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Clear Cell Sarcoma

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
Networked: 168 relevant articles (5 outcomes, 18 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

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

Experts

1. Fisher, David E: 5 articles (07/2015 - 06/2006)
2. Moritake, Hiroshi: 4 articles (12/2015 - 05/2002)
3. Sugimoto, Tohru: 4 articles (12/2015 - 05/2002)
4. Davis, Ian J: 4 articles (05/2013 - 06/2006)
5. Ichikawa, Hideki: 3 articles (12/2015 - 03/2013)
6. Sakurai, Yoshinori: 3 articles (12/2015 - 03/2013)
7. Andoh, Tooru: 3 articles (12/2015 - 03/2013)
8. Fukumori, Yoshinobu: 3 articles (12/2015 - 03/2013)
9. Sudo, Tamotsu: 3 articles (12/2015 - 03/2013)
10. Ono, Koji: 3 articles (12/2015 - 03/2013)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Clear Cell Sarcoma:
1. Caffeine (No Doz)FDA LinkGeneric
2. DNA (Deoxyribonucleic Acid)IBA
3. Proteins (Proteins, Gene)IBA
4. RNA-Directed DNA Polymerase (Reverse Transcriptase)IBA
5. Dermatofibrosarcoma protuberansIBA
01/01/2011 - "Whereas cutaneous angiosarcoma, myxofibrosarcoma, undifferentiated pleomorphic sarcoma of the skin showing morphologic similarities to atypical fibroxanthoma, and cutaneous leiomyosarcoma arise predominantly in elderly patients, dermatofibrosarcoma protuberans, epithelioid sarcoma, and superficially located clear cell sarcoma are seen more often in younger patients."
08/01/2014 - "In this review we aim to discuss the molecular implications for targeted therapy in selected rare soft tissue and bone sarcoma subtypes, including dermatofibrosarcoma protuberans (DFSP), alveolar soft part sarcoma (ASPS), clear cell sarcoma (CCS), giant cell tumor of bone (GCTB) and perivascular epithelioid cell neoplasms (PEComas). "
10/01/2012 - "This study includes 174 tissue microarray cases previously reported by Nonaka and colleagues, which include cases of melanoma, dermatofibrosarcoma protuberans, neurofibroma, synovial sarcoma, clear-cell sarcoma, malignant peripheral nerve sheath tumor (MPNST), perineurioma, and schwannoma. "
01/01/2014 - "The sarcoma tissue samples were collected from the archives of the Institute of Pathology, University of Heidelberg and were composed of 39 myxoid liposarcomas (MLS), 61 dedifferentiated liposarcomas, 15 pleomorphic liposarcomas, 27 leiomyosarcomas, 25 synovial sarcomas (SS), 35 malignant peripheral nerve sheath tumors (MPNST), 40 undifferentiated pleomorphic sarcomas, 17 myxofibrosarcomas, 9 low grade fibromyxoid sarcomas, 10 cases of dermatofibrosarcoma protuberans, 31 solitary fibrous tumors (SFT), 8 extraskeletal myxoid chondrosarcomas, 9 angiosarcomas, 6 alveolar soft part sarcomas, 5 clear cell sarcomas and 4 epithelioid sarcomas. "
11/01/2011 - "Diagnoses covered include undifferentiated high-grade pleomorphic sarcoma, fibrosarcoma, dermatofibrosarcoma protuberans, liposarcoma, synovial sarcoma, malignant peripheral nerve sheath tumor, clear cell sarcoma, hemangioendothelioma, hemangiopericytoma, angiosarcoma, and leiomyosarcoma."
6. Vincristine (Oncovin)FDA LinkGeneric
01/01/1998 - "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 - "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 - "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). "
7. Dactinomycin (Ac-De)FDA LinkGeneric
01/01/1998 - "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 - "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 - "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). "
8. AntigensIBA
9. AntibodiesIBA
10. tebufenozide (Mimic)IBA

Therapies and Procedures

1. Drug Therapy (Chemotherapy)
2. Boron Neutron Capture Therapy
3. Salvage Therapy
4. Adjuvant Chemotherapy
5. Nephrectomy