Incidental renal cell carcinoma post bilateral nephrectomy in autosomal dominant polycystic kidney disease

BACKGROUND Renal cell carcinoma(RCC)is more common in patients with autosomal dominant polycystic kidney disease(ADPKD)than in the general population.Diagnosing RCC in ADPKD is challenging due to the presence of multiple renal cysts,often leading to delays and difficulties in distinguishing RCC from cyst infection or hemorrhage.A total of 38 kidneys were excised from 19 patients,with a mean age of 56.8 years and an average hemodialysis duration of 84.2 months.Eight patients underwent open nephrectomies,and 11 underwent hand-assisted laparoscopic nephrec-tomies.RCC was detected in 15.8%of kidneys,affecting 21.1%of patients.Two patients had multifocal RCC in both kidneys.All RCC cases were pT1 stage,with the largest lesion averaging 16.5 mm in diameter.The average operative duration was 120 minutes,with intraoperative blood loss averaging 184.2 mL.Five patients required blood transfusions.Postoperative complications occurred in five patients,with a mean hospital stay of 17.1 days.The mean follow-up period was 28.1 months.CONCLUSION The prevalence of RCC is higher in patients with ADPKD with ESRD than in those with ESRD alone.Thus,clinicians should be cautious and implement surveillance programs to monitor the development of RCC in patients with ADPKD,particularly those on dialysis.

Low expression of fatty acid oxidation related gene ACADM indicates poor prognosis of renal clear cell carcinoma and is related to tumor immune infltration

This research aims to identify the key fatty acid beta-oxidation(FAO)genes that are altered in kidney renal clear cell carcinoma(KIRC)and to analyze the role of these genes in KIRC The Gene Expression Omnibus(GEO)and FAO datasets were used to identify these key genes.Wilcoxon rank sum test was used to assess the levels of acyl-CoA dehydrogenase medium chain(ACADM)between KIRC and non cancer samples.The logistic regression and Wilcoxon rank sum test were used to explore the association between ACADM and clinical features.The diagnostic performance of ACADM for KIRC was asessed using a diagnostic receiver operating ch aracteristic(ROC)curve.The co-expressed genes of ACADM were identifed in LinkedOmics database,and their function and pathway enrichment were analyzed.The correlation between ACADM expression level and immune infitration was analyzed by Gene Set Variation Analysis(GSVA)method Additionally,the proliferation,migration,and invasion abilities of KIRC cells were assessed after overexpressing ACADM.Following differential analysis and intersection,we identifed six hub genes,induding ACADM.We found that the expression level of ACADM was decreased in KIRC tissues and had a better diagnostic efect(AUC=0.916).Survival analysis suggested that patients with decreased ACADM expression had a worse prognosis.According to correlation analysis,a variety of dinical features were associated with the expression level of ACADML By analyzing the infiltration level of immune cells,we found that ACADM may be related to the enrichment of immune cells.Finally,ACADM overexpression inhibited proliferation,migration,and invasion of KIRC cells.In conclusion,our findings suggest that reduced ACADM expression in KIRC patients is indicative of poor prognosis.These results imply that ACADM may be a diagnostic and prognostic marker for individuals with KIRC,offering a reference for dinicians in diagnosis and treatment.

Primary small cell carcinoma of kidney after renal transplantation:a case report and literature review

Extrapulmonary small cell carcinoma （EPSCC） is a rare neoplasm comprising 2.5％ to 5％ of small cell carcinomas （SCCs）.Bladder SCC is the most common site of genitourinary tract.Primary renal SCC is extremely rare.We report a case of primary SCC of the kidney which is rarely reported in the urinary tract and presents an aggressive clinical picture.A 59-year-old female visited a urologic clinic with complaint of persistent left flank soreness 10 years after undergoing renal transplantation.Abdominal computed tomography showed a left renal pelvis tumor.After the patient received left nephroureterectomy with bladder cuff resection,her pathology results showed SCC.After surgery,she received adjuvant systemic chemotherapy,and her recovery has been uneventful as of 8 months.Primary renal SCC presents with an advanced tumor stage and a short median survival period,therefore early intervention and close follow-up are recommended.

The Clinical Association of Autosomal Dominant Polycystic Kidney Disease and renal cell Carcinoma

Autosomal Dominant Polycystic Kidney Disease (ADPKD) is not currently considered to be a risk factor for renal cell carcinoma (RCC). We present data from our institution demonstrating incidence of RCC with ADPKD above the incidence rate for RCC in the general population, as well as that in patients with end-stage renal disease (ESRD). The discussion relates our findings in the context of the current literature including recent case reports published in this entity.

