Tubulocystic Renal Cell Carcinoma Research Articles

A Bosniak III Cyst Unmasking Tubulocystic Renal Cell Carcinoma in an Adolescent: Management with Selective Arterial Clamping and Robotic Enucleation

The Bosniak classification of renal cysts aims to provide a probabilistic risk assessment indicating the likelihood of malignancy from imaging findings. Originally designed to classify adult renal cysts based on computed tomography findings, the Bosniak classification has been extended to pediatric patients, with some adjustments made with the aim of accommodating magnetic resonance imaging (MRI) and ultrasonography (US). Bosniak IV lesions are rare in adolescents, indicating localized renal cell carcinoma and requiring surgical intervention. In contrast, Bosniak III lesions can be treated conservatively, although there is a lack of specific guidelines on their management. We present a case of a 14-year-old boy with a Bosniak III lesion, which was incidentally detected during the US evaluation of a left varicocele. After a 12-month follow-up, MRI revealed progression to a Bosniak IV cyst. Robot-assisted tumor enucleation was performed with selective artery clamping when the patient was 15. Histopathology showed tubulocystic renal cell carcinoma without adverse features. Immunocytochemistry supported a favorable prognosis of this rare tumor (<1% of renal tumor), thus obviating the need for adjuvant treatment. At the 18-month follow-up, no recurrence or distant metastasis were observed. This case highlights the importance of an aggressive treatment in persistent Bosniak III and Bosniak IV renal cysts in children and adolescents and the necessity to offer a nephron-sparing surgery.

Cystic Features in Renal Epithelial Neoplasms and Their Increasing Clinical and Pathologic Significance.

Most cystic renal tumors after resection (Boniak IIF to IV cysts) have an indolent course despite the significantly higher proportion of malignant [ie, renal cell carcinoma (RCC)] diagnosis. Most cystic renal tumors have clear cell histology that include cystic clear cell RCC and multilocular cystic renal neoplasm of low malignant potential (MCNLMP). There is growing evidence to suggest that MCNLMP, cystic clear cell RCC, and noncystic clear cell RCC form a cystic-to-solid biological spectrum with MCNLMP representing the most indolent form and with cystic clear cell RCC behaving better than noncystic (solid) clear cell RCC. Extensively (>75%) cystic clear cell RCC also has an excellent outcome similar to MCNLMP stressing the need to reevaluate the histologic criteria that separate these 2 cystic clear cell tumors. Other tumors with clear cells that can be extensively cystic such as the recently reclassified noncancerous clear cell papillary renal tumor and the newly described MED15::TFE3 RCC also have indolent course and may mimic MCNLMP. Cystic features occur also in renal tumors with nonclear cell histology including tumors capable of metastasis such as acquired cystic disease-associated, tubulocystic, fumarate hydratase-deficient, and eosinophilic solid and cystic RCCs. Cystic imaging presentation of some renal tumors such as papillary RCC can be attributed in part to pseudocystic necrosis and hemorrhage. It is important to know that tubulocystic RCC may have a lower Bosniak class presentation that overlaps with benign renal cysts (Bosniak I to IIF) that are managed conservatively. This review highlights the cystic renal tumors with clear cell and nonclear cell morphologies including some novel RCC subtypes that may have cystic features. The presence of cystic features and their extent may aid in the classification and prognostication of renal neoplasms underscoring its increasing importance in the pathologic diagnosis and reporting of renal neoplasia.

Fumarate Hydratase-Deficient Renal Cell Carcinoma With Predominant Tubulocystic Features Mimics Tubulocystic Renal Cell Carcinoma.

