Cases Of Multilocular Cystic Renal Cell Carcinoma Research Articles

Multilocular cystic renal cell carcinoma: A case report and review of the literature

Multilocular cystic renal cell carcinoma (MCRCC), which exhibits low-stage and low-grade characteristics, is a special type of RCC. MCRCC is extremely rare and generally develops at ages >50 years. We herein report a case of MCRCC in a 28-year-old man, which, to the best of our knowledge, is the youngest case reported worldwide to date. The patient presented with irritative bladder symptoms for 1 year. Dynamic enhanced computed tomography (CT) imaging revealed a mass with inhomogeneous enhancement in the left kidney, with a rich blood supply. B-ultrasonography also revealed a renal protruding mass. As the mass was highly suspicious to be a malignant tumor, laparoscopic radical nephrectomy was performed and MCRCC was definitively diagnosed by pathological examination. The patient has been regularly followed up for 6 months, without complications or disease recurrence.

Multilocular cystic renal cell carcinoma: A rare case report with review of literature

Multilocular cystic renal cell carcinoma (MCRCC) is a rare adult cystic tumor of the kidney and is considered as a distinct type of clear cell renal cell carcinoma. Most patients are asymptomatic and they are detected incidentally. MCRCC is consistently low nuclear grade and has an excellent prognosis. Concern still persists regarding its true malignant potential. Although surgical resection is curative, lymph node metastasis has been reported recently. There are very few reports on large series of cases and limited available follow-up. We report a case of MCRCC in a 60-year-old male patient who presented with abdominal distention. Careful follow-up is essential in these patients for better prognosis.

Multilocular Cystic Renal Cell Neoplasm of Low Malignant Potential: A Series of 76 Cases

IntroductionMultilocular cystic clear cell renal cell neoplasm of low malignant potential or multilocular cystic renal cell carcinoma (MCRCC) is a rare distinct subtype of clear cell renal cell carcinoma (RCC). No large series of cases have been reported to date. The present study aimed to characterize the clinical and pathologic features of MCRCC. Patients and MethodsFrom January 2006 to December 2014, 76 cases were identified as MCRCC among 4345 patients with RCC at our institution. Their clinical and characteristics, surgical management, pathologic features, and outcomes were retrospectively reviewed. ResultsThe incidence of MCRCC in our patients with RCC was 1.7%. The mean age at diagnosis was 46.7 ± 10.5 years (range, 18 to 80 years). Most cases showed no symptoms. Nuclear grade was unrelated to the tumor size (P = .112) and TNM stage (P = .451). Of these 76 patients, 66 (86.8%) were followed up for a median of 52 months, and no tumor recurrence or metastasis was found. ConclusionThe incidence of MCRCC in patients with RCC is low. The nuclear grade of MCRCC cases was unrelated to the tumor size and TNM stage, suggesting that the current stage criteria might not suitable for this lesion. Patients with MCRCC have an excellent prognosis; thus, the follow-up interval after surgery can be longer to minimize unnecessary examinations.

CT differential diagnosis of cystic nephroma and multilocular cystic renal cell carcinoma

To study the CT findings of cystic nephroma (CN) and multilocular cystic renal cell carcinoma (MCRCC) and to improve the accuracy of preoperative diagnosis of these two diseases. The CT findings of nine CN cases and 19 MCRCC cases confirmed by pathology were blindly reviewed and compared with their pathological results. Fisher's exact test and independent-samples T test were applied to statistically analyze some of the CT features of the CN and MCRCC lesions. The thickness of cystic walls and partitions in the nine CN cases ranged from 0.5 to 5 mm. Cystic walls and partitions were slightly thicker in some parts without visible mural nodules. Varying amounts of solid tissue could be found in all the 19 MCRCC tumors, and the cystic walls and partitions were found partially thickened ranging from 3 mm to 13 mm. Eight cases were with mural nodules (nodule diameter: 4.5-16 mm). Nine cases of CN tumors were lobulated and 7 protruded into the renal sinus. Three out of the 19 MCRCC presented shallow lobulation, and 7 tumors protruded into the renal sinus. The CT contrast-enhancement scanning displayed moderate delayed enhancement in the cystic walls and partitions in 8 cases. The enhanced scanning revealed that all the nine cases showed enhancement of the cystic walls and partitions, while 8 cases of them had mild to moderate delayed enhancement. The cystic walls, partitions and nodules were enhanced in 19 MRCC cases, among them 17 cases displayed obvious enhancement in the cortical phase. Among the differences of CT findings between MC and MRCC, the shallow lobulation, protruding into the renal sinus, mural nodules, cystic wall and partition thickness, and net growth in the cortical and nephrographic phase were statistically significantly different (P<0.05 for all). CT scan can provide significant evidence for CN and MCRCC diagnosis. CN cases usually present relatively thin and even cystic walls and partitions without mural nodules and with shallow lobulation and protruding into the renal sinus. The enhancement is mild to moderate, dynamic and delayed, while the opposite CT findings may indicate a higher possibility of MCRCC.

