Clear Cell Sarcoma Of Kidney Research Articles

CLEAR CELL SARCOMA KIDNEY: Clinical Features and Outcome

Clear cell sarcoma of kidney (CCSK) is a rare, highly malignant tumor. The clinical features and treatment outcome of 12 patients with CCSK are reported. From 1982 through December 1996, 12 cases of CCSK were seen at the Regional Cancer Centre, Trivandrum, India. Patients were staged according to NWTS III recommendation. They were treated with chemotherapy containing vincristine, actinomycin, and Adriamycin and radiotherapy. The survival curve was calculated by the Kaplan-Meier method. Mass and pain in the abdomen were the presenting symptoms. Male/female ratio was 3:1. Six had stage I, 4 had stage II, and 2 had stage III disease. Of the 12, 10 were evaluable, 6 are alive, and 3 recurred in 9 evaluable. Six patients are alive free of disease 10 to 108 months after diagnosis. The overall survival and disease-free survival of the 10 patients are 64 and 56%. It would appear that combined modality treatment can cure two thirds of children with CCSK. Effective treatment needs to be developed for children who fail after first line treatment.

Sarcoma renal de células claras fuera de la edad habitual

We report a case of a 17 year old woman with a clear cell sarcoma of kidney treated at the Division of Urology of Hospital General de Agudos Dr. José M. Ramos Mejía dependent of Goverment of Buenos Aires City. This report is based on the unusual age of tumor presentation and its good course and results after radiotherapy and chemotherapy, with 10 years survival after survergy.

Veno-Occlusive Disease-Like Hepatotoxicity in two Children Receiving Chemotherapy for Wilms' Tumor and Clear Cell Sarcoma of Kidney

We report two pediatric cases who developed veno-occlusive disease—like hepatotoxicity while receiving chemotherapy for Wilms' tumor and clear cell sarcoma of kidney. The chemotherapeutics, including vincristine, actinomycin D, and epirubicin in case 1 and vincristine and actinomycin D in case 2, were given before the hepatotoxicity developed. Other possible causes of hepatotoxicity were excluded. Recovery was observed with supportive therapy after 2 and 1 weeks, respectively. After recovery, the children tolerated continued chemotherapy without any decrease in the doses of drugs. We conclude that vincristine and actinomycin D were the cause of this rare form of hepatotoxicity and that chemotherapy for the underlying malignant disease could be given safely after clinical recovery.

Clear cell sarcoma of kidney: a clinicopathological study of eight cases from Malaysia.

Eight cases of clear cell sarcoma of kidney were seen in the Department of Pathology, University Hospital, Kuala Lumpur, Malaysia over the 16-year period from 1973 to 1989. Five of the patients were males. Six patients were Malay, one Chinese and one Indian. The patients' ages ranged from 8 months to 3 years. Clear cell sarcoma was the original diagnosis in two patients while six were diagnosed as blastemal-predominant Wilms' tumours at presentation. Metastases developed in five patients. Metastatic sites included the thoracic vertebra, skull, orbit, humerus, radius, ulna, shoulder, lung and liver. The prolonged survival, of 9 years and 9 months, seen in one patient despite omission of Adriamycin (doxorubicin) from the chemotherapeutic protocol is highlighted. We also emphasise the histological factors which are of help in differentiating clear cell sarcoma from Wilms' tumour.

Clear cell sarcoma of kidney: report of the first Malaysian case

Clear cell sarcoma of kidney (CCSK) is a rare but distinct tumor of childhood frequently confused with Wilms' tumor (nephroblastoma). It has a characteristic histology, a marked predilection for metastasis to bone, and an aggressive clinical course with a high relapse rate in spite of surgical excision, chemotherapy and radiotherapy. We report the first histologically proven CCSK in a Malaysian patient. This was an 8-mth-old Malay boy who was clinically diagnosed to have stage I Wilms' tumor. Despite treatment, he developed multiple metastases 10 mths after initial presentation and died soon after. Emphasis is placed on recognizing this entity in view of (1) its naturally aggressive behaviour and (2) the prospect of improving prognosis with currently recommended intensified chemotherapeutic regimes. Its immunohistochemical profile of vimentin-positivity and negativity for epithelial membrane antigen, cytokeratin and Factor-8 related antigen is more in favour of a mesenchymal or glomerular origin than a tubular or vascular origin.