Fumarate hydratase (FH)-deficient renal cell carcinoma (RCC) rarely exhibits a predominant tubulocystic architecture with few other components. RCC with pure tubules and cysts lined by eosinophilic tumor cells with prominent nucleoli would raise the diagnosis of tubulocystic RCC. It is important to differentiate the 2 entities because they lead to different outcomes. To address the concern, a multicenter study was implemented to explore useful clinicopathologic features in differentiation between tubulocystic FH-deficient RCC and tubulocystic RCC. Clinical factors included age, sex, tumor size, and outcome. Morphologic factors included cell morphology, presence or absence of a nontubulocystic component, and stromal findings. Immunohistochemistry, fluorescence in situ hybridization, and next-generation sequencing were performed to explore the protein expression and molecular profiles of the 2 entities. We evaluated 6 patients with tubulocystic RCC and 10 patients with tubulocystic FH-deficient RCC. Tubulocystic RCC exhibited a small size (<4.0 cm, pT1a), low Ki-67 index (<5%), retained FH, and negative 2SC expression. Tubulocystic FH-deficient RCC had a relatively large size and a high Ki-67 index. Perinucleolar haloes, loss of FH, and 2SC positivity were always observed. Pure tubulocystic architecture was not observed in FH-deficient RCC, because focal nontubulocystic components can always be seen. We emphasized multiple sectioning to identify a nontubulocystic architecture to exclude tubulocystic RCC. Moreover, tumor size, FH/2SC staining, and the Ki-67 index can differentiate tubulocystic FH-deficient RCC from tubulocystic RCC. The diagnosis of tubulocystic RCC was not recommended in renal mass biopsy because of the limited tissues sampled.

A rare malignity observed in the appearance of angiomyolipoma; tubulocystic renal cell carcinoma after partial nephrectomy

Cystic neoplasms of the kidney are quite rare. Because they contain various differential diagnoses and their radiological features are not specific, their diagnosis is very difficult except for histopathological data. Usually, they can be confused radiologically with benign cysts of the kidney or angiomyolipoma. Radical or partial nephrectomy is the most commonly preferred curative treatment method when it shows features like rapidly growing or malignancy. Histopathological examination is required for definitive diagnosis. In this article, we aimed to present a rare case of tubulocystic renal cell carcinoma after partial nephrectomy in our clinic, who was followed up in another center for years with the pre-diagnosis of angiomyolipoma, in the light of the literature.

Thermoablative Treatment of De Novo Tumor in Kidney Allograft.

The overall cancer risk increases in transplant patients, including in kidney allografts. This study aimed to analyze the outcome of patients with kidney allograft malignant tumors who underwent percutaneous thermal ablation. We included 26 renal allograft tumors, including 7 clear-cell renal cell carcinoma (RCCs), 16 papillary RCCs, 1 clear-cell papillary RCC, and 2 tubulocystic RCCs, treated in 19 ablation sessions. Outcomes of thermal ablation therapy were assessed, including technical success, adverse events, local tumor progression, development of metastases, survival after thermal ablation, and changes in renal function. Success rate was achieved in all ablation sessions (primary success rate: 96%; secondary success rate: 100%). No adverse events were observed in grades 3, 4, or 5. The median follow-up period was of 34 mo (15-69 mo). Two patients died during follow-up from a cause independent of renal cancer. The median decrease in estimated glomerular filtration rate 1 y after procedure was -4 (interquartile range, -7 to 0) mL/min/1.73 m 2 . One patient returned to dialysis within the year of the procedure. Percutaneous thermal ablation shows convincing results for treating malignant renal graft tumors and should be a useful treatment option. The shorter hospitalization time, the advantage of avoiding a potentially challenging dissection of the transplant, and the excellent preservation of allograft function appear encouraging to extend this indication.

Renal Cell Carcinoma in the Background of Autosomal Dominant Polycystic Kidney Disease: Report of Two Cases and Review of Literature

AbstractPatients with autosomal dominant polycystic kidney disease (ADPKD), especially those with renal failure, carry a higher risk of developing renal cell carcinoma (RCC) compared to the general population. Genetic mutations associated with ADPKD are known but a direct link associated with RCC is still controversial. We discuss the clinical course of two such patients. The first patient was diagnosed with ADPKD at the age of 10 years with an unreported tubulocystic RCC focus on his renal biopsy that was picked up on review 16 years later when he presented with vertebral metastases determined to have originated from the RCC. He was doing well on multikinase inhibitors till 4 years of diagnosis with metastatic disease when he succumbed to progressive disease after 3 lines of systemic therapy. The second patient was diagnosed with ADPKD in middle age and papillary RCC 3 years later. Within 3 months of cancer diagnosis, there was progression to metastatic disease and rapid decline despite systemic therapies. We surmise that the diagnosis of RCC may be missed in ADPKD till the advanced stages. Patients with ADPKD should be monitored regularly with imaging and biopsy if needed. Histology may be varied but once diagnosed, systemic therapies may help disease control.