Multilocular cystic renal cell carcinoma – Case report of a low grade tumor

Multilocular cystic renal cell carcinoma (MCRCC) is a rare variant of renal cell carcinoma with good prognosis and well defined diagnostic criterion. Here we report a case of MCRCC and discuss its morphological, immunohistochemical, genetic and prognostic characteristics along with important differential diagnosis.

Multilocular cystic renal cell carcinoma: A rare case report

Multilocular cystic renal cell carcinoma (MCRCC) is an uncommon low grade RCC with unique morphologic features. MCRCC also known as multilocular clear cell RCC is a rare cystic tumor of the kidney with an excellent outcome. MCRCC has been recognized as a separate subtype of RCC in the 2004 World Health Organization (WHO) classification of adult renal tumors. MCRCC is diagnosed on the basis of strict histological criteria according to 2004 WHO classification. It is usually included in the group of tumors of undetermined malignant potential with low nuclear grade. We present a case of MCRCC in a 43-year-old female patient who presented with pain in left lumbar region and hematuria. Left-sided radical nephrectomy was performed, and on histopathologic examination it was diagnosed as MCRCC with Fuhrman nuclear grade 1. Immunohistochemistry with epithelial membrane antigen and vimentin confirmed the diagnosis.

Multilocular cystic renal cell carcinoma a diagnostic dilemma: A case report in a 30-year-old woman

Multilocular cystic renal cell carcinoma (MCRCC), also known as multilocular clear cell renal cell carcinoma (RCC), is a rare cystic tumor of the kidney with an excellent outcome. It occurs in about 3.1-6% of the conventional RCC. It is usually included in the group of tumors of undetermined malignant potential with low nuclear grade. We present a case of MCRCC in a 30-year-old female patient presenting incidentally as an apparently benign-looking multicystic space occupying lesion in the upper pole of right kidney. Right-sided simple nephrectomy was performed, and on histopathologic examination it was found to be MCRCC, stage 1 with Fuhrman nuclear grade 1. Immunohistochemistry with epithelial membrane antigen and vimentin confirmed the diagnosis.

Multilocular Cystic Renal Cell Carcinoma

Multilocular cystic renal cell carcinoma (RCC) is an uncommon renal neoplasm composed of thin fibrous septa lining multiple cystic spaces and associated with an excellent prognosis. Clear cells with generally low-grade nuclear features line the cystic spaces and may be present within the fibrous septa, although solid mass-forming areas are by definition absent. Despite the excellent prognosis, molecular-genetic alterations are similar to those of clear cell RCC. Immunohistochemical staining characteristics, however, have not been well elucidated. We studied 24 cases of multilocular cystic RCC, classified according to the 2004 World Health Organization System. Immunohistochemical analysis was performed using an automated immunostainer for CD10, cytokeratin 7 (CK7), α-methylacyl-CoA-racemase, epithelial membrane antigen (EMA), cytokeratin CAM 5.2, carbonic anhydrase IX (CA-IX), estrogen/progesterone receptors, smooth muscle actin, PAX-2, and vimentin. Twenty-four cases of grade 1 to 2 clear cell RCC were stained for comparison. Multilocular cystic RCC and control cases of clear cell RCC showed the following results, respectively: CD10 (63%, 96%), CK7 (92%, 38%), α-methylacyl-CoA-racemase (21%, 67%), vimentin (58%, 33%), estrogen receptor (8%, 8%), CAM 5.2 (100%, 96%), EMA, CA-IX, PAX-2 (all 100%), and progesterone receptor (0%). Smooth muscle actin highlighted myofibroblastic cells within the septa of multilocular cystic RCC and the fine capillary vascular network of clear cell RCC. In summary, multilocular cystic RCC showed expression of common clear cell RCC markers CA-IX, EMA, and PAX-2, supporting the hypothesis that multilocular cystic RCC is a subtype of clear cell RCC. In contrast to clear cell RCC, tumors less frequently expressed CD10 (63% and often focal vs. 96% and diffuse) and more frequently expressed CK7 (92%), often diffusely (63%). Coexpression of CA-IX and CK7 represents a point of overlap with the recently described clear cell papillary RCC, which also may show a prominent cystic architecture. However, the latter lacks mutation of the VHL gene and deletion of chromosome 3p by molecular methodologies.

