A case of bilateral renal cell carcinoma showing a rapid course of death in a long-term hemodialysis patient due to polycystic kidney

A case of bilateral renal cell carcinoma in a 42-year-old polycystic kidney male is reported. He had been treated with hemodialysis for 22 years. An abnormal small mass was found in one of the left renal cystic lesions by screening ultrasonography and CT scan at the 19th year of the hemodialysis. Left radical nephrectomy was performed and the histological diagnosis was a renal cell carcinoma (RCC). There was no evidence of recurrence and metastasis, however, he presented with asymptomatic macrohematuria two years after the operation. CT scan demonstrated the rapidly progressing right renal tumor and multiple para-aortic lymph node swelling. Right nephrectomy and lymphadenectomy were performed and pathological examination showed the advanced RCC with multiple lymph node metastasis. Eleven months after the second operation followed by interferon therapy. he died of multiorgan metastasis of the RCC. This is the first bilateral RCC case in polycystic kidney patient treated with hemodialysis in Japan.     

Kidney Transplantation in a Patient With Tuberous Sclerosis Complex: A Case Report

BackgroundTuberous sclerosis complex (TSC) is a rare autosomal dominant genetic disease caused by mutations of either of 2 genes, TSC1 and TSC2. Renal manifestations include angiomyolipomas (AMLs), multiple cysts, and renal cell carcinoma. AMLs increase bleeding tendency and the risk of renal insufficiency which end-stage develops in 1% of affected patients.Case reportA 38-year-old woman suffering from TSC since early childhood has developed multiple complications associated with this disease. The patient was diagnosed with brain tumor—giant cell astrocytoma—which was removed in 1992. In 2006, right nephrectomy was performed due to the unsuccessful right renal artery embolization after the massive hemorrhage into the AML. Moreover, the right idiopathic pneumothorax occurred twice. Therefore, the video-assisted thoracoscopic surgery and pleurodesis were conducted (2006, 2013). The patient is intellectually disabled and unable to make decisions on her own. Her legal guardians (parents) make all decisions associated with her treatment. Diagnostic and therapeutic procedures demanding cooperation were conducted under anesthesia. Because of end-stage renal failure, the patient required the renal replacement therapy (RRT). Preemptive kidney transplantation (KTx) was the best solution for this patient. Procedures such as hemodialysis and peritoneal dialysis were infeasible to perform due to the intellectual disability that inhibits essential cooperation. During KTx qualification tests, the expanding AML with risk of hemorrhage was noticed. The patient was qualified for simultaneous left nephrectomy and KTx from the living donor (her father). The surgery was performed on the 2nd of June 2020. The patient is looked after by her parents, stays in good general condition. The patient's creatinine level is maintained at 0.6 to 0.8 mg/dL.ConclusionPatients with significant intellectual disability that prevents maintaining conscious cooperation who require RRT must have individually adjusted therapy. In the case of the presented patient, it was decided to perform the preemptive kidney transplantation from her determined father.     

A case of renal cell carcinoma in tuberous sclerosis

A 43-year-old woman with mental retardation, epilepsy, and urinary stone disease had a right renal tumor. Acne-like anthema around the nose and dental pits of the nine teeth were typical signs of tuberous sclerosis (TSC), and the biopsy finding of the facial anthema was consistant with TSC. The pathological diagnosis of laparoscopic nephrectomy was renal cell carcinoma in the hemorrhagic cyst. The TSC-related renal cell carcinoma tends to develop bilaterally in younger individuals compared with the sporadic RCC. This case is the 27th case of TSC-related RCC in Japan.     

Solitary breast mass as initial presentation of clinically silent metastatic renal cell carcinoma

Metastasis to the breast from extramammary tumors is rare. Breast metastases of renal cell carcinoma (RCC) origin have been described in sporadic case reports. We present a patient with a solitary breast mass representing the manifestation of clinically silent, metastatic RCC. A 76-year-old female was 12 years prior removed from radical nephrectomy for localized RCC. Her new breast mass was identified on physical examination. Pathology of the resected mass was diagnostic of metastatic RCC and subsequent imaging studies demonstrated a 1.9 cm renal mass in her solitary kidney. The patient elected subcutaneous Interleukin-2 immunotherapy as primary treatment for her recurrent RCC.     