ТУБУЛОКИСТОЗНАЯ ПОЧЕЧНО-КЛЕТОЧНАЯ КАРЦИНОМА: КЛИНИЧЕСКОЕ НАБЛЮДЕНИЕ И ОБЗОР ЛИТЕРАТУРЫ

Tubulocystic renal cell carcinoma is a rare malignant epithelial tumor with unique morphological features. It usually demonstrates indolent behavior with low recurrence rate and low metastatic potential. Here we present literature review and a case report of 65 y.o. woman with tubulocystic renal cell carcinoma. Pitfalls of differential diagnostic with other cystic renal neoplasms are shown.

Carcinome à cellules rénales FH (fumarate hydratase)-déficient : à propos d’un cas

Fumarate hydratase deficient renal cell carcinoma (FH-RCC) is a rare malignant neoplasia caused by constitutive or somatic mutations in the FH gene whose diagnosis is primordial, requiring genetic counselling. Because of histological heterogeneity, such tumors have been in the past misclassified as "type 2papillary carcinoma", "tubulo-cystic renal cell carcinoma" or "high grade papillary carcinoma". We report here a case of FH deficient renal cell carcinoma (FH-RCC) in a 69years old patient. Through this observation, we precise the epidemiological and histological aspects and diagnosis criteria of this rare tumor.

A Rare and Unusual Renal Tumour: A Case Report

Introduction: Multilocular cystic renal cell neoplasm of low malignant potential is a rare histologically identified form of clear cell renal cell carcinoma included in 2016 Renal tumour classification of WHO. It is a low risk neoplasm with clear cells that have less expansile proliferation than other clear cell variants of renal cell carcinoma with no recurrence and metastasis with few exceptional cases. The incidence of multilocular cystic renal cell carcinoma is estimated to be 1-2 percent.6,12 Although the WHO classification of multilocular cystic renal cell carcinoma with low malignant potential was reported in 2004. The WHO 2016 categorization of renal cell tumour has been assessed as a special entity with no documented cases of metastasizing and recurrent tumour development.4 We present a rare clinical case of multilocular cystic renal cell neoplasm with low malignant potential, which should not be mistaken with other differential diagnoses such as cystic necrosis in conventional renal cell carcinoma, tubulo cystic renal cell carcinoma, multilocular cystic nephroma, clear cell variant of renal cell carcinoma and other benign multilocular renal tumors.

The Utility of Immunohistochemistry in Diagnosing Tubulocystic Renal Cell Carcinoma with Papillary Morphology

Tubulocystic renal cell carcinoma (TC-RCC) is a recently recognized, rare but distinct malignant entity. Pathologists have endeavored to completely define its histomorphologic, immunohistochemical and molecular features. Recounted is a case where the diagnosis of TC-RCC was confounded by presence of papillary morphology. Immunohistochemical expression of alpha-methyl acyl-CoA-racemase and vimentin with corresponding negativity for CK7 and CD10, following distinctive gross and microscopic findings, confirmed a diagnosis of TC-RCC. This report demonstrates the strategic value of performing immunohistochemistry studies to establish a diagnosis of TC-RCC especially when unusual histologic features are encountered. Immunohistochemistry continues to be the most practical approach to diagnosis as molecular testing methods, such as next generation sequencing, remain unfeasible in the local setting. Cautious prognostication is required as accounts of recurrence and metastasis continue to emerge.

New developments in existing WHO entities and evolving molecular concepts: The Genitourinary Pathology Society (GUPS) update on renal neoplasia.