Simultaneousoccurrenceoftransitionalcellcarcinomaandrenalcell carcinomainthesamekidney:acasereportandreviewoftheliterature

This article reports a case of simultaneous occurrence of 2 primary renal tumors of different histology, a transitional cell carcinoma and a renal cell carcinoma. in the same kidney. The histological, immunohistochemical and ultrastructural changes of the tumors were described. A review of the literature to date revealed this case to be rare. only 24 other cases were reported previously.

Kidney re-transplantation after living donor graft nephrectomy due to de novo chromophobe renal cell carcinoma:A case report

BACKGROUND There are few reported cases of allograft nephrectomy due to malignancy followed by successful renal re-transplantation two years later.In this paper,we report a patient who underwent kidney re-transplantation after living donor graft nephrectomy due to de novo chromophobe renal cell carcinoma(ChRCC)involving the allograft kidney.CASE SUMMARY A 34-year-old man underwent living kidney transplantation at the age of 22 years for end-stage renal disease.Maintenance immunosuppression consisted of tacrolimus,mycophenolate mofetil(MMF),and prednisone.Six years posttransplantation,at another hospital,ultrasonography revealed a small mass involving the upper pole of the graft.The patient declined further examination and treatment at this point.Seven years and three months post-transplantation,the patient experienced decreasing appetite,weight loss,gross hematuria,fatigue,and oliguria.Laboratory tests showed anemia(hemoglobin level was 53 g/L).Contrast-enhanced computed tomography revealed a large heterogeneous cysticsolid mass involving the upper pole of the renal allograft.Graft nephrectomy was performed and immunosuppressants were withdrawn.Histological and immunohistochemical features of the tumor were consistent with ChRCC.One year after allograft nephrectomy,low doses of tacrolimus and MMF were administered for preventing allosensitization.Two years after allograft nephrectomy,the patient underwent kidney re-transplantation.Graft function remained stable with no ChRCC recurrence in more than 2-years of follow-up.CONCLUSION De novo ChRCC in kidney graft generally has a good prognosis after graft nephrectomy and withdrawal of immunosuppression.Kidney re-transplantation could be a viable treatment.A 2-year malignancy-free period may be sufficient time before re-transplantation.

The Association between Autosomal Dominant Polycystic Kidney Disease and Renal Cell Carcinoma

Objectives: The relationship between autosomal dominant polycystic kidney disease (ADPKD) and renal cell carcinoma (RCC) is investigated to determine a link that would guide management due to elevated RCC risk. Current literature is inconclusive on this topic. Methods: This study is a retrospective chart review of patients having undergone nephrectomy. Those with pathology and history consistent with ADPKD were reviewed for presence of RCC. Results: The review at this institution revealed RCC in 18% of ADPKD patients who underwent nephrectomy. These rates are significantly higher than those found in the general population, and even greater than those would be expected in patients suffering end-stage renal disease (ESRD). Conclusions: Due to the increased prevalence of RCC in ADPKD, clinicians managing patients with ADPKD should maintain a high index of suspicion. Our data suggest a link between ADPKD and RCC, most likely at the genetic level. In light of this, we feel that further genetic research is needed to potentially discover the link between these two disease processes.

Treatment options for localised renal cell carcinoma of the transplanted kidney

Currently,there is no consensus among the transplant community about the treatment of renal cell carcinoma(RCC)of the transplanted kidney.Until recently,graftectomy was universally considered the golden standard,regardless of the characteristics of the neoplasm.Due to the encouraging results observed in native kidneys,conservative options such as nephron-sparing surgery(NSS)(enucleation and partial nephrectomy)and ablative therapy(radiofrequency ablation,cryoablation,microwave ablation,high-intensity focused ultrasound,and irreversible electroporation)have been progressively used in carefully selected recipients with early-stage allograft RCC.Available reports show excellent patient survival,optimal oncological outcome,and preserved renal function with acceptable complication rates.Nevertheless,the rarity and the heterogeneity of the disease,the number of options available,and the lack of long-term follow-up data do not allow to adequately define treatment-specific advantages and limitations.The role of active surveillance and immunosuppression management remain also debated.In order to offer a better insight into this difficult topic and to help clinicians choose the best therapy for their patients,we performed and extensive review of the literature.We focused on epidemiology,clinical presentation,diagnostic work up,staging strategies,tumour characteristics,treatment modalities,and follow-up protocols.Our research confirms that both NSS and focal ablation represent a valuable alternative to graftectomy for kidney transplant recipients with American Joint Committee on Cancer stage T1aN0M0 RCC.Data on T1bN0M0 lesions are scarce but suggest extra caution.Properly designed multi-centre prospective clinical trials are warranted.