Collecting duct carcinoma with acquired cystic disease of the kidney in a long-term hemodialysis patient

Abstract:   We report a very rare case of collecting or Bellini duct carcinoma (CDC) found in a 60-year-old male who had received hemodialysis therapy for 21 years. Screening with ultrasonography revealed a solid tumor originating from the cyst wall in the right kidney with acquired cystic disease of the kidney. Subsequent computed tomography (CT) and angiography could not detect another renal tumor. Right radical nephrectomy was performed. The tumor detected preoperatively was composed of papillary renal cell tumor (RCC) and multiple clear cell carcinoma, pathologically. In addition to the tumors, CDC was revealed in the central medulla with the involvement of regional lymph nodes. Three months later, left nephrectomy was performed because left RCC was suspected during CT. The histological diagnosis was multiple clear cell carcinomas. Peritonitis carcinomatosa appeared and the patient died 13 months later.     

Synchronous ipsilateral conventional renal cell and transitional cell carcinoma

Simultaneous occurrence of renal cell carcinoma (RCC) and transitional cell carcinoma (TCC) in the same kidney is unusual. We report a 61-year-old man with ipsilateral synchronous renal adenocarcinoma and renal pelvic TCC. He was referred to our department for gross hematuria and right flank pain. CT and MRI studies revealed a 57 × 50 mm irregular and infiltrative upper right kidney mass with necrotic components. A right radical nephrectomy was done. Pathological diagnosis was a high grade tumor originating from just beneath the intact urothelium of renal pelvis and infiltrating through the parenchyma showing solid and occasional tubular growth patterns. A second tumor in close proximity to the first was reported as well differentiated RCC. This is a rare case of combined renal malignancies.     

Cystic renal cell carcinoma,suspected because of lack of regression of renal cysts after renal transplantation in a dialysis patient with acquired renal cystic disease

A 34-year-old man who had been on dialysis for about 6 years, and had acquired renal cystic disease, received a renal transplantation. However, in spite of the success of the transplantation, one area without cyst regression was observed in the original kidney. Therefore, carcinoma was suspected and nephrectomy was performed. Histology revealed cystic renal cell carcinoma (RCC). No case of cystic RCC occurring in a dialysis patient has previously been reported. Cystic RCC should be suspected in a cystic mass in the original kidney which does not regress after successful renal transplantation. Received: August 23, 2002 / Accepted: November 25, 2002 Acknowledgments We thank Dr. Shigehiko Sato, Department of Internal Medicine, Tonami General Hospital, who referred this patient to us. Correspondence to:I. Ishikawa     

Renal oncocytomatosis in a long-term hemodialysis patient treated by laparoscopic surgery

A 50-year-old female, who had been on maintenance hemodialysis for 22 years, consulted our clinic because of a left renal mass detected incidentally by ultrasonography. Computed tomography (CT) demonstrated a solid hypervascular mass, suggesting a renal cell carcinoma (RCC), in the left atrophic kidney. Left hand-assisted laparoscopic nephrectomy (HALN) was performed. The histopathological diagnosis was renal oncocytomatosis. Renal oncocytomatosis in a long-term hemodialysis patient is extremely rare. We report the first case of renal oncocytomatosis in a long-term hemodialysis patient treated with hand-assisted laparoscopic nephrectomy.     

Membranous nephropathy complicated by renal cell carcinoma

We experienced a patient with membranous nephropathy (MN) and renal cell carcinoma (RCC). Although MN is known to be commonly associated with malignant tumors, association with RCC is rare. In our patient, no malignant focus was found in the major organs examined, except for a small lesion in the right kidney. Diagnosis of a small RCC (1.6 × 1.6cm), which was made by renal biopsy and histology, was impossible by other, imaging, methods. As surgical treatment for the patient, laparoscopic partial nephrectomy was performed successfully, alleviating the symptoms of nephrotic syndrome for up to 21 months.     

