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Clear Cell Sarcoma

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114  relevant articles (2 outcomes, 7 trials/studies) found for this Disease

Description: A sarcoma of young, often female, adults of the lower extremities and acral regions, intimately bound to tendons as circumscribed but unencapsulated melanin-bearing tumors of neuroectodermal origin. An ultrastructural finding simulates flattened and curved barrel staves, corresponding to the internal structures of premelanosomes. There is a 45-60% mortality in clear cell sarcoma. (Segen, Dictionary of Modern Medicine, 1992)

Also Known As:
Sarcoma, Clear Cell; Clear Cell Sarcomas; Sarcomas, Clear Cell; Melanoma, Malignant, of Soft Parts

Relationship Network

Disease Context: Research Results

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Neoplasms: 387640
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Neoplasms by Histologic Type: 1
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Connective and Soft Tissue Neoplasms
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Sarcoma: 10281
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Clear Cell Sarcoma: 114
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Connective Tissue Neoplasms: 6
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Clear Cell Sarcoma: 114

Related Diseases

1. Neoplasm Metastasis (Metastasis)
2. Wilms Tumor (Wilm's Tumor)
3. Neoplasms (Cancer)
4. Rhabdoid Tumor (Rhabdoid Tumors)
5. Ewing's Sarcoma (Sarcoma, Ewing)