The Genitourinary Pathology Society (GUPS) reviewed recent advances in renal neoplasia, particularly post-2016 World Health Organization (WHO) classification, to provide an update on existing entities, including diagnostic criteria, molecular correlates, and updated nomenclature. Key prognostic features for clear cell renal cell carcinoma (RCC) remain WHO/ISUP grade, AJCC/pTNM stage, coagulative necrosis, and rhabdoid and sarcomatoid differentiation. Accrual of subclonal genetic alterations in clear cell RCC including SETD2, PBRM1, BAP1, loss of chromosome 14q and 9p are associated with variable prognosis, patterns of metastasis, and vulnerability to therapies. Recent National Comprehensive Cancer Network (NCCN) guidelines increasingly adopt immunotherapeutic agents in advanced RCC, including RCC with rhabdoid and sarcomatoid changes. Papillary RCC subtyping is no longer recommended, as WHO/ISUP grade and tumor architecture better predict outcome. New papillary RCC variants/patterns include biphasic, solid, Warthin-like, and papillary renal neoplasm with reverse polarity. For tumors with 'borderline' features between oncocytoma and chromophobe RCC, a term "oncocytic renal neoplasm of low malignant potential, not further classified" is proposed. Clear cell papillary RCC may warrant reclassification as a tumor of low malignant potential. Tubulocystic RCC should only be diagnosed when morphologically pure. MiTF family translocation RCCs exhibit varied morphologic patterns and fusion partners. TFEB-amplified RCC occurs in older patients and is associated with more aggressive behavior. Acquired cystic disease (ACD) RCC-like cysts are likely precursors of ACD-RCC. The diagnosis of renal medullary carcinoma requires a negative SMARCB1 (INI-1) expression and sickle cell trait/disease. Mucinous tubular and spindle cell carcinoma (MTSCC) can be distinguished from papillary RCC with overlapping morphology by losses of chromosomes 1, 4, 6, 8, 9, 13, 14, 15, and 22. MTSCC with adverse histologic features shows frequent CDKN2A/2B (9p) deletions. BRAF mutations unify the metanephric family of tumors. The term "fumarate hydratase deficient RCC" ("FH-deficient RCC") is preferred over "hereditary leiomyomatosis and RCC syndrome-associated RCC". A low threshold for FH, 2SC, and SDHB immunohistochemistry is recommended in difficult to classify RCCs, particularly those with eosinophilic morphology, occurring in younger patients. Current evidence does not support existence of a unique tumor subtype occurring after chemotherapy/radiation in early childhood.

Early Occurrence of Two Distinct Histological Types of Renal Cell Carcinoma in End-Stage Renal Disease Patient on Haemodialysis

Background: The occurrence of renal cell carcinoma is a well-known complication in end-stage renal disease on haemodialysis. Various histological types of renal cell carcinomas are observed in these patients and varies with the duration of haemodialysis. Though the synchronous association of two renal cell carcinomas in the patients are known, the existence of such dual renal tumours in the patient on dialysis is extremely rare and unheard in the English literature. Moreover, tubulocystic renal cell carcinoma is rarely reported in this setting.&#x0D; Case report and Discussion: We describe an unusual early synchronous occurrence of two tumours with distinct histology i.e. Papillary renal cell carcinoma (PRCC, type I) and Tubulocystic renal cell carcinoma (TC-RCC) in a patient with end-stage renal disease on haemodialysis for a duration less than a year. Though exact etiological factors peculiar to the occurrence of these tumours are not known, increased oxidative stress occurring in end-stage renal disease patient on haemodialysis might play an important role in carcinogenesis.&#x0D; Conclusion: Renal cell carcinoma with more than one histological type may occur exceedingly early without any symptoms in these patients. Radiologists and urologists should be aware of it for early diagnosis and prompt treatment. Pathologists should also be more cautious while grossing and pick the sub-centimetric primary or secondary tumours that may have an impact on patient survival.

Tubulocystic renal cell carcinoma with poorly differentiated foci and loss of fumarate hydratase: A case report

Tubulocystic renal cell carcinoma (RCC) is one of the newly recognized subtypes of RCC. It has a unique cystic morphology and indolent behavior. During the last decade, few studies have been published describing tubulocystic RCC with poorly differentiated foci. A subset of these cases are associated with loss of fumarate hydratase which is a characteristic feature of hereditary leiomyomatosis and renal cell carcinoma-associated RCC. However, these two entities represent two distinct subtypes of RCC in the recent WHO Classification of kidney tumors. Herein, we are describing a rare case of tubulocystic renal cell carcinoma with poorly differentiated foci and loss of fumarate hydratase.