Renal cell carcinoma with a huge solitary metastasis to the contralateral adrenal gland: A case report

Renal cell carcinoma (RCC) is capable of metastasizing to several organs. Synchronous isolated contralateral adrenal metastasis of the primary RCC is, however, very rare. Herein we report a case of RCC with a huge solitary metastasis to the contralateral adrenal gland that was surgically treated. We scheduled nephrectomy for the left primary RCC and adrenalectomy for the right adrenal tumor. However, at surgery we found a huge right adrenal tumor that had invaded the right kidney, right renal vein, and inferior vena cava. Therefore right nephrectomy was performed simultaneously with resection and reconstruction of the inferior vena cava. Pathological findings demonstrated that the left renal tumor and right adrenal tumor had the same histology. Although the patient required hemodialysis, he remains well at six months postoperatively. So far, there have been only two cases of a solitary contralateral metastatic adrenal tumor that was larger than the primary RCC, thus the present case is the third one.     

Bilateral renal cell carcinoma in a patient receiving long-term dialysis

Bilateral renal cell carcinoma developing in a 55-year-old male receiving long-term dialysis is reported. The patient, who had undergone maintenance hemodialysis for 12 years, was admitted in July, 1986 for the purpose of extensive examination of a right renal mass. CT scan and ultrasonography demonstrated an enlarged bilateral kidney associated with multiple cysts, containing a solid mass in the right upper pole. The patients underwent right nephrectomy under the diagnosis of right renal tumor. The solid tumor 4 x 3 cm in size revealed a grade 1 to 2 renal cell carcinoma pathologically. Two small tumors were recognized in other portions of the kidney. Multiple cysts of varying sizes, the largest 2 cm in diameter, replaced the renal parenchyma. The cyst walls frequently contained hyperplastic changes. The patient has been subsequently followed up for 2 years and 9 months and underwent left nephrectomy for suspected left renal tumor. The surface of the left kidney was covered by numerous cysts. The result of pathological examination was renal cell carcinoma, which was recognized in a total of 7 regions of the left kidney. The patient remains well on hemodialysis, with no evidence of recurrence or metastasis.     

长期血透患者获得性囊性肾病合并肾癌8例报告并文献复习

目的:学习长期血透患者获得性囊性肾病合并肾癌的筛查和诊治方法。方法:回顾性分析我院维持性血透获得性囊性肾病合并肾癌患者8例,均为B超和CT诊断为双肾多发性囊肿合并肾实质性占位,并行后腹腔镜下根治性肾切除术,术后维持规律性血透,并严密随访。结果:长期血透患者226例,获得性囊性肾病105例(46.5%),获得性囊性肾病合并肾癌8例(3.5%),在获得性囊性肾病中发生率为7.6%(8/105),其中男5例,女3例,年龄(58.6±16.4)岁,血透(12.2±6.9)年。8例患者(9次)行后腹腔镜下根治性肾切除术,手术均成功,出血(45.2±20.3)ml,手术时间(72.5±20.3)min,无严重手术并发症,术后病理3例为透明细胞癌和6例为乳头状癌。住院天数为(7.5±2.4)d。随访12～63个月,无瘤存活5例。结论:肾癌在获得性囊性肾病患者中发病率高,随着血透患者寿命的延长,血透3年后需重视和建立肾癌筛查机制,腹腔镜下根治性肾切除术安全有效、恢复快,并注重患者心脑血管疾病及糖尿病等并发症的积极治疗,有助于进一步延长血透患者寿命。     

A case of acquired cystic disease of bilateral kidneys with renal cell carcinoma following long-term hemodialysis]

We report a case of bilateral renal cell carcinoma which developed during about 14 years of hemodialysis. The patient of male was a 39-year-old with a chief complaint of macrohematuria 14 years prior to dialysis therapy. Computed tomography revealed multiple cystic changes of bilateral kidneys and a high density area in the right kidney. He was admitted to our department in April 1988. He was suspected of renal cell carcinoma of the right kidney and underwent transperitoneal radical nephrectomy on the right kidney. The left kidney was also resected simultaneously in part because it failed to function and in part because the long-term hemodialyzed patients are considered to have a complication of renal cell carcinoma at a high frequency. Pathological diagnosis was bilateral acquired cystic disease of the kidney (ACDK) with renal cell carcinoma. The kidney develops multiple cystic changes following long-term hemodialysis, which forms a high risk for development of renal cell carcinoma. This is a case of bilateral renal cell carcinoma which occurred after long-term hemodialysis, was reported with discussion and reference to the literature.     

