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)

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.
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PMID:Clear cell sarcoma of kidney: a clinicopathological study of eight cases from Malaysia. 132 18

Clear cell sarcoma of the hand. Case report. Histologic, ultrastructural and immunohistochemical features of a case of clear cell sarcoma (also called malignant melanoma of soft parts) arisen in the index finger of the right hand in a 28 year-old-woman are described in this report. Typical forms of this tumor are deeply located and associated with tendons and aponeuroses, lacking cutaneous involvement. The tumor has to be differentiated from other benign and malignant lesions of the soft parts, such as a giant cell tumor of tendon sheaths and a fibrosarcoma. The demonstration of melanin and a positive immunohistochemical reaction for S-100 protein and HMB-45 (a melanin-associated antigen) can assist in the differential diagnosis. In spite of a slow and protracted clinical course, many of the patients experience multiple local recurrences and distant metastases. Prognosis is poor in a high percentage of cases.
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PMID:[Clear cell sarcoma of the hand. Description of a case]. 236 89

Twenty-two histologically confirmed cases of Clear Cell Sarcomas (C.C.S.) seen over a sixteen year period, are retrospectively analysed from a clinico-pathological view-point. The tumours occurred in young adults, average 36 years. Males predominated, and the lower extremity particularly the thigh and foot were the most commonly involved. Grossly, a connection with aponeurosis and tendon sheath has not been recorded consistently. Microscopically the tumour was distinctive, with their short fascicles and nests of polygonal to fusiform cells. The "clear" cytoplasm stressed in it's very name was not the rule. Melanin was demonstrated in 7 of the 22 cases, i.e. 32%, and these patients did not behave any differently from those where pigment was not seen. Immunocytochemistry for S-100 protein was done in 14 cases and was positive in 13 cases. Follow-up information revealed that 11 patients were dead of advanced disease in an average period of 24 months. Two patients were alive with disease for an average period of 3 years 7 months. Only 4 patients had no evidence of disease for periods ranging from 6 months to 26 months. Metastasis to nodes was a very frequent event, but lung and bony metastasis has also been documented.
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PMID:Clear cell sarcomas of the tendon sheath. An experience of 22 cases seen over 16 years. 256 Sep 90

The clinical and histopathological features of four cases of clear cell sarcoma of kidney (CCSK) or Bone metastasising renal tumour of childhood (BMRTC) are presented. These cases were identified among 107 primary renal tumours in childhood over a period of 15 years (1973-1987) in the Trivandrum Medical College. Of the 107 cases 96 (89.7 percent) were nephroblastomas and 7 (6.6 percent) were Mesoblastic Nephromas. The incidence of Clear cell sarcoma was 3.7 percent. Abdominal mass and haematuria were the most common clinical features. All the four cases occurred in male children with no predilection for the right or the left kidney. At the time of presentation bone metastasis was not present in any of the four cases. Metastasis to scapula and skull was detected ten months after nephrectomy in one case. Of the four patients three were in stage I disease at the time of diagnosis. All the four cases showed the typical gross morphology and the classic microscopic pattern of Clear Cell sarcoma kidney. The treatment was similar in all the four cases with Surgery followed by radiotherapy and chemotherapy (Vincristine, Adriamycin Actinomycin D and cyclophosphamide). Only one of the four patients is alive and well 12 months after surgery. The literature is reviewed along with a discussion of the gross pathology, histology and histogenesis of clear cell sarcoma of kidney.
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PMID:Clear cell sarcoma of kidney (CCSK)--a clinicopathological study of 4 cases. 259 97

Clear cell sarcoma is an indolent tumor of uncertain histogenesis. Twenty-seven patients ranging in age from 9 to 57 years (average, 28.5 years) were followed an average of 7.0 years. Females outnumbered males by 2 to 1. Distal extremity locations predominated. At presentation, the tumor was localized in 21 patients, regionally metastatic in 5, and disseminated in 1. Surgery was the primary therapy for 26 patients. Adjuvant treatment was nonstandardized, and its effectiveness is undetermined. Local recurrence developed in 10 patients, regional metastases in 9, and widespread dissemination in 12. Twelve patients died of the disease from 7 months to 10 years after diagnosis. Only 11 patients remained free of disease. It would appear that wide excision, or perhaps even radical excision or amputation, is the surgical treatment of choice.
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PMID:Clear cell sarcoma. A clinicopathologic study of 27 cases. 661 10

