Department for Kidney Transplantation, Zagreb , Croatia
LJUBICA BUBIĆ FILIPI
; Zagreb University Hospital Centre, Department of Internal Medicine, Department of Nephrology, Arterial Hypertension and Kidney Transplantation, Zagreb , Croatia
TAJANA TURK
; Osijek University Hospital Centre, Department for Diagnostic and Interventional Radiology, J. J. Strossmayer University in Osijek, Faculty of Medicine, Osijek, Croatia
Abstract
Aim: The aim of this case report is to point out the specifi city of clinical, diagnostic and therapeutic approach to the patient with the autosomal dominant polycystic kidney disease (ADPKD), renal cell carcinoma along with preserved kidney function. We used patient medical chart, as well as relevant literature from online medical databases (PubMed, EM-base). Case report: We describe a case of a 63-year-old patient with ADPKD, renal cell carcinoma and preserved kidney function until nephrectomy. ADPKD along with hepatic cysts has been known since 2009. Because of the suspicious renal mass detected by ultrasound, non-contrast computed tomography (CT) was performed in 2009, which did not confi rm the presence of renal tumor. In 2012, the patient suffered right-fl ank pain and therefore underwent contrast CT and magnetic resonance imaging (MRI), which confi rmed renal tumor that morphologically seemed like oncocytoma. For that reason, he has been under urologist supervision ever since 2012. Laboratory blood and urine test results were within the normal range all the time and the patient only complained of right-fl ank pain. Further follow up revealed enlargement of the renal mass on MRI and contrast CT. The patient was informed about his condition from the beginning, but he did not accept nephrectomy. However, in December 2015, he agreed and radical nephrectomy of the right kidney was performed. Histopathologic
report showed that it was a clear renal cell carcinoma, 6,5x5x5cm, pT3aN0MX (tumor invaded renal sinus fat). In the fi rst three postoperative days, a decline in kidney function was observed, with serum creatinine up to 165 μmol/L. At patient discharge from the hospital, creatinine was 132 μmol/L and urea 9.8 mmol/L. Three months after the operation, serum creatinine was stable (135 μmol/L) and multi-slice CT of the thorax, abdomen and pelvis was normal. Regarding the histopathologic report, the patient was advised to present for follow up by both urologist and nephrologist because of the evident kidney failure that had begun after nephrectomy. Discussion: Renal cell carcinoma is an infrequent complication of ADPKD. It does not occur with increased frequency when compared to the general population. The diagnosis of renal cell carcinoma is more diffi cult to establish in ADPKD than in the general population since fi ndings such as hematuria, fl ank mass, or complex cysts are common in ADPKD in the absence of malignancy. Malignancy should be suspected if the patient complains of systemic signs and symptoms (fever, anorexia, fatigue, weight loss) or if there is rapid growth of a complex cyst. However, there are several characteristics of renal cell carcinoma in ADPKD, i.e. fever, and tumors are more often bilateral, multicentric and sarcomatoid. CT scanning with contrast and MRI are often able to distinguish malignancy from a complex cyst. MRI is considered to be superior to CT in detecting renal cancers. Considering that clinical, radiological and histologic presentation of renal cell carcinoma in this case report was rather unusual, along with the fact that the patient had refused nephrectomy for several years, we can conclude that the outcome is principally favorable for the patient. Conclusion: We present an instructive case of renal cell carcinoma in a patient with ADPKD. Clinical presentation, radiological and histologic characteristics are different from the usual presentation of renal cell carcinoma in ADPKD. It is necessary to keep in mind an individual approach.
Keywords
renal cell carcinoma; kidney failure; autosomal dominant polycystic kidney disease
Hrčak ID:
216551
URI
Publication date:
30.1.2019.
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