Chromophobe renal cell carcinoma and 'capsulomas' with acquired cystic disease of the kidney in a long-term hemodialysis patient

Abstract:   Patients receiving long-term hemodialysis tend to develop renal cell carcinoma (RCC). Among such cases, chromophobe RCC and so-called 'capsulomas' are rarely reported. Here, we report a case of a Japanese woman in her early 70s, who developed both renal lesions after 17 years of hemodialysis. The patient received radical nephrectomy for enlarging renal mass. Grossly, the resected kidney showed a dominant tumor and small-sized subcapsular nodules. Histologically, two types of neoplasm, chromophobe RCC and 'capsuloma', existed with acquired cystic disease of the kidney. Chromophobe RCC had eosinophilic cytoplasm with perinuclear halos, and some tumor cells showed oncocytic features. Hale's colloidal iron staining showed a weakly positive cytoplasmic reaction. Immunohistochemistry was diffusely positive for cytokeratin 7, but negative for vimentin in the tumor cells. 'Capsulomas' were multiple subcapsular nodules composed almost entirely of smooth muscle-like cells with immunoreactivity for melanosome-associated antigen detected by HMB-45.     

A case of renal cell carcinoma arising in bilateral original kidneys after failure of transplant graft function]

We report a case of renal cell carcinoma arising in bilateral original kidneys after failure of transplant graft function. A 47-year-old man had received a living related renal transplantation on December 10, 1985. He had resumed hemodialysis (HD) therapy because of graft failure on January 8, 1996. Periodic computed tomography (CT), after resumption of HD, revealed multiple cystic change in bilateral original kidneys and a mass in the right kidney. He was referred to our hospital on August 4, 1998 for management of the increased right renal lesion. Abdominal angiography demonstrated a hypervascular and solid mass not only in the right kidney but also in the left kidney. He underwent transperitoneal bilateral nephrectomy. Histopathological examination revealed renal cell carcinoma, bilaterally, with alveolar type and granular cell subtype. He was free of evidence of recurrence and metastasis for 30 months after nephrectomy.     

Acquired renal cystic disease

In 1977, Dunnill et al. described a new disorder, bilateral multiple renal cystic disease. It occurred among hemodialysis patients whose original illness had not been cyst-related. Acquired cystic disease of the kidney (ACDK) is commonly observed in patients undergoing hemodialysis. The incidence of ACDK is 40-50% in reports of autopsy and surgical specimens, rising to more than 90% after 5-10 years of dialysis. The volume of the kidneys decreases in the first 3 years of dialysis and then increases as the rate of cyst formation increases. In male patients undergoing long-term hemodialysis the incidence of ACDK is markedly high. ACDK is also found in patients before hemodialysis. The primary concern in patients with ACDK is the increased incidence (5-19%) of renal cell carcinoma (RCC). The incidence is about twelve to eighteen times higher than that in the general population and the cancers may be asymptomatic. Therefore, screening is essential if carcinomas are to be detected early. Regular screening by ultrasonic examination or CT scan is needed. A patient requires nephrectomy when the kidney cancer exists or is suspected by dynamic CT scan. Nephrectomy is performed only on the side with renal mass. It has been argued that RCC associated with ACDK are innocuous and do not predispose the patient to an increased risk of death from RCC. RCC arising from ACDK is considered to be a tumor of low malignant potential, compared with classic RCC. However, RCC has been reported to metastasize in 16% of the patients on dialysis and to be the cause of death in 2% of the kidney transplant recipients. The etiology of ACDK is unclear and its incidence increases with the duration of dialysis. ACDK patients have a propensity to develop adenocarcinoma. The increased incidence of RCC in ACDK patients warrants careful radiologic monitoring of end-stage kidneys in selected patients.     