Advances of multidetector computed tomography in the characterization and staging of renal cell carcinoma

Renal cell carcinoma(RCC) accounts for approximately 90%-95% of kidney tumors. With the widespread use of cross-sectional imaging modalities, more than half of RCCs are detected incidentally, often diagnosed at an early stage. This may allow the planning of more conservative treatment strategies. Computed tomography(CT) is considered the examination of choice for thedetection and staging of RCC. Multidetector CT(MDCT) with the improvement of spatial resolution and the ability to obtain multiphase imaging, multiplanar and threedimensional reconstructions in any desired plane brought about further improvement in the evaluation of RCC. Differentiation of RCC from benign renal tumors based on MDCT features is improved. Tumor enhancement characteristics on MDCT have been found closely to correlate with the histologic subtype of RCC, the nuclear grade and the cytogenetic characteristics of clear cell RCC. Important information, including tumor size, localization, and organ involvement, presence and extent of venous thrombus, possible invasion of adjacent organs or lymph nodes, and presence of distant metastases are provided by MDCT examination. The preoperative evaluation of patients with RCC was improved by depicting the presence or absence of renal pseudocapsule and by assessing the possible neoplastic infiltration of the perirenal fat tissue and/or renal sinus fat compartment.

Renal cell carcinoma: Evolving and emerging subtypes

Our knowledge of renal cell carcinoma(RCC) is rapidly expanding. For those who diagnose and treat RCC, it is important to understand the new developments. In recent years, many new renal tumors have been described and defined, and our understanding of the biology and clinical correlates of these tumors is changing. Evolving concepts in Xp11 translocation carcinoma, mucinous tubular and spindle cell carcinoma, multilocular cystic clear cell RCC, and carcinoma associated with neuroblastoma are addressed within this review. Tubulocystic carcinoma, thyroid-like follicular carcinoma of kidney, acquired cystic disease-associated RCC, and clear cell papillary RCC are also described. Finally, candidate entities, including RCC with t(6;11) translocation, hybrid oncocytoma/chromophobe RCC, hereditary leiomyomatosis and RCC syndrome, and renal angiomyoadenomatous tumor are reviewed. Knowledge of these new entities is important for diagnosis, treatment and subsequent prognosis. This review provides a targeted summary of new developments in RCC.

Application of the revised Tumour Node Metastasis （TNM） staging system of clear cell renal cell carcinoma in eastern China： advantages and limitations

This study was designed to evaluate whether the revised 2010 Tumour Node Metastasis （TNM） staging system could lead to a more accurate prediction of the prognosis of renal cell carcinoma （RCC） patients. A total of 1216 patients who had undergone radical nephrectomy or partial nephrectomy for RCC from 2003 to 2011 were enrolled. All of the patients had pathologically confirmed clear cell RCC （ccRCC）. All cases were staged by both the 2002 and 2010 TNM staging systems after pathological review, and survival data were collected. Univariate and multivariate Cox regression models were used to evaluate cancer-specific survival （CSS） and progression-free survival （PFS） after surgery. Continuous variables, such as age and tumour diameter, were calculated as mean values and standard deviations （s.d.） or as median values. Survival was calculated by the Kaplan-Meier method, and the log-rank test assessed differences between groups. Statistically significant differences in CSS and PFS were noted among patients in T3 subgroups using the new 2010 staging system. Therefore, the revised 2010 TNM staging system can lead to a more accurate prediction of the prognosis of ccRCC patients. However, when using the revised 2010 staging system, we found that more than 92% of patients （288/313） with T3 tumours were staged in the T3a subgroup, and their survival data were not significantly different from those of patients with T2b tumours. In addition, T2 subclassification failed to independently predict survival in RCC patients.

Microphthalmia family of transcription factors associated renal cell carcinoma

The microphthalmia(MiT)subfamily of transcription factors includes TFE3,TFEB,TFEC,and MITF.In the 2016 World Health Organization classification,MiT family translocation renal cell carcinoma(tRCC)including Xp11 tRCC and t(6;11)RCC,was newly defined as an RCC subtype.Xp11 and t(6;11)RCC are characterized by the rearrangement of the MiT transcription factors TFE3 and TFEB,respectively.Recent studies identified the fusion partner-dependent clinicopathological and immunohistochemical features in TFE3-rearranged RCC.Furthermore,RCC with TFEB amplification,melanotic MiT family translocation neoplasms,was identified may as a unique subtype of MiT family associated renal neoplasms,along with MITF associated RCC.In this review,we will collect available literature of these newly-described RCCs,analyze their clinicopathological and immunohistochemical features,and summarize their molecular and genetic evidences.We expect this review would be beneficial for the understanding of these rare subtypes of RCCs,and eventually promote clinical management strategies.