Tubulocystic renal cell carcinoma: a distinct clinicopathologic entity with a characteristic genomic profile

Tubulocystic renal cell carcinoma, a unique tumor, was recently included as a new entity in the World Health Organization classification of renal tumors. It has variably been reported to be related to other renal cell carcinomas, including papillary renal cell carcinoma, fumarate hydratase-deficient carcinoma, and others, likely because many such carcinomas may show variable amounts of tubulocystic architecture. The published data characterizing the molecular features of these tumors are inconsistent. We studied nine “pure” tubulocystic renal cell carcinomas, as defined by International Society of Urologic Pathologists (ISUP) and World Health Organization (WHO), by targeted next-generation sequencing, and fluorescence in situ hybridization for X and Y chromosomes, to investigate if these show any unique characteristics or any overlap with known mutational/molecular profiles or copy number alterations in other subtypes of renal cell carcinoma. All nine tubulocystic carcinomas demonstrated combined losses at chromosome 9 and gains at chromosome 17, as well as, loss of chromosome Y (in 5/5). None of the tumors showed mutational profiles characteristic of other renal neoplasms, including those seen in fumarate hydratase-deficient renal cell carcinoma. Recurrent mutations in chromatin-modifying genes, KMT2C and KDM5C, were detected in two of nine tumors. Thus, tubulocystic renal cell carcinoma, if defined strictly, at the clinical and pathologic level, demonstrates genomic features distinct from other subtypes of renal cell carcinoma. These findings support the contention that tubulocystic renal cell carcinoma should be diagnosed only using strict morphological criteria and only when presenting in a “pure” form; presence of variable papillary, poorly differentiated, or other architectural patterns most likely do not belong to the category of tubulocystic renal cell carcinoma.

Tubulocystic renal cell carcinoma – a case report

Tubulocystic renal cell carcinoma – a case report

Papillary Adenomas and Other Small Epithelial Tumors in the Kidney: An Autopsy Study.

The aim of this work was to study small neoplasms of the epithelium of the renal tubules; kidneys from 402 unselected autopsies were sectioned at 1 to 2 mm intervals. All lesions were examined histologically. A total of 232 papillary adenomas were found in 76 patients (19%), ranging from 1 to 35 adenomas/patient (mean: 3, median: 2). Patients with papillary adenomas were older (range: 27 to 90 y) (P<0.0001), more commonly smokers (P=0.01), and more frequently had glomerulosclerosis (P=0.0001) than those without. Papillary adenomas ranged in size from 0.5 to 5 mm (mean: 1.4, median: 1) and were morphologically classified in 4 subtypes. Type A adenomas consisting of papillae and/or tubules covered by cells with scant cytoplasm, often with psammoma bodies, were the most common (149 adenomas). In 30, the papillae were broad with lymphocytes in the cores (type B). Thirty were more cystic lined by columnar cells and contained macrophages and psammoma bodies (type C). Sixteen were composed of large eosinophilic cells with pseudostratified nuclei, occasionally near the apical membrane (type D). Four were unclassified. Mixtures of types were observed only once (3 adenomas in one patient, each composed of a mixture of types B and D). Other epithelial lesions included 31 adrenal rests, 5 oncocytomas, 2 clear cell papillary renal cell carcinomas, 2 tubulocystic renal cell carcinomas, and 2 acquired cystic disease-associated renal cell carcinomas. In conclusion, papillary adenoma was the most common small epithelial neoplasm. The first type of papillary adenoma (type A) closely resembles papillary renal cell carcinoma, type 1, and the fourth (type D) resembles type 2. No other type of renal cell neoplasm was found (including clear cell renal cell carcinoma and chromophobe renal cell carcinoma). No nephrogenic rest was found. Angiomyolipomas and renomedullary interstitial cell tumors were found, and these findings have been reported in earlier papers.

Re: Tubulocystic Renal Cell Carcinoma: A Review of Literature Focused on Radiological Findings for Differential Diagnosis.

Re: Tubulocystic Renal Cell Carcinoma: A Review of Literature Focused on Radiological Findings for Differential Diagnosis.

Computed Tomography Perfusion Measurements in Renal Lesions Obtained by Bayesian Estimation, Advanced Singular-Value Decomposition Deconvolution, Maximum Slope, and Patlak Models: Intermodel Agreement and Diagnostic Accuracy of Tumor Classification.