Bilateral renal cell carcinoma associated with polycystic kidney disease: case report and literature review

A 56-year-old Japanese man consulted a urologist because of urethral bleeding. He had been undergoing hemodialysis for the past 15 years due to polycystic kidney disease. Computed tomography revealed an irregular cyst wall in the left kidney. Since a neoplasm could not be ruled out, we removed the left kidney, by laparoscopic radical nephrectomy after obtaining the patient's consent. Histopathologic diagnosis was renal cell carcinoma. Fourteen months after the operation, urethral bleeding recurred. Further examination of the bladder and the urethra revealed no significant abnormalities. The patient insisted on right nephrectomy. Therefore, laparoscopic radical nephrectomy was performed. Histopathologic diagnosis was also renal cell carcinoma. Renal cell carcinoma in patients with end-stage renal disease is fairly common and is associated with acquired cystic kidney disease. However, renal cell carcinoma associated with polycystic kidney disease is extremely rare.     

Two cases of renal cell carcinoma arising in the native kidney following renal transplantation--clinical study and review of 26 cases reported in Japan

Renal transplant recipients have an increased risk of developing malignancies due to long-term immunosuppression. Two cases of renal cell carcinoma arising from the native kidney following renal transplantation are reported. Case 1; A 60-year-old man underwent cadaveric renal transplantation on July 31, 1995. He had resumed hemodialysis because of graft failure on June 5, 2000. He was referred to our hospital with macroscopic hematuria on March, 2002. Computed tomography (CT) demonstrated a right renal mass of about 3.0 cm in diameter with bilateral acquired cystic disease of the kidney (ACDK). The radical nephrectomy was performed. Case 2; A 55-year-old man underwent cadaveric renal transplantation on November 15, 1996. He had resumed hemodialysis because of graft failure on June 5, 1996. A left renal mass measuring 3.0 cm in diameter as well as bilateral ACDK were found by CT on July, 2002. Left radical nephrectomy was performed. The histological diagnosis of both renal tumors was renal cell carcinoma, clear cell type, G2, pT1a. Fifty-six renal transplantations (48 were renal allografts from cadavers, while 8 were from living donors) were performed in our hospital from 1980 to 2002. Two patients (3.6%) developed renal cell carcinoma after transplantations. We also discuss a clinical study and review of 26 cases reported in Japan. Since the incidence of malignant neoplasia is high, CT and ultrasonography should be performed routinely on the patients with renal transplantation.     

Acquired renal cystic disease mimicking adult polycystic kidney disease in a patient undergoing long-term hemodialysis.

Acquired renal cystic disease (ARCD) is defined as the development of multiple cysts in the renal cortex and medulla in patients with chronic renal failure who are free from congenital polycystic kidney disease. ARCD develops generally in contracted kidneys. We report a case of grotesque enlargement of a single kidney in a patient who had been receiving hemodialysis for 18 years. Although the exact causes of ARCD are not known, 3 factors may contribute to the development of nephromegaly; the sex, the duration of hemodialysis and previous unilateral nephrectomy. As in polycystic disease, when the involved kidney reaches considerable size, ARCD may have a favorable effect on anemia caused by chronic renal failure.     

Residual renal function after partial or radical nephrectomy for renal cell carcinoma

Renal cell carcinoma (RCC) is often detected incidentally and early. Currently, open partial nephrectomy and laparoscopic total nephrectomy form competing technologies. The former is invasive, but nephron-sparing; the other is considered less invasive but with more loss of renal mass. Traditionally, emphasis has been placed on oncologic outcomes. However, a patient with an excellent oncologic outcome may suffer from morbidity and mortality related to renal failure. Animal models with hypertension and diabetic renal disease indicate accelerated progression of pre-existing disease after nephrectomy. Patients with RCC are older and they have a high prevalence of diabetes and hypertension. The progression of renal failure may also be accelerated after a nephrectomy. Our analysis of the available literature indicates that renal outcomes in RCC patients after surgery are relatively poorly defined. A strategy to systematically evaluate the renal function of patients with RCC, with joint discussion between the nephrologist and the oncologic team, is strongly advocated.