Synchronous sporadic bilateral multiple chromophobe renal cell carcinoma accompanied by a clear cell carcinoma and a cyst: A case report

BACKGROUND Renal cell carcinomas are usually unilateral.However,they are bilateral in 2%to 4%of sporadic cases and is considerably more common in familial cases.Synchronous sporadic bilateral multiple chromophobe renal cell carcinoma(CHRCC)with different subtypes is rare.CASE SUMMARY In this case report,we describe a case of synchronous bilateral CHRCC with two histological variants,accompanied by a clear cell carcinoma and a cyst in a 50-year-old male.The patient underwent retroperitoneal laparoscopic bilateral nephron-sparing surgery and there was no serious postoperative renal dysfunction.CONCLUSION We report a rare case of synchronous bilateral CHRCC with two histological variants associated with a clear cell carcinoma and a cyst.

Recurrent renal cell carcinoma leading to a misdiagnosis of polycystic liver disease: A case report

BACKGROUND Polycystic liver disease(PCLD) with a large cystic volume deteriorates the quality of life of patients through substantial effects on the adjacent organs,recurrent cyst infections, cyst rupture, and hemorrhage. Surgical or radiological intervention is usually needed to alleviate these symptoms. We report a rare case of the cystic metastasis of renal cell carcinoma(RCC), which was misdiagnosed as PCLD, as a result of the clinical and radiological similarity between these disorders.CASE SUMMARY A 74-year-old female who had undergone nephrectomy for papillary-type RCC(PRCC) was suffering from abdominal pain and the recurrent intracystic hemorrhage of multiple cysts in the liver. Imaging studies and aspiration cytology of the cysts showed no evidence of malignancy. With a diagnosis of autosomal dominant polycystic liver disease, the patient received hepatectomy for the purpose of mass reduction and infectious cyst removal. Surgery was performed without complications, and the patient was discharged on postoperative day 14. Postoperatively, the pathology revealed a diagnosis of recurrent PRCC with cystic formation.CONCLUSION This case demonstrates the importance of excluding the cystic metastasis of a cancer when liver cysts are observed.

Ipsilateral synchronous papillary and clear renal cell carcinoma:A case report and review of literature

BACKGROUND There is limited information on ipsilateral synchronous papillary renal cell carcinoma(PRCC)and clear cell renal cell carcinoma(CCRCC).Therefore,these rare tumors are often misdiagnosed preoperatively as a single tumor with intrarenal metastasis or some other diseases.Effective management and long-term overall survival might be affected because the prognosis of the two tumors differs.CASE SUMMARY We describe a case of ipsilateral synchronous PRCC and CCRCC with two histological variants in a 72-year-old man,whose mass was found incidentally,with no other chief complaints and vital signs were normal.Initial ultrasound revealed a hypoechoic lobular mass with a volume of 7.8 cm×4.8 cm×2.8 cm in the middle to lower pole of the left kidney.A subsequent contrast-enhanced computed tomography scan showed a single endophytic mass of 7.5 cm in diameter.The patient underwent laparoscopic left radical nephrectomy.A final diagnosis of ipsilateral synchronous PRCC and CCRCC was confirmed by pathological examination.There was no recurrence or metastasis after 25 mo follow-up.CONCLUSION We report a case of ipsilateral synchronous PRCC and CCRCC,and review related literature to estimate the prevalence of similar cases.The above descriptions may be expected to help understand the disease,and improve diagnosis in the future.

Thyroid follicular renal cell carcinoma excluding thyroid metastases:A case report

BACKGROUND Thyroid follicular renal cell carcinoma is a special type of renal cell carcinoma newly recognized in recent years.It has attracted attention because of its unique histology,immunophenotype,and clinical characteristics.It has a very low incidence,and the number of case reports available for review is limited.Moreover,a thyroid mass with type of tumour is rare.CASE SUMMARY We report a case of a renal mass with a bilateral thyroid mass that was accidentally discovered in a 60-year-old man during physical examination.B-mode ultrasound showed a hypoechoic mass in the middle and lower parenchyma of the right kidney,and computed tomography showed an iso-density shadow tumour in the right kidney.Contrast agents had a significant continuous enhancement effect on the tumour,and the enhancement was not uniform.After partial nephrectomy,pathological analysis was performed to rule out the possibility that the renal tumour was caused by thyroid tumour metastasis.Needle biopsy of the thyroid tumour confirmed that the renal cell carcinoma was not related to the thyroid tumour.The patient was alive at the last postoperative follow-up.CONCLUSION This is the third published case in which thyroid tumour biopsy was performed to confirm that thyroid follicular renal cell carcinoma is not thyroid related.

