Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Clear cell sarcoma of the kidney (CCSK) is a distinct, highly malignant renal tumour of childhood which was diagnosed in 5 (3.9%) of 133 primary renal tumours in children under 15 years of age in the period from 1976 to 1990. The clinical and pathological features of these patients are presented. The most common clinical features were abdominal mass and haematuria. The male to female ratio was 1: 1.5 and there was predilection for the left side (4:1). The age of patients varied from 5 months to 4 years (average age 23 months). In four children the tumour was in Stage I, and in one child in Stage II. Three tumours showed classical microscopic pattern of CCSK. The treatment for all patients consisted of preoperative chemotherapy, radical surgery and additional aggressive chemotherapy. Four patients developed metastases: three of them to bones and one to the liver. Three patients died of the tumour, one is alive but with the tumour (3 years follow-up), and one child is alive and without any evidence of disease (3-years follow-up). Although relatively uncommon, these tumours are responsible for 50% of all children's deaths from renal tumours and, so, their precise classification is essential for proper treatment.
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PMID:[Clear cell sarcoma of the kidney in children]. 130 7

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.
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PMID:Clear cell sarcoma of kidney: report of the first Malaysian case. 137 51

Clear cell sarcoma of the kidney (CCSK) is a highly malignant childhood tumor, distinguished from classic Wilms' tumor by its propensity to metastasize to the skeletal system. Authors described a case of CCSK from a 3-year-old boy in the right kidney, showing various histologic features, such as classic, epithelioid, trabecular, neurilemmoma-like, cystic and entrapped collecting tubular pattern. Ultrastructurally epithelial differentiation was absent. Immunohistochemically, none of the intrinsic tumor cells showed positive staining with the antibodies against the keratin, S-100 protein, carcinoembryonic antigen, vimentin, desmin and myoglobin, suggesting primitive mesenchymal cell in origin.
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PMID:Clear cell sarcoma of the kidney--a case report. 263 49

We report an unusual case of renal sarcoma in a young adult. Histological examination demonstrated many similarities to the histopathological features of clear cell sarcoma of the kidney. Immunohistochemically, none of the intrinsic tumor cells showed positive staining with the antibodies against the intermediate filament proteins, epithelial membrane antigen, S100 protein, neuron-specific enolase, Leu-7 or myoglobin. The clinical course of this tumor was that of high grade malignancy, resulting in death with generalized metastases 13 months after tumor resection.
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PMID:Unusual renal sarcoma in a young adult: its similarities to clear cell sarcoma of the kidney. 356 Mar 36

The clinical and pathologic features of 21 children with clear cell sarcoma of the kidney are presented. These cases were identified among 517 primary renal tumors collected from four pediatric institutions. Abdominal mass and hematuria were the usual presenting signs. The male to female ratio was 1.3 to 1. Although the mean age at diagnosis was 3 years, the sarcomas were diagnosed earlier in boys (2 years, 7 months) than in girls (3 years, 7 months). Tumor size and weight may have accounted for this difference, since the average tumor weights were 899 g for boys and 635 g for girls. Right kidney locations predominated (14 cases). The most common site for metastasis was the skeleton (12 patients), and the skull was almost invariably involved (10 children). Bone metastases preceded the development of metastases elsewhere in nine patients, in five of whom the involvement was polyostotic. Of the 12 patients with osseous metastases, nine died within five years of diagnosis, and one died eight years and nine months after diagnosis; the remaining two patients were successfully treated with a combination of surgery, radiotherapy, and actinomycin D, vincristine, cyclophosphamide, and Adriamycin. Eight of the nine patients with lung metastases died within five years of diagnosis. Until substantial proof is found that clear cell sarcoma of the kidney is related to nephroblastoma, its identification as a non-Wilms' sarcoma would seem to be appropriate. This tumor is not the only sarcoma of the kidney, but it appears to be the most common renal sarcoma of childhood.
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PMID:Clear cell sarcoma of the kidney: a clinicopathologic study of 21 patients with long-term follow-up evaluation. 406 84