Experts

1. Nielsen, Torsten O: 1 article (06/2008)
2. Kwan, Wanda: 1 article (06/2008)
3. Liu, Shuzhen: 1 article (06/2008)
4. Lubieniecka, Joanna M: 1 article (06/2008)
5. Cheng, Hongwei: 1 article (06/2008)
6. Mahaira, Louisa: 1 article (05/2008)
7. Anagnostopoulos, Athanassios K: 1 article (05/2008)
8. Tsangaris, George T: 1 article (05/2008)
9. Tsimplouli, Chrisiida: 1 article (05/2008)
10. Dimas, Konstantinos: 1 article (05/2008)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Clear Cell Sarcoma:
1. DNA (Deoxyribonucleic Acid)IBA
2. Proteins (Proteins, Gene)IBA
3. RNA-Directed DNA Polymerase (Reverse Transcriptase)IBA
4. Vincristine (Oncovin)FDA LinkGeneric
01/01/1998 - "PATIENTS AND METHODS: Between August 6, 1986 and September 1, 1994, 1,687 previously untreated children less than 16 years of age with stages I to II/favorable histology (FH) or stage I/anaplastic histology WT (low-risk [LR] group) or stages III to IV/FH WT or stages I to IV/clear cell sarcoma of the kidney (high-risk [HR] group) were randomized to treatment that included vincristine and either divided-dose (standard [STD]) courses (5 days) or single-dose (pulse-intensive [PI]) treatment with dactinomycin"
03/01/1979 - "Clear cell sarcoma of tendons and aponeuroses treated with bleomycin and vincristine."
12/01/1998 - "PATIENTS AND METHODS: Between August 6, 1986 and September 1, 1994, 905 previously untreated children aged younger than 16 years with stage II favorable histology (FH) WT (low-risk [LR]), stages III to IV FH WT, or stages I to IV clear-cell sarcoma of the kidney (high-risk[HR]) were randomized after the completion of 6 months of chemotherapy to discontinue (short) or continue for 9 additional months (long) treatment with chemotherapy regimens that included vincristine and either divided-dose (standard [STD]) courses (5 days) or single-dose (pulse-intensive [PI]) treatment with dactinomycin"
09/01/1991 - "According to the National Wilms' Tumor Studies (NWTS), a group of patients with histologic features of anaplasia or sarcomatous Wilms' tumors (malignant rhabdoid tumors and clear cell sarcomas) were less responsive to vincristine and actinomycin"
10/01/1994 - "PURPOSE: To evaluate the effect of the sequential addition of doxorubicin (DOX) and cyclophosphamide (CTX) to the combination of vincristine (VCR) and dactinomycin (AMD) on the relapse-free survival of children with clear-cell sarcoma of the kidney (CCSK)"
Order ALL the reference details at left...
5. Dactinomycin (Ac-De)FDA Link
01/01/1998 - "PATIENTS AND METHODS: Between August 6, 1986 and September 1, 1994, 1,687 previously untreated children less than 16 years of age with stages I to II/favorable histology (FH) or stage I/anaplastic histology WT (low-risk [LR] group) or stages III to IV/FH WT or stages I to IV/clear cell sarcoma of the kidney (high-risk [HR] group) were randomized to treatment that included vincristine and either divided-dose (standard [STD]) courses (5 days) or single-dose (pulse-intensive [PI]) treatment with dactinomycin"
12/01/1998 - "PATIENTS AND METHODS: Between August 6, 1986 and September 1, 1994, 905 previously untreated children aged younger than 16 years with stage II favorable histology (FH) WT (low-risk [LR]), stages III to IV FH WT, or stages I to IV clear-cell sarcoma of the kidney (high-risk[HR]) were randomized after the completion of 6 months of chemotherapy to discontinue (short) or continue for 9 additional months (long) treatment with chemotherapy regimens that included vincristine and either divided-dose (standard [STD]) courses (5 days) or single-dose (pulse-intensive [PI]) treatment with dactinomycin"
09/01/1991 - "According to the National Wilms' Tumor Studies (NWTS), a group of patients with histologic features of anaplasia or sarcomatous Wilms' tumors (malignant rhabdoid tumors and clear cell sarcomas) were less responsive to vincristine and actinomycin"
10/01/1994 - "PURPOSE: To evaluate the effect of the sequential addition of doxorubicin (DOX) and cyclophosphamide (CTX) to the combination of vincristine (VCR) and dactinomycin (AMD) on the relapse-free survival of children with clear-cell sarcoma of the kidney (CCSK)"
Order ALL the reference details at left...
6. AntibodiesIBA
7. Melanins (Melanin)IBA
8. Activating Transcription Factor 1IBA
9. Transcription Factors (Transcription Factor)IBA
06/01/1994 - "The different translocation partners of the EWS gene, all of which are putative or definite transcription factor genes, may be responsible for the biological differences between DSRCT, Ewing's sarcoma, and clear cell sarcoma."
09/01/1995 - "Clear cell sarcoma, Ewing's sarcoma/primitive neuroectodermal tumor, and desmoplastic round cell tumor also show involvement of chromosome 22 with formation of a hybrid gene between the Ewing's sarcoma gene on band q12 and a transcription factor gene"
09/01/1995 - "Furthermore, six of the translocations, namely the t(11;22), t(21;22), and t(7;22) of Ewing's sarcoma, the t(12;22) of clear cell sarcoma, the t(12;16) of myxoid liposarcoma, and the t(11;22) of desmoplastic small round cell tumor, achieve this following a peculiar pattern, consisting in the fusion of a gene with an RNA-binding domain (EWS or TLS) with a transcription factor gene (FLI1, ERG, ETV1, ATF-1, CHOP, or WT1)"
09/15/2006 - "PURPOSE: Clear cell sarcoma (CCS) usually arises in the lower extremities of young adults and is typically associated with a t(12;22) translocation resulting in the fusion of EWS (EWSR1) with ATF1, a gene encoding a member of the cyclic AMP-responsive element binding protein (CREB) family of transcription factors"
04/01/2003 - "The Activating Transcription Factor 1 (ATF1)/Cyclic AMP Response Element Binding Protein (CREB) family of transcription factors are particularly relevant targets due to their strong association with melanoma and clear cell sarcoma"
Order ALL the reference details at left...
10. ParaffinIBA

Therapies and Procedures

1. Drug Therapy (Chemotherapy)
2. Nephrectomy
3. Adjuvant Chemotherapy
4. Heterologous Transplantation (Xenotransplantation)
5. Palliative Care (Palliative Medicine)

Best Treatments:
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