Partial Nephrectomy for Renal Cell Carcinoma: Risk Factors for Acute Post-Operative Hemorrhage and Impact on Subsequent Hospital Course and Complete Nephrectomy Rate. An Analysis of 200 Consecutive Cases

Purpose: Clinical guidelines recommend partial nephrectomy (PN) as the preferred method of surgical excision of the small renal tumor whenever feasible. PN has comparable cancer cure rates to that of radical nephrectomy in this setting, and decreased risk of chronic kidney disease. A recognized devastating complication following partial nephrectomy is acute post-operative hemorrhage (APOH) from the reconstructed kidney. Risk factors for hemorrhage following partial nephrectomy remain poorly elucidated, as does the impact of hemorrhage on subsequent hospital stay. Identification of risk factors for hemorrhage may lead to a better understanding of and reduction of this complication. Material and Methods: We utilized a prospectively managed database comprised of patients undergoing open partial nephrectomy at our institution by the same surgical team from January 2006 to July 2012. Clinicopathologic factors assessed APOH for their relationships, including patient age, gender, diabetes, smoking, hypertension, coronary artery disease, American Society of Anesthesia Score (ASA), tumor size, RENAL nephrotomy score, pathologic result, cancer margin status, operative time, and intra-operative blood loss. The impact of APOH on subsequent hospital course was evaluated and compared with the entire cohort. Results: Data were analyzed from 200 consecutive patients. We identified 7 patients (3.5%) who experienced APOH. Compared with the entire cohort, APOH resulted in an increased hospital length of stay (median, 5 days;range, 2-11 days, p = 0.001), an increased transfusion requirement (median, 6 units;range, 1-16 units. p = 0.001), a greater risk of selective angiographic embolization (median, 2 procedures;range, 0-3, p = 0.001), and completion nephrectomy (n = 2, p = 0.001). One patient in the APOH group experienced cardiac arrest and was resuscitated. Clinicopathologic factors associated

Ambiguous clear cell carcinoma in medullary sponge kidney:A case report

Medullary sponge kidney(MSK)is a characteristic renal malformation,with a relatively low incidence.Radiologically,identification of MSK is sometimes ambiguous when compared to a renal mass.Here,we report a novel renal clear cell carcinoma in MSK,and discuss our approach to treatment.We recommended that a preoperative biopsy should be performed,followed by a comprehensive discussion regarding the appropriate perioperative preparations and careful surgical techniques that should be performed for this complex disease.

Nephron-sparing surgery for small renal cell carcinoma

Objective：To investigate the method and effect of nephron-sparing surgery in the treatment of small renal cell carcinoma. Methods： From August 1 997 to October 2008, 48 cases of small renal cell carcinomas were confirmed by surgery and pathology, and reviewed retrospectively. Of the 48 cases, there was 1 patient with bilateral tumors, 8 with solitary kidney tumors, 1 with unilateral tumor and a damaged contralateral kidney, and 38 with unilateral tumors and a normal contralateral kidney; 9 underwent tumor enucleation and the remaining patients received partial nephrectomy. Results：There were no local tumor recurrences and/or tumor metastasis at a mean followup of 60 months. Conclusion： Confirming conclusions from other centers, we have found that nephron-sparing surgery is an effective treatment for small renal cell carcinomas.

Decreased cross-sectional muscle area in male patients with clear cell renal cell carcinoma and peritumoral collateral vessels

BACKGROUND Sarcopenia is the loss of skeletal muscle mass(SMM)and is a sign of cancer cachexia.Patients with advanced renal cell carcinoma(RCC)may show cachexia.AIM To evaluate the amount of SMM in male clear cell RCC(ccRCC)patients with and without collateral vessels.METHODS In this study,we included a total of 124 male Caucasian patients divided into two groups:ccRCCa group without collateral vessels(n=54)and ccRCCp group with collateral vessels(n=70).Total abdominal muscle area(TAMA)was measured in both groups using a computed tomography imaging-based approach.TAMA measures were also corrected for age in order to rule out age-related effects.RESULTS There was a statistically significant difference between the two groups in terms of TAMA(P<0.05)driven by a reduction in patients with peritumoral collateral vessels.The result was confirmed by repeating the analysis with values corrected for age(P<0.05),indicating no age effect on our findings.CONCLUSION This study showed a decreased TAMA in ccRCC patients with peritumoral collateral vessels.The presence of peritumoral collateral vessels adjacent to ccRCC might be a fine diagnostic clue to sarcopenia.