Sarcomatoid renal cell carcinoma with autosomal dominant polycystic kidney disease: a case report and literature review
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CASE REPORT
Sarcomatoid renal cell carcinoma with autosomal dominant polycystic kidney disease: a case report and literature review Yuji Hakozaki1 · Kiyotaka Uchiyama2,3 · Akane Yanai3 · Daisuke Yamada4 · Yuka Kamijo3 · Yoshitaka Ishibashi3 Received: 8 February 2020 / Accepted: 4 October 2020 © Japanese Society of Nephrology 2020
Abstract Diagnosis of renal cell carcinoma (RCC) in patients with autosomal dominant polycystic kidney disease (ADPKD) is challenging and often delayed due to accompanying multiple renal cysts. Sometimes, it is difficult to distinguish RCC from cyst infection or hemorrhage. We herein present the case of a patient with ADPKD undergoing long-term hemodialysis whose sarcomatoid RCC was difficult to diagnose and was confirmed via nephrectomy. A 53-year-old male, undergoing hemodialysis since 20 years for end-stage renal disease secondary to ADPKD, was admitted to our hospital with a 3-week history of fever at > 38 °C and right flank pain. Clinical manifestations were compatible with cyst infection. Magnetic resonance images of the lesion identified in the lower right kidney, revealing slightly high signal intensity on T1-weighted images, low signal intensity on T2 weighted images, and restricted diffusion on diffusion-weighted images, were also consistent with those of cyst infection. Therefore, antibiotic therapy with ciprofloxacin, doripenem, and vancomycin was initiated. However, the patient’s symptoms did not improve. Consequently, right nephrectomy was performed for both diagnosis and treatment, which revealed a sarcomatoid RCC with metastasis to the regional lymph node. The patient gradually developed cachexia and died on day 106 post-admission. The present case illustrates the difficulty of diagnosing RCC in patients with ADPKD, particularly sarcomatoid RCC, which is a rare and aggressive variant of RCC, even with the use of various types of imaging modalities. An early decision of nephrectomy may be necessary in such cases. Keywords Autosomal dominant polycystic kidney disease · Renal cell carcinoma · End-stage renal disease · Hemodialysis · Sarcomatoid
Introduction Autosomal dominant polycystic kidney disease (ADPKD) is the most common autosomal dominant hereditary renal disease, which frequently leads to end-stage renal disease * Kiyotaka Uchiyama [email protected] 1
Department of Urology, Graduate School of Medicine, The University of Tokyo, 7‑3‑1 Hongo, Bunkyo‑ku, Tokyo 113‑8654, Japan
2
Division of Endocrinology, Metabolism and Nephrology Department of Internal Medicine, Keio University School of Medicine, 35 Shinanomachi, Shinjuku‑ku, Tokyo 160‑8582, Japan
3
Division of Nephrology, Japanese Red Cross Medical Center, 4‑1‑22 Hiroo, Shibuya‑ku, Tokyo 150‑8935, Japan
4
Department of Radiology, St Luke’s International Hospital, 9‑1 Akashi‑cho, Chuo‑ku, Tokyo 104‑8560, Japan
(ESRD), necessitating dialysis during or after the sixth decade of life. The estimated prevalence of ADPKD is 1 in 1000–2500 individuals [1]. Renal cell carcinoma (RCC), which originat
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