Myoid Gonadal Stromal Tumor with Epithelial Differentiation (? Testicular Myoepithelioma)

Clear cell sarcoma of kidney (CCSK) is an aggressive childhood renal tumor of unknown histogenesis that has not been reported to occur outside the kidney. The article describes an extrarenal neoplasm arising in the pelvic soft tissues of a 13-year-old boy that was composed predominantly of uniform mesenchymal cells with optically clear cytoplasm supported by an arborizing network of small blood vessels, which was indistinguishable in appearance from CCSK. The electron microscopic findings, although nonspecific, were essentially identical to those of CCSK, with tumor cells displaying fine chromatin, electron-lucent cytoplasm, and intercellular collagen but no evidence of tissue-specific differentiation. Immunocytochemical studies showed positivity for vimentin but negative results for desmin, myoglobin, cytokeratin, epithelial membrane antigen, S-100 protein, neuron-specific enolase and factor VIII-related antigen. Tumor cells were also nonreactive with Ulex lectin. This unusual pelvic tumor and CCSK may both derive from primitive mesenchymal cells and may represent phenotypic but not necessarily histogenetic analogs.

Congenital Mesoblastic Nephroma: A Clinicoradiologic Study of 17 Cases Representing the Pathologic Spectrum of the Disease

Congenital mesoblastic nephroma (CMN) is a rare infantile renal tumor with a generally excellent prognosis. We describe 17 tumors that fit into the pathologic spectrum of CMN proposed by Beckwith, which ranges from benign renal tumors, through atypical “gray zone” lesions of more aggressive potential, to “crossover” tumors akin to clear cell sarcoma of kidney. Nine patients with histologically typical CMN were significantly younger and had smaller tumors than did eight patients with atypical CMN. Clinical features did not differ in the two groups of patients. A distinctive “ring sign” on renal sonography was commonly seen in patients with typical intrarenal CMN. All 17 patients were alive with no evidence of disease at a mean follow-up of 10 years. Nephrectomy was adequate therapy for younger infants and for those with typical CMN. Nephrectomy was probably also adequate therapy for infants 3 months of age or younger with atypical CMN, even if the tumor extended to the surgical resection margins and into the perinephric connective tissues. Adjuvant chemotherapy or radiation or both should be reserved for patients older than 3 months who have grossly unresected tumors and for those patients whose fumors have an unequivocally malignant histologic appearance or evidence of aggressive biologic behavior.

Congenital mesoblastic nephroma: A clinicoradiologic study of 17 cases representing the pathologic spectrum of the disease

Congenital mesoblastic nephroma (CMN) is a rare infantile renal tumor with a generally excellent prognosis. We describe 17 tumors that fit into the pathologic spectrum of CMN proposed by Beckwith, which ranges from benign renal tumors, through atypical "gray zone" lesions of more aggressive potential, to "crossover" tumors akin to clear cell sarcoma of kidney. Nine patients with histologically typical CMN were significantly younger and had smaller tumors than did eight patients with atypical CMN. Clinical features did not differ in the two groups of patients. A distinctive "ring sign" on renal sonography was commonly seen in patients with typical intrarenal CMN. All 17 patients were alive with no evidence of disease at a mean follow-up of 10 years. Nephrectomy was adequate therapy for younger infants and for those with typical CMN. Nephrectomy was probably also adequate therapy for infants 3 months of age or younger with atypical CMN, even if the tumor extended to the surgical resection margins and into the perinephric connective tissues. Adjuvant chemotherapy or radiation or both should be reserved for patients older than 3 months who have grossly unresected tumors and for those patients whose tumors have an unequivocally malignant histologic appearance or evidence of aggressive biologic behavior.

小児肉腫型腎腫瘍の臨床像 : II. 骨転移をきたした淡明細胞肉腫型腎腫瘍の2症例報告と文献的考察

小児肉腫型腎腫瘍の臨床像 : II. 骨転移をきたした淡明細胞肉腫型腎腫瘍の2症例報告と文献的考察

Wilms' tumor and other renal tumors of childhood: A selective review from the National Wilms' Tumor Study Pathology Center

Selected studies of Wilms' tumor and related renal neoplasms in children, which have been based on the National Wilms' Tumor Study Pathology Center collection of more than 2,600 renal tumors of childhood, are reviewed. The purpose of the review is to illustrate the value of the collaborative approach to uncommon pathologic specimens and to distinguish several tumors often confused with Wilms' tumor, including renal adenocarcinoma, renal teratoma, and renal neurogenic tumors. The unfavorable prognostic significance of anaplastic cells in Wilms' tumor is emphasized. Two recently described clinicopathologic entities--clear cell sarcoma of kidney and malignant rhabdoid tumor of kidney--and morphologic variants often confused with Wilms' tumor or congenital mesoblastic nephroma of infancy are discussed. Clear cell sarcoma of kidney and malignant rhabdoid tumor of kidney are, apparently, distinctive neoplasms, not Wilms' tumor variants.