Multilocular Cystic Renal Cell Carcinoma: Comparison of Imaging and Pathologic Findings

The purpose of this study was to retrospectively correlate the imaging and pathologic features of multilocular cystic renal cell carcinoma (RCC), a low-grade neoplasm that has an excellent prognosis. Institutional databases were searched for the period between 2001 and 2010 to identify cases of resected renal tumors that had been evaluated with CT or MRI and been analyzed by a uropathologist to confirm the histologic diagnosis of multilocular cystic RCC. The images (nine CT, 14 MRI) were reviewed, and a Bosniak cyst category was assigned. Of 23 confirmed cases of multilocular cystic RCC, imaging revealed seven lesions were Bosniak category IIF, 13 were category III, and three were category IV. Pathologic examination of the category IIF lesions revealed 99% fluid, 0.001-1% clear cells lining the septum, and 0% fibrosis. The category III lesions were 98-99% fluid, 1-2% clear cells, and 0% fibrosis. The category IV lesions were 20-40% fluid, 1-5% clear cells, and 60-80% fibrosis. The patient demographics were similar across groups. Clinical follow-up showed no evidence of recurrent or metastatic disease. Multilocular cystic RCC is a rare cystic lesion of the kidney that is low risk to the patient and benign in behavior. It has a variable imaging pattern, the Bosniak category ranging from IIF to IV. As multilocular cystic RCC lesions increase in complexity on images (higher Bosniak category), there is a corresponding increase in the volume of malignant cells lining the tumor and an increase in the presence of vascularized fibrous tissue. Regardless of the imaging appearance, the behavior of these tumors was benign in this study. Clinicians and radiologists should be aware that when this carcinoma is reported to occur, the patient has an excellent prognosis.

A clinical analysis of multilocular cystic renal cell carcinoma and its correlation with mutation of the von Hippel-Lindau gene

Objective To discuss the diagnosis and surgical management of multilocular cystic renal cell carcinoma (MCRCC) and to evaluate the gene function of the mutation of von Hippel-Lindau (VHL) gene in MCRCC. Methods Seventeen MCRCC cases (11 men and 6 women) out of 512 cases of renal cell carcinoma from 2000 to 2010 were retrospectively analyzed. The mean age of the 17 patients was 46 years (37-61 years). Ultrasonography and CT were available in all 17 cases, and 1 case was misdiagnosed as parapelvic renal cyst. The mutation of VHL gene was detected by PCR in the specimens of can-cerous tissue and adjacent normal tissue from 11 cases of MCRCC. Results Three of 17 cases underwent nephron sparing surgery, the others underwent radical nephrectomy. One case underwent unroofing of parapelvic renal cyst, but the rapid frozen pathology of the cyst wall showed renal cell carcinoma of clear type. As a result, radical nephrectomy was eventually performed. All 17 cases were confirmed as MCRCC by eva-luating pathological characteristics, such as the cyst wall lined by single or several layers of clear tumor cells and the nuclei which were small and anachromasis. Clinical stages of all cases were T1N0M0, in which there were 14 cases with pathological T1G1 and 3 cases with pathological T1G2. All patients underwent a follow-up of 9 to 36 months (mean, 12 months) without recurrence or metastasis. Mutation of VHL gene was detected in 7 of 11 cases (64%), but all adjacent normal tissues were negative. Conclusions As a rare subtype of renal cell carcinoma, MCRCC is difficult to diagnose. CT is an essential measure in diagnosis of MCRCC preoperatively. Because of the good prognosis of reported cases, nephron sparing surgery for the treatment of MCRCC is recommended. VHL gene mutations may play an important role in the carcinogenesis of MCRCC. Key words: Kidney diseases, cystic; Carcinoma, renal cell; Hippel-Lindau disease; Genes; Mutation

