The clinical experience of the novel drug temsirolimus on eight patients with metastatic renal cell carcinoma and who were refractory to other forms of treatment is reported. Although none of the patients showed complete or partial response, three patients had stable disease. One patient was prematurely withdrawn due to pneumonitis. Five patients died during the period of observation of twenty months and the median survival time from start of treatment was ten months. Three patients showed no evidence of adverse events (AE). Five patients showed dyslipidemia and two had pneumonitis for which, the drug had to be withdrawn in one of them. None had significant leucopenia. We conclude that temsirolimus has activity even in heavily pretreated patients in advanced renal cell carcinoma and in addition, has the benefits of ease of administration and good tolerability.
Clear-cell metastatic renal cell carcinoma

Cutaneous metastasis from renal cell carcinoma is unusual and have been reported infrequently. We report a case of metastatic renal cell carcinoma in a 33-year-old man who presented with a 3.5X2.0 cm-sized round, brightly red colored nodule on the scalp for 1 month. A skin biopsy revealed the typical clear cell adenocarcinoma with clear cytoplasm and oval hyperchromatic nuclei arranged in glandular pattern consistent with metastatic renal cell carcinoma. In most instances, once cutaneous involvement is manifested the disease is widespread and has a poor prognosis. But, our case presented only a single cutaneous nodule, and workup showed no evidence of further metastasis for 1 year.
Adenocarcinoma, Clear Cell ; Adult ; Biopsy ; Carcinoma, Renal Cell* ; Cytoplasm ; Humans ; Neoplasm Metastasis ; Prognosis ; Scalp* ; Skin

Adenocarcinoma of Lung ; Adenocarcinoma, Clear Cell ; Carcinoma, Renal Cell/pathology* ; Humans ; Kidney Neoplasms/pathology* ; Lung Neoplasms/pathology*

Renal cell carcinoma (RCC) is notorious for its propensity to metastasize even after a prolonged period of remission following nephrectomy. The metastatic spread can occur months or even years after initial treatment, which necessitates a heightened level of clinical awareness and vigilance in patients with a history of renal malignancy, particularly who present with new or unexplained nasal symptoms. Although RCC most commonly metastasize to the lungs, bones and liver, its involvement in the nasal cavity is exceedingly rare, posing significant diagnostic challenges due to the non-specific nature of symptoms. We describe a case of metastatic renal cell clear cell carcinoma presenting with recurrent epistaxis and unilateral nasal obstruction. Immunohistochemistry studies play a crucial role in confirming the diagnosis and ruling out potential differential diagnoses, along with a comprehensive clinical history of the patient.

Human ; Male ; Middle Aged: 45-64 Yrs Old ; Clear Cell Renal Cell Carcinoma ; Carcinoma, Renal Cell ; Metastasis ; Neoplasm Metastasis ; Nasal Cavity ; Epistaxis

Pediatric renal tumors represent a diverse group, which include Wilms' tumor (WT), renal cell carcinoma (RCC), clear cell sarcoma of the kidney (CCSK), congenital mesoblastic nephroma, malignant rhabdoid tumor of the kidney (MRTK) and primitive neuroectodermal tumor. WT (85%) and RCC (8%) are the most prevalent types. WT predominates among the 1- to 10-year age group, but RCC exceeds WT in children over age 10 years. Pediatric renal tumors are genetically, histologically and clinically heterogeneous. The overall survival for children with localized WT is currently more than 90%, whereas poorer survival rates are observed for anaplastic WT, metastatic WT, metastatic CCSK, MRTK, metastatic RCC and relapsed WT. Therefore risk-stratified treatment is important to minimize treatment morbidity while preserving survival. This review focuses on distinct characteristics of each tumor type and optimal stratified treatment.
Carcinoma, Renal Cell ; Child ; Humans ; Kidney ; Nephroma, Mesoblastic ; Neuroectodermal Tumors, Primitive ; Rhabdoid Tumor ; Sarcoma, Clear Cell ; Survival Rate ; Wilms Tumor

