尿毒症患者的雙側多發性多型態腎細胞癌
黃烱焜1、林志杰1,2,3、林登龍1,2,3、張延驊1,2,3
台北榮民總醫院 泌尿部1；國立陽明大學醫學院 泌尿學科2；書田泌尿科學研究中心3
Bilateral Multiple Multi-morphology Renal Cell Carcinoma in an End-Stage Renal Disease (ESRD) Patient
Chiung-Kun Huang1, Chih-Chieh Lin1,2,3, Alex T-L Lin1,2,3,Yen-Hwa Chang1,2,3,
Department of Urology, Taipei Veterans General Hospital1; School of Medicine and Shu-Tien Urological Institute2, National Yang-Ming University, Taiwan3
Introduction:
Renal cell cancer represents 2-3% of all cancers. There is a 1.5:1 male predominance, with a peak incidence between 60 and 70 years. Etiological factors include smoking, obesity and hypertension. Renal cell carcinomas comprise a broad spectrum of histopathological entities described in the 2016 World Health Organization (WHO) classification. There are three main RCC types: clear cell (ccRCC), papillary (pRCC - type I and II) and chromophobe (chRCC). Other renal tumors constitute the remaining 10-15% of renal cortical tumors.
Case presentation:
A 74-year-old male with a notable history of hypertension, obesity, chronic atrial fibrillation, old stroke, aortic arch and descending aorta aneurysm status post stent implantation, end-stage renal disease under continuous ambulatory peritoneal dialysis (CAPD) for more than 10 years. He complained of intermittent gross hematuria since 2016/9. Urine cytology showed no malignant cells and PSA was about 2.3ng/ml at that time. CT showed bilateral multiple renal cyst with a largest 3.5cm complex cyst over right upper pole of kidney in 2016/9. Cystoscopy revealed enlarged prostate without kissing. There was no papillary tumor in bladder but multiple erythematous lesions with contact bleeding over trigone and posterior wall. Biopsy showed chronic inflammation. Patient underwent MRI for persisted hematuria and disclosed PI-RADS v2 category 5 lesion at left peripheral zone of prostate in 2017/5. Image fusion targeted biopsy was performed and showed adenocarcinoma of prostate, Gleason grade 4+4, score 8, cT3aN0Mx, iPSA: 2.3ng/ml in 2017/6. Androgen deprivation therapy started since then. Gross hematuria recurred with clot retention in 2017/7. TUR check bleeding was performed with incidental finding of a small papillary lesion at right lateral wall of bladder. Biopsy revealed renal cell carcinoma. CT urography (CTU) was done and revealed bilateral atrophic renal parenchyma with multiple cysts in both kidneys. Right renal cyst about 5.9cm in size showed internal high density and increased in size as compared with previous image in 2016/9. Surgical intervention was rejected by the patient at that time. Gross hematuria requiring blood transfusion recurred one month later. CTU showed blood clot in right collecting system with distended renal pelvis favoring renal tumor with bleeding. Bilateral nephroureterectomy and cystoprostectomy was performed 2 months later. Pathology revealed adenocarcinoma of prostate, Gleason score 4+4, with extraprostatic extension on left posterior region, pT3a (TNM, 2010 and 1997 version). Three tumor lesions were found in right kidney. There were a 4.5 cm Xp11 translocation renal cell carcinoma, Fuhrman grade 3, with renal capsular and pelvicalyceal system invasion, pT3a (AJCC 7th edition, 2010); a 0.8cm clear cell papillary renal cell carcinoma(CCPRCC), Fuhrman grade 2, pT1a and a 0.6cm acquired cystic disease associated renal cell carcinoma, Fuhrman grade 3, pT1a. Six tumor lesions were found at left kidney. There were four 0.6~0.8cm in size, acquired cystic disease associated renal cell carcinoma, Fuhrman grade 3, pT1a and 2 0.8~0.9cm in size clear cell papillary renal cell carcinoma(CCPRCC), Fuhrman grade 2, pT1a.
Currently, he has been followed up for 6 months after operation without evidence of tumor recurrence by CT scan. The patient is doing well and under regular hemodialysis.
Conclusion:
This is a rare case of bilateral multi-morphology renal cell carcinoma in patient with end-stage renal disease (ESRD). Translocation renal cell carcinoma with chromosomal translocations involving TFE3 (Xp11.2), or less frequently, TFEB (6p21). Xp11 translocation RCC was originally described as a pediatric neoplasm representing 20–40% of pediatric RCCs with a much lower frequency in the adult population. Clear cell papillary renal cell carcinoma is increasingly recognized as a distinct tumor with unique morphology. It was less aggressive and without genetic alterations compared to clear cell carcinoma. Cystic degenerative changes (acquired cystic kidney disease [ACKD]) and a higher incidence of RCC are typical features of ESRD. Renal cell cancers of native end-stage kidneys are found in about 4% of patients. Their lifetime risk of developing RCCs is at least ten times higher than in the general population. Compared with sporadic RCCs, RCCs associated with ESRD are generally multicentric and bilateral, found in younger patients (mostly male), and are less aggressive.