Multilocular Cystic Renal Cell Carcinoma: A report of two cases with a rare feature

Multilocular cystic renal cell carcinoma (MCRCC) is a relatively are cystic tumor of the kidney. Because of its distinct characteristics in prognosis and natural history, MCRCC was recognized as a separate subtype of renal cell carcinoma in the 2004 WHO classification of adult kidney tumors. 1 We report two cases of MCRCC, one from antemortem surgical specimen and the other from the post mortem (autopsy) which was an incidental finding of smallest size which is a rare occurrence and probably the first reported case.

Role of CD2-associated protein and nephrin expression in multilocular cystic renal cell carcinoma

To investigate the role of CD2-associated protein (CD2AP) and nephrin expression in the multilocular cystic renal cell carcinoma (MCRCC) METHODS: Seventy four specimens of renal lesions, including 29 cases of MCRCC, 32 case of clear cell renal cell carcinoma (CCRCC), and 13 cases of simple renal cyst (SRC), were collected during operation. Immunohistochemistry was used to detect the CD2AP expression. Five wax lumps from each of the 3 groups of specimen, totally 15, were randomly selected to undergo re-sectioning and immunohistochemistry so as to compare the levels of staining intensity and location of expression of CD2AP and nephrin in the same sections. The expression of CD2AP in the MCRCC specimens was stronger than that in the CCRCC that was in turn stronger than that than that in the simple renal cyst (SRC) specimens. The epithelium lining the cysts in MCRCC was strongly positive in CD2AP compared to other parts. CCRCC showed a weak expression of CD2AP, while CD2AP was negative in the SRC specimens. The staining intensity and location of expression were completely identical for nephrin and CD2AP among the three types of tissue specimens. The CD2AP expression is much stronger in MCRCC than in CCRCC and SRC. CD2AP may be used as a biomarker in differential diagnosis of MCRCC, CCRCC, and SRC. The identical expression of CD2AP and nephrin in MCRCC and their specific expression in podocytes are important hints for the mechanism of MCRCC.

Clinicopathologic features and molecular genetic analysis of multilocular cystic renal cell carcinoma

To assess the clinicopathological features and molecular genetic changes of multilocular cystic renal cell carcinoma (MCRCC). All the data reviewed were from the files of pathology department of Changhai hospital collected from 1990 to 2006. In totally 706 cases of renal cell carcinoma studied, there were 21 MCRCC cases identified. The clinical and pathological features were assessed, immunohistochemical staining was performed, and loss of heterozygosity (LOH) and microsatellite instability (MSI) were assessed using four microsatellite markers on chromosomes 3, 9 and 14. Of the 21 patients, the age ranged from 34 to 72 years (mean 50 years), 19 were male and two female. Tumors were found incidentally in 18 patients during physical examination, three patients had anemia or microhematuria. Among the 21 patients, 10 tumors were in the left kidney and 11 in the right. Eighteen patients were stage T1, two stage T2, and one stage T3 with perinephric tissue involvement. Follow up information was available in 20 patients, all showed no evidence of tumor recurrence or metastasis. Grossly, the tumor size ranged from 0.3 cm to 10.0 cm in the greatest dimension, consisting of multilocular cysts with variable sizes which contained light yellow, colloid or hemorrhagic fluid. The septae varied in thickness (ranged 0.1 cm to 0.5 cm, mean 0.2 cm). Microscopically the cysts were lined by single to multilayered epithelial cells with clear or lightly eosinophilic cytoplasm. There were clusters of clear cells seen in the septae stroma. Sixteen tumors were of Fuhrman grade 1, and five were of Fuhrman grade 2. Immunohistochemically, the clear cells were positive for vimentin, ABC, CAM5.2 and EMA. Six samples were positive for CD10, and 16 were positive for NSE. Among 21 patients, PCR amplification was successful in 11 patients. Microsatellite alterations were found in five patients. LOH was observed in 3 of 11 MCRCC (27%), two were at D3S1560 locus, and one at D14S617 locus. MSI frequency was identified in 2 of 11 MCRCC (18%), locating at D9S168 or D14S617 locus, respectively. MCRCC is an uncommon tumor of kidney, constituting 2.9% of all RCC enrolled into the study. It has distinctive clinical and pathological characteristics with an excellent outcome. Results indicated that MCRCC is a rare entity with low malignant potential.