Cutaneous metastasis from renal cell carcinoma is not unusual, occurring in 3 to 7%, although it may be overlooked. We report a case of metastatic renal cell carcinoma in a 74-year-old man who was presented with a 1.5 x 1.5 x 0.5 cm-sized nodule on the right zygoma for 3 months. A skin biopsy demonstrated the typical clear cell adenocarcinoma with PAS(+) granules in the cytoplasm and honeycombed or glandular configuration, compatible with metastatic renal cell carcinoma. Kidney ultrasonogram and abdomina1 CT scanning showed a large cystic mass on the upper pole of the left kidney. Furthermore radiologic studies revealed multiple metastatic lesions in the lung, liver and brain. He died 4 months later.
Adenocarcinoma, Clear Cell ; Aged ; Biopsy ; Brain ; Carcinoma, Renal Cell* ; Cytoplasm ; Humans ; Kidney ; Liver ; Lung ; Neoplasm Metastasis ; Skin ; Tomography, X-Ray Computed ; Ultrasonography ; Zygoma*

When the tumor is advanced with distant metastasis or unresectable initially, preoperative chemotherapy could be applied in the treatment of Wilms' tumor We experienced 6 cases of favorable type of Wilms' tumor, 1 case of clear cell sarcoma and 1 case of renal cell carcinoma. They were treated with preoperative chemotherapy and underwent CT Scans before and after the therapy. Pathologic changes after chemotherapy in Wilms' tumor were known from previous reports as subtotal hemorrhagic necrosis, cystic change, clusters of foamy histiocytes, granulation tissue formation, primitive nephrogenic tissues and peripherally remained focal areas of blastemal infiltration. Changes of CT findings after chemotherapy were internal necrosis(6/6), decrease in size(5/6), decrease and absence of regional lymph node enlargement(4/6) and improved or disappeared metastatic lesions(3/3). Although our study had some limitations such as small numbers of cases and all cases were favorable types, we thought that there were good correlations between change of CT findings and subtotal hemorrhagic necrosis after preoperative chemotherapy in Wilms' tumor.
Carcinoma, Renal Cell ; Drug Therapy* ; Granulation Tissue ; Histiocytes ; Lymph Nodes ; Necrosis ; Neoplasm Metastasis ; Sarcoma, Clear Cell ; Tomography, X-Ray Computed ; Wilms Tumor

Adenocarcinoma, Clear Cell ; metabolism ; pathology ; surgery ; Adult ; Carcinoma ; metabolism ; pathology ; Carcinoma, Mucoepidermoid ; metabolism ; pathology ; Carcinoma, Renal Cell ; metabolism ; pathology ; secondary ; Diagnosis, Differential ; Humans ; Keratins ; metabolism ; Male ; Nasal Cavity ; Nose Neoplasms ; metabolism ; pathology ; surgery ; S100 Proteins ; metabolism

OBJECTIVETo 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.

METHODSThe 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.

RESULTSThe 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).

CONCLUSIONSCT 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.

Adenocarcinoma, Clear Cell ; diagnostic imaging ; Carcinoma, Renal Cell ; diagnostic imaging ; Diagnosis, Differential ; Humans ; Kidney ; diagnostic imaging ; Kidney Diseases, Cystic ; diagnostic imaging ; Kidney Neoplasms ; diagnostic imaging ; Tomography, X-Ray Computed

OBJECTIVETo assess the clinicopathological features and molecular genetic changes of multilocular cystic renal cell carcinoma (MCRCC).

METHODSAll 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.

RESULTSOf 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.

CONCLUSIONSMCRCC 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.

Adenocarcinoma, Clear Cell ; genetics ; pathology ; Adult ; Aged ; Biomarkers ; Carcinoma, Renal Cell ; genetics ; pathology ; Female ; Humans ; Keratins ; genetics ; Kidney ; pathology ; Kidney Diseases, Cystic ; genetics ; pathology ; Kidney Neoplasms ; genetics ; pathology ; Male ; Middle Aged ; Neoplasm Staging ; World Health Organization