Clear cell sarcoma or malignant melanoma of soft parts is a rare tumor with a predilection for the extremities. Although characterized by a slow clinical course, prognosis is poor because of a high incidence of local recurrences and distant metastases. In this report, two additional cases which originated in the toes are presented.
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PMID:Clear cell sarcoma (malignant melanoma of soft parts). Presentation of two additional cases. 849 27

Clear cell sarcoma, also called malignant melanoma of soft parts, is an uncommon neoplasm that involves tendons or aponeuroses of the lower extremity. The CT features of a clear cell sarcoma arising from the abdominal wall with later peritoneal dissemination are described. Peritoneal sarcomatosis from soft tissue sarcomas is a very rare condition previously unreported in the radiologic literature. Metastases to peritoneal surfaces must therefore be considered a possible site for systemic dissemination of soft tissue sarcomas.
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PMID:Clear cell sarcoma of the abdominal wall with peritoneal sarcomatosis: CT features. 1052 63

Clear cell sarcoma is a rare soft tissue neoplasm whose clinical behavior and outcome has not been previously characterized. This study reviewed the clinical characteristics and outcome of all children with clear cell sarcoma of the soft tissues who were treated at St. Jude Children's Research Hospital from March 1962 through August 1998. Of 225 children with nonrhabdomyosarcomatous soft tissue sarcomas, 5 (2.2%) were diagnosed with clear cell sarcoma. Median age at diagnosis was 15 years 3 months. Primary sites included the extremities (n = 3), chest wall (n = 1), and abdomen (n = 1). At diagnosis 3 patients had localized disease. Following surgical resection (n = 3), radiotherapy (n = 2), and chemotherapy (n = 1) all three survive disease-free 10, 11, and 90 months after diagnosis, respectively. The remaining two patients with metastatic disease at diagnosis died 21 days and 9 months after diagnosis. Clear cell sarcoma of the soft tissues is rare in pediatrics. Complete surgical resection with negative margins is the most effective treatment for this disease. Patients with metastatic disease are candidates for multiinstitutional chemotherapy trials.
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PMID:Clear cell sarcoma of soft tissues in children and young adults: the St. Jude Children's Research Hospital experience. 1059 94

Clear cell sarcoma is a rare tumor with a poor prognosis. The therapeutic approach in the metastatic disease stage is controversial: to the authors' knowledge the use of concurrent chemoimmunotherapy has not been previously reported. We present a case of a 57-year-old male with metastatic clear cell sarcoma treated simultaneously with subcutaneous interferon-a 2b and six courses of chemotherapy according to the CyVEDIC regimen. Disease stabilization lasting 17 months was achieved.
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PMID:Concurrent chemoimmunotherapy in metastatic clear cell sarcoma: a case report. 1077 76

A variety of pediatric renal masses may be differentiated from Wilms tumor on the basis of their clinical and imaging features. Wilms tumor is distinguished by vascular invasion and displacement of structures and is bilateral in approximately 10% of cases. Nephroblastomatosis occurs most often in neonates and is characterized by multiple bilateral subcapsular masses, often associated with Wilms tumors. Renal cell carcinoma is unusual in children except in association with von Hippel-Lindau syndrome and typically occurs in the 2nd decade. Mesoblastic nephroma is the primary consideration in a neonate with a solid renal mass. Multilocular cystic renal tumor is suggested by a large mass with multiple cysts and little solid tissue. Clear cell sarcoma is distinguished by frequent skeletal metastases, and rhabdoid tumor is distinguished by its association with brain neoplasms. Angiomyolipoma frequently contains fat and is associated with tuberous sclerosis. Renal medullary carcinoma occurs in patients with sickle cell trait or hemoglobin SC disease and manifests as an infiltrative mass with metastases. Ossifying renal tumor of infancy is differentiated from mesoblastic nephroma by the presence of ossified elements. Metanephric adenoma lacks specific features but is always well defined. Renal lymphoma is characterized by multiple homogeneous masses, often with associated adenopathy.
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PMID:Pediatric renal masses: Wilms tumor and beyond. 1111 13


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