The aims of this study were to evaluate the agreement of computed tomography (CT)-perfusion parameter values of the normal renal cortex and various renal tumors, which were obtained by different mathematical models, and to evaluate their diagnostic accuracy. Perfusion imaging was performed prospectively in 35 patients to analyze 144 regions of interest of the normal renal cortex and 144 regions of interest of renal tumors, including 21 clear-cell renal cell carcinomas (RCC), 6 papillary RCCs, 5 oncocytomas, 1 chromophobe RCC, 1 angiomyolipoma with minimal fat, and 1 tubulocystic RCC. Identical source data were postprocessed and analyzed on 2 commercial software applications with the following implemented mathematical models: maximum slope, Patlak plot, standard singular-value decomposition (SVD), block-circulant SVD, oscillation-limited block-circulant SVD, and Bayesian estimation technique. Results for blood flow (BF), blood volume (BV), and mean transit time (MTT) were recorded. Agreement and correlation between pairs of models and perfusion parameters were assessed. Diagnostic accuracy was evaluated by receiver operating characteristic (ROC) analysis. Significant differences and poor agreement of BF, BV, and MTT values were noted for most of model comparisons in both the normal renal cortex and different renal tumors. The correlations between most model pairs and perfusion parameters ranged between good and perfect (Spearman ρ = 0.79-1.00), except for BV values obtained by Patlak method (ρ = 0.61-0.72). All mathematical models computed BF and BV values, which differed significantly between clear cell RCCs, papillary RCCs, and oncocytomas, which introduces them as useful diagnostic tests to differentiate between different histologic subgroups (areas under ROC curve, 0.83-0.99). The diagnostic accuracy to discriminate between clear-cell RCCs and the renal cortex was the lowest based on the Patlak plot model (area under ROC curve, 0.76); BF and BV values obtained by other algorithms did not differ significantly in their diagnostic accuracy. Quantitative perfusion parameters obtained from different mathematical models cannot be used interchangeably. Based on BF and BV estimates, all models are a useful tool in the differential diagnosis of kidney tumors, with the Patlak plot model yielding a significantly lower diagnostic accuracy.

Clear Cell Renal Cell Carcinoma

Renal cell carcinoma, clear cell type, forming a mass in the lower and mid portion of the kidney. Which is the most common subtype of renal cell carcinoma?a.Clear cellb.Papillaryc.Chromophobed.Unclassified Answer: a. Clear cell Clear cell renal cell carcinoma is the most common subtype of renal cell carcinoma (RCC), comprising 65% to 70% of RCC.1Moch H. Humphrey P.A. Ulbright T.M. Reuter V.E. WHO Classification of Tumours of the Urinary System and Male Genital Organs.4th ed. IARC Press, Lyon, France2016Google Scholar Papillary RCC comprises 15% to 20% and chromophobe RCC comprises 5% to 7% of RCC.1Moch H. Humphrey P.A. Ulbright T.M. Reuter V.E. WHO Classification of Tumours of the Urinary System and Male Genital Organs.4th ed. IARC Press, Lyon, France2016Google Scholar Other renal cell tumor subtypes include collecting duct carcinoma, renal medullary carcinoma, mucinous tubular and spindle cell carcinoma, RCC unclassified, papillary adenoma, and oncocytoma. Recently described renal cell tumors include multilocular cystic renal neoplasm of low malignant potential, MiT family translocation RCC, tubulocystic RCC, acquired cystic disease–associated RCC, clear cell papillary RCC, succinate dehydrogenase–deficient RCC, and hereditary leiomyomatosis and RCC-associated RCC. Additional emerging/provisional entities include oncocytic RCC occurring after neuroblastoma, thyroid-like follicular RCC, ALK rearrangement–associated RCC, and RCC with (angio)leiomyomatous stroma.1Moch H. Humphrey P.A. Ulbright T.M. Reuter V.E. WHO Classification of Tumours of the Urinary System and Male Genital Organs.4th ed. IARC Press, Lyon, France2016Google Scholar Although complex, the classification of renal cell tumors is important diagnostically and prognostically.

Tubulocystic renal cell carcinoma: a review of literature focused on radiological findings for differential diagnosis.

Tubulocystic renal cell carcinoma (TC-RCC) has been classified as an independent subtype according to the 2016 World Health Organization (WHO) classification. It is a rare subtype that predominantly affects men. Although few in number, radiological imaging reports have suggested that TC-RCC is characterized by multilocular cystic lesions, which are categorized as the Bosniak classification II-IV, with signature pathological characteristics comprising numerous small cysts or a tubular structure. The Bosniak classification system facilitates patient management; however, the differentiation of cystic tumors exhibiting similar imaging findings remains impossible; in fact, the differentiation of multilocular cystic RCC, adult cystic nephroma, and mixed epithelial and stromal tumor remains challenging. This review aims to discuss TC-RCC with a focus on implications of radiological findings in the differential diagnosis of TC-RCC.