Clear cell sarcoma of the kidney (CCSK) is a malignant childhood tumor distinguished from Wilms' tumor by its gross, microscopic, and ultrastructural morphologic features and its propensity to metastasize to bone. Some clinical features of 75 cases of CCSK registered in the National Wilms' Tumor Studies I-III are presented along with the results of electron microscopic study of 12 tumors. Ultrastructural features of the tumor are compared to those of tumors of pericytes, Schwann's cells, renomedullary interstitial cells, fibroblasts, other renal tumors, and other clear cell tumors. Fine structural properties of primitive cells with variable numbers of organelles do not strongly suggest a histogenetic origin. The effect of formalin fixation on perinuclear cytoplasmic filaments and the tendency of tumor cell extensions to enfold the pale extracellular matrix explain the vacuolated appearance of some tumor cells.
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PMID:Clear cell sarcoma of the kidney with emphasis on ultrastructural studies. 609 89

Immunohistochemical study and flow cytometric DNA analysis were done on seven cases of clear cell sarcoma of the kidney (CCSK) to speculate its histogenesis and to access the diagnostic usefulness of these methods in the differential diagnosis of Wilm's tumor. Clinically, CCSK is a rare malignant renal tumor of children with a propensity to metastasize to bone. Arborizing vascular pattern surrounding the tumor cells which have clear cytoplasm is characteristic histologic finding. Immunohistochemically, only vimentin was diffusely demonstrated in the tumor cell membrane and cytoplasm. In flow cytometric DNA analysis, four cases showed diploidy and two cases near diploidy. CCSK is a separate disease entity with characteristic clinicopathologic, immunohistochemical and flow cytometric findings in distinction from Wilms' tumor. Considering the histologic and immunohistochemical findings, the possible histogenetic mechanism of CCSK seems to be in common with congenital mesoblastic nephroma (CMN), that is primitive mesenchymal cells which committed early stromagenic activity.
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PMID:Clear cell sarcoma of the kidney--immunohistochemical study and flow cytometric DNA analysis of 7 cases. 797 43

22 cases of clear cell sarcoma of the kidney (CCSK) were studied, 15 of them ultrastructurally and 7--immunohistochemically. CCSK is a rare malignant tumour of children, predominantly males, with an involvement of one kidney and frequent multiple metastases to the bones. Typical, epithelioid-trabecular, hyalinizing, cystic and myxoid variants are distinguished at the light microscopic level. As vimentin was found in tumour cells in all cases, antibodies against vimentin and cytokeratin can be recommended for differential diagnosis between CCSK and Wilms tumour and rhabdoid tumour. Peculiar vascular pattern can be revealed by means of antibodies against factor VIII. Immunohistochemical properties (presence of vimentin) and electron microscopy allow suggesting histogenic relation of CCSK with pericytes.
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PMID:[Clear-cell sarcoma of the kidney: the clinico-morphological, immunohistochemical and electron microscopic study of 22 cases]. 798 60

In recent reports on renal tumors of childhood with bone involvement neoplasms originally considered to be Wilms tumor have been assigned to new groups. After reviewing the literature, we knew that Wilms tumor rarely metastasizes in this way. Our case illustrates the unique biological feature of the rare, unfavorable histology Wilms tumor variant know as 'clear cell sarcoma of the kidney' (CCSK). Metastases to the spinal cord, as observed in our patient are distinctly unusual. To our knowledge, only two previous cases have been reported in the world literature.
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PMID:Spinal cord and bone metastasizing renal tumor of childhood. 855 25

Childhood kidney tumors seldom metastasize into the cranial cavity unless it is a special histological variant. We report a 4-year-old boy with multiple intracranial metastases in the left parietotemporal and right cerebellar area from primary clear cell sarcoma of the kidney without evidence of bony metastases. Metastatic tumor revealed nests of uniformly polygonal cells with clear cytoplasm demarcated by delicate fibrovascular arcades. Tumor cells were positive for vimentin and negative for cytokeratin, S-100 protein, desmin, and myoglobin. Cellular proliferation rate measured by PCNA, and Ki-67 was not significantly different between primary tumor mass and metastatic brain lesion. Expression of p53 oncoprotein was not evident in both lesions. These findings suggested that the relapse and metastasis of clear cell sarcoma of the kidney was probably due to regrowth of micro-metastases which were present at an early stage of disease.
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PMID:Intracranial metastasis from clear cell sarcoma of the kidney--a case report. 936 10


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