Multilocular Cystic Renal Cell Carcinoma

The 2004 World Health Organization (WHO) classification of kidney tumors recognizes multilocular cystic renal cell carcinoma (MCRCC) as a rare variant of clear cell renal cell carcinoma with a good prognosis. Available information on its clinical significance is limited. The study cohort included 45 MCRCC cases classified according to 2004 WHO criteria obtained through a multi-institutional international search. Most patients had unilateral MCRCC with no side predominance that was found incidentally; 62% were men, but women had tumors at an earlier age ( P = .385). MCRCC occurred slightly more often in men than in women (1.7:1). At diagnosis, 82% of patients had stage T1 and 16%, stage T2; 1 patient had stage T3. The Fuhrman grade was 1 (62%) or 2 (38%), with smaller tumors (≤4 cm) most likely Fuhrman grade 1 ( P = .911). All 45 patients were alive with no evidence of disease at mean follow-up of 66.1 months, confirming an extremely good prognosis after surgery and a 5-year disease-specific survival rate of 100%. To rename this tumor as multilocular cystic renal cell neoplasm of low malignant potential might help urologists approach the patients conservatively.

Multilocular Cystic Renal Cell Carcinoma : A Report of 45 Cases of a Kidney Tumor of Low Malignant Potential

The 2004 World Health Organization (WHO) classification of kidney tumors recognizes multilocular cystic renal cell carcinoma (MCRCC) as a rare variant of clear cell renal cell carcinoma with a good prognosis. Available information on its clinical significance is limited. The study cohort included 45 MCRCC cases classified according to 2004 WHO criteria obtained through a multi-institutional international search. Most patients had unilateral MCRCC with no side predominance that was found incidentally; 62% were men, but women had tumors at an earlier age (P = .385). MCRCC occurred slightly more often in men than in women (1.7:1). At diagnosis, 82% of patients had stage T1 and 16%, stage T2; 1 patient had stage T3. The Fuhrman grade was 1 (62%) or 2 (38%), with smaller tumors (<or=4 cm) most likely Fuhrman grade 1 (P = .911). All 45 patients were alive with no evidence of disease at mean follow-up of 66.1 months, confirming an extremely good prognosis after surgery and a 5-year disease-specific survival rate of 100%. To rename this tumor as multilocular cystic renal cell neoplasm of low malignant potential might help urologists approach the patients conservatively.

Diagnosis and treatment of multilocular cystic renal cell carcinoma: a study of 22 cases

To summarize the diagnosis and treatment of multilocular cystic renal cell carcinoma (MCRCC), a special subtype of renal cell carcinoma. The clinical data of 22 cases of MCRCC diagnosed and treated from January 1998 to December 2002 in the First Hospital of Peking University were analyzed retrospectively. 482 patients with renal cell carcinomas were hospitalized during that period with a male-to-female ratio of 2.09:1. Out of the 482 cases 22 cases (4.56%) were classified as MCRCC with a male-to-female ratio of 2.67:1, not significantly different from that of the general group of renal cell carcinoma patients (P > 0.05). The mean age of the patients of MCRCC was 46.6 years, significantly lower than that of the patients with other types of renal cell carcinomas (57.83 years, P< 0.01). Eighteen cases were diagnosed correctly as renal carcinomas before operation. Six patients (27%) were at the stage pT(1)N(0)M(0), 15 (68%) at the stage pT(2)N(0)M(0), and 1 (5%) at the stage pT(3)bN(0)M(0). The classification according to the tumor nuclear grading system showed 5 carcinomas (23%) of tumor nuclear grade 1, 17(77%) are of tumor nuclear grade 2. Eighteen patients underwent radical renal nephrectomy and 4 underwent partial nephrectomy. The preoperative diagnosis of MCRCC, difficult to be differentiated from other cystic renal diseases, mainly depends on imaging studies. Intraoperative frozen-section or nephron sparing surgery will benefit the patients if the preoperative diagnosis is unclear, especially for the patients of category 3 or 4 of Bosniak's system.

