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:

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 04/01/2013 - "The efficacy of caffeine-potentiated chemotherapy in clear cell sarcoma." 02/01/2013 - "This study reports the effect of caffeine-potentiated chemotherapy for clear cell sarcoma in five patients. " 02/01/2013 - "Caffeine-potentiated chemotherapy was administered to five patients with clear cell sarcoma. " 02/01/2013 - "Caffeine-potentiated chemotherapy for clear cell sarcoma: a report of five cases." 02/01/2013 - "Caffeine-potentiated chemotherapy can be effective treatment for clear cell sarcoma not only as initial therapy, but also as salvage therapy."
2.	DNA (Deoxyribonucleic Acid)IBA 09/01/1994 - "Clear cell sarcoma of the kidney--immunohistochemical study and flow cytometric DNA analysis of 7 cases." 12/01/1999 - "Clear cell sarcoma of kidney in an adolescent and in young adults: a report of four cases with ultrastructural, immunohistochemical, and DNA flow cytometric analysis." 04/15/1991 - "Eleven soft tissue clear cell sarcomas (CCS) were examined flow cytometrically for their DNA content with a correlation of the DNA with the patients' survival. " 09/01/1994 - "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. " 12/03/1999 - "Tumor cell viability in clear cell sarcoma requires DNA binding activity of the EWS/ATF1 fusion protein."
3.	Proteins (Proteins, Gene)IBA 08/01/1996 - "Eight histologically-confirmed cases of clear cell sarcoma of the kidney (CCSK) were studied for possible mutations in the p53 tumor suppressor gene by the immunohistochemical demonstration of mutant p53 proteins using a monoclonal (DO7: Dako) and a polyclonal (AB565: Chemicon) antibody to p53 protein. " 07/01/2006 - "Immunostaining for hMLH1 and hMSH2 was preserved in all the clear cell sarcomas but loss of immunostaining for one or both proteins was seen in 6/11 melanomas (55%). " 01/01/1998 - "The focus of this review will be on the translocation events associated with Ewing's sarcomas/PNETs (ES), alveolar rhabdomyosarcoma (ARMS), malignant melanoma of soft parts (MMSP) (clear cell sarcoma), desmoplastic small round cell tumor (DSRCT), synovial sarcoma (SS), and liposarcoma (LS), and the potential for targeting the resulting chimeric proteins in novel immunotherapies." 03/29/2001 - "The EWS/ATF1 and EWS/FLI1 fusion proteins associated with Clear Cell Sarcoma and Ewing's Sarcoma, respectively, were utilized to study the comparative effect of the EWS component on two different DNA binding partners. " 12/03/1999 - "These studies demonstrate a direct role for the EWS/ATF1 fusion protein in maintaining tumor cell viability of Clear Cell sarcoma and indicate that intracellular antibodies may be used to achieve a phenotypic knockout of tumor-related proteins as a method to explore their function."
4.	RNA-Directed DNA Polymerase (Reverse Transcriptase)IBA 04/01/1999 - "Only a small number of clear cell sarcoma (CCS) cell lines have been reported, including the cell line HS-MM. In the present study, this cell line, maintained for more than 4 years since establishment, was further characterized by cytogenetic studies, fluorescence in situ hybridization (FISH) analysis, and reverse transcriptase-polymerase chain reaction (RT-PCR). " 04/01/2008 - "Clear cell sarcoma of soft tissue: diagnostic utility of fluorescence in situ hybridization and reverse transcriptase polymerase chain reaction." 12/01/2006 - "EWS-ATF-1 fusion transcript, which is usually detected in clear cell sarcoma, was not demonstrated on reverse transcriptase-polymerase chain reaction. " 12/01/2005 - "As demonstrated by cytogenetics and reverse-transcriptase polymerase chain reaction, between 70% and over 90% of clear cell sarcomas have a t(12;22) translocation, fusing the EWS and ATF1 genes on chromosomes 22q12 and 12q13, respectively. " 02/01/2001 - "A balanced translocation, t(12;22)(q13;q13), which can be detected, amongst others, using the reverse transcriptase polymerase chain reaction (RT-PCR) or fluorescent in situ hybridization (FISH), has been identified in a high percentage (50-75%) of clear cell sarcomas and is presumed to be tumour specific. "
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 11/01/2007 - "Immunohistochemistry without antigen retrieval for CD117 was carried out on tissue microarrays consisting of 274 Wilms' tumours, 13 clear cell sarcomas of the kidney (CCSK), 10 mesoblastic nephromas (MN), and 7 rhabdoid tumours of the kidney (RTK). " 05/01/2002 - "Newly established clear cell sarcoma (malignant melanoma of soft parts) cell line expressing melanoma-associated Melan-A antigen and overexpressing C-MYC oncogene." 04/15/1991 - "Clear cell sarcomas and melanomas were also examined for their immunoreactivity to S-100 protein and HMB-45 antigen. " 02/01/2012 - "In this study, we evaluated 2 new melanoma markers, monoclonal antibodies KBA62 and PNL2 to yet unidentified antigens, using a large panel of metastatic melanomas (n=214), desmoplastic melanomas (n=34), gastrointestinal mucosal melanomas (n=54), benign nevi (n=27), clear cell sarcomas (n=16), and nonmelanocytic tumors (n=1218). "
9.	AntibodiesIBA 12/03/1999 - "These studies demonstrate a direct role for the EWS/ATF1 fusion protein in maintaining tumor cell viability of Clear Cell sarcoma and indicate that intracellular antibodies may be used to achieve a phenotypic knockout of tumor-related proteins as a method to explore their function." 05/01/2003 - "PNL2, as anti-Melan A and HMB-45 antibodies, stained most of the clear cell sarcoma cells, and a few cells in angiomyolipomas and lymphangioleiomyomatosis. " 03/01/1990 - "To test whether these observations are reproducible in the differential diagnosis of soft tissue sarcomas, anti-HLA-DR antibodies LK8D3 and LN3 were applied to formalin-fixed, paraffin-embedded sections of MFH, neurofibrosarcoma (NFS), leiomyosarcoma (LMS), synovial sarcoma (SS), fibrosarcoma (FS), angiosarcoma (AS), Kaposi's sarcoma (KS), chondrosarcoma (ChS), "dedifferentiated" chondrosarcoma (DChS), osteosarcoma (OS), epithelioid sarcoma (ES), and clear cell sarcoma (CCS; malignant melanoma of soft parts). "
10.	tebufenozide (Mimic)IBA 08/01/2012 - "Recently, it was reported that clear cell sarcoma can occur in the skin and mimic a broad spectrum of entities, including spindle cell melanoma. " 04/01/2007 - "Clear cell sarcoma of the kidney (CCSK) can display diverse morphological patterns and mimic various other pediatric renal tumors. " 08/01/2012 - "This study confirms that malignant dermal tumors that mimic but do not exactly replicate spindle cell melanoma should raise suspicion for cutaneous clear cell sarcoma and prompt the investigation for the confirmatory gene fusion t(12;22)." 12/01/2005 - "Importantly, EWS FISH is negative in malignant melanoma, a clinically dissimilar tumor, which may closely mimic clear cell sarcoma histologically and immunohistochemically. "