Multilocular cystic renal cell carcinoma: a clinicopathological, immuno- and lectin histochemical study of nine cases.

Multilocular cystic renal cell carcinoma (MCRCC) is an uncommon variant of renal neoplasm and its histogenesis is unclear. The aim of this study was to use immuno- and lectin histochemistry to delineate histochemical patterns which might indicate the histogenetic origin of MCRCC from a particular part or parts of the nephron. We present our experience with nine cases of MCRCC. Fifteen cases of renal cell carcinoma with cystic degeneration (RCC-CD) were selected for comparison with MCRCC. We carried out clinicopathological and immunohistochemical examinations of the MCRCC cases. Clinically, the prognosis of the patients was quite good, in that all nine patients are alive and without recurrence at the time of this report. The MCRCCs reacted strongly in a higher proportion of cases with the distal nephron markers, such as peanut agglutinin (PNA, 88.9%) and MUC1-core antibody (MUC1, 100%), but none reacted preferentially with proximal nephron markers such as vimentin, Leu M1 and Lotus tetragonolobus (LTA). The RCC-CD tumours reacted with vimentin (40%), Leu M1 (66.7%) and LTA (86.7%). Except for two cases, the RCC-CD tumours did not react with PNA or MUC1 core antibody. These results illustrate the different patterns of expression of MCRCC and RCC-CD and suggest that MCRCC originates from the distal nephron. Therefore, MCRCC should be differentiated from other types of renal cell carcinoma on the basis of the histogenesis of the tumour and the clinicopathological findings.

Multilocular cystic renal cell carcinoma: a series of 12 cases and review of the literature

Objectives. To assess the presentation and diagnosis of, and treatment options for, multilocular cystic renal cell carcinoma (MCRCC). MCRCC is a rare entity and the true incidence and biologic behavior of MCRCC are not well known. Methods. We reviewed the pathology files at the Queen Elizabeth II Health Sciences Centre for a 9-year period (1991 to 2000). All cases of RCC with a cystic component were identified. The clinical features, radiologic features, surgical procedure, pathologic features, and follow-up outcomes of the MCRCC cases were studied. Results. We identified 12 cases of MCRCC comprising 3.1% of the RCC cases. The tumor was incidentally found in 67% of the cases. Ultrasonography was found to be a useful screening test, but computed tomography is the imaging study of choice to identify malignant features. Magnetic resonance imaging can be used in equivocal cases. For treatment, radical nephrectomy was done in 58% and partial nephrectomy in 33%. The mean tumor size was 3.4 cm. The mean solid component of the tumor was 9.5%. The mean tumor grade was 1.1. None of the 10 patients (83%) available for follow-up showed any evidence of recurrence. They were followed up for an average of 42.5 months. Conclusions. MCRCC is an uncommon subtype (3%) of RCC. The term MCRCC should be used exclusively to identify cystic RCC with a small volume (25% or less) of neoplastic clear cells in the cyst walls. The benign clinical course of these lesions suggests that patients may benefit from nephron-sparing surgery.

Multilocular Cystic Renal Cell Carcinoma

Multilocular cystic renal cell carcinoma (MCRCC) appears to be a distinct subtype of renal cell carcinoma with characteristic gross and microscopic features. The authors' ten-year experience (1977-1987) included six cases of MCRCC that were followed for a minimum of two years, with neither recurrence nor metastasis observed in any of the cases. During this period, there were 855 urologic procedures for the upper urinary tract, with 256 neoplasms or cysts identified. These included 32 simple cysts, 41 transitional cell carcinomas, 133 renal cell carcinomas, 17 papillary renal cell carcinomas, and 33 miscellaneous tumors. Histologically, the MCRCCs were well-demarcated multicystic lesions containing variably sized aggregates of neoplastic clear cells showing grade 1 nuclear features and little or no mitotic activity. The cyst walls were densely fibrotic, and the lining was often devoid of epithelium. Flow-cytometric analysis performed in five of the six cases with the use of paraffin-embedded tissue showed the tumors to be diploid in all instances, with low proliferative activity. The authors believe that this tumor is a low-grade variant of renal cell carcinoma and should be studied further to determine appropriate therapy.