Therapies and Procedures

1.	Drug Therapy (Chemotherapy) 01/01/2003 - "Complete remission of metastatic clear cell sarcoma with DAV chemotherapy." 09/01/2011 - "There have been few studies documenting the response rate and progression-free survival in clear cell sarcoma patients treated with palliative chemotherapy. " 09/01/2011 - "Conventional chemotherapy has minimal activity in clear cell sarcoma as documented by the response rate of 4% and median progression-free survival of 11 weeks in this retrospective series. " 09/01/2011 - "The prospectively maintained databases of two referral centres were searched to identify clear cell sarcoma patients treated with chemotherapy. " 09/01/2011 - "Chemotherapy in clear cell sarcoma."
2.	Boron Neutron Capture Therapy 06/01/2014 - "Boron neutron capture therapy as new treatment for clear cell sarcoma: trial on different animal model." 03/01/2013 - "Boron neutron capture therapy (BNCT) selectively destroys human clear cell sarcoma in mouse model." 12/01/2015 - "Boron neutron capture therapy (BNCT) as a new approach for clear cell sarcoma (CCS) treatment: Trial using a lung metastasis model of CCS." 12/01/2011 - "Boron neutron capture therapy for clear cell sarcoma (CCS): biodistribution study of p-borono-L-phenylalanine in CCS-bearing animal models."
3.	Salvage Therapy 02/01/2013 - "Caffeine-potentiated chemotherapy can be effective treatment for clear cell sarcoma not only as initial therapy, but also as salvage therapy."
4.	Adjuvant Chemotherapy 01/01/2013 - "A review of the English literature revealed only five cases after its original description by Argani et al. Recognition of this entity can spare a child from potentially toxic adjuvant chemotherapy that might be used to treat malignant lesions which are part of the differential diagnosis, particularly clear cell sarcoma of kidney (CCSK)." 07/01/2000 - "Recognition of this entity can spare a child potentially toxic adjuvant chemotherapy that might be used for lesions in its differential diagnosis, specifically clear cell sarcoma of the kidney." 07/01/2009 - "The differentiation of metanephric stromal tumor from clear cell sarcoma of the kidney will spare the child from the ill effects of adjuvant chemotherapy. " 09/01/2001 - "It is possible that the identification of this renal tumor could spare a child the toxic adjuvant chemotherapy that would be administered if confused with histologically similar tumors such as clear cell sarcoma of the kidney."
5.	Nephrectomy 01/01/1998 - "The histology was favourable Wilms' tumour in 44, anaplastic in 12, unclassified in 8, clear-cell sarcoma in 4, and rhabdoid tumour in 3. Although a total nephrectomy was generally performed, partial renal surgery was performed for 6 bilateral and 4 unilateral tumours, the latter including 2 fused kidneys. " 03/01/1993 - "He had survived radical nephrectomy, chemotherapy, and radiotherapy for a congenital clear cell sarcoma of the right kidney. " 01/01/1991 - "Right radical nephrectomy was performed, and pathology proved the tumor to be a clear cell sarcoma, a sarcomatous variant of Wilms' tumor, of the kidney. " 04/01/1999 - "At review of the nephrectomy slides, the diagnoses were: Wilms tumor (16 patients), with anaplasia in one case, rhabdoid tumor (2 patients), neuroblastoma (2 patients), mesoblastic nephroma (2 patients), clear cell sarcoma (1 patient), malignant teratoma (1 patient), and renal cell carcinoma (1 patient). "