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At least 19 recordsLinked to original sources
PubMed1984

[Soft tissue clear cell sarcomas. Reevaluation of clear cell sarcomas of the tendons and the aponeuroses in 14 cases].

A retrospective study is realized including 14 cases of clear cell sarcomas of tendons and aponeuroses, found in the files of Institut Gustave-Roussy (1969-1983). The main microscopic features are emphasized. In comparison with other soft tissue sarcomas, clear cell sarcomas are clinically characterized by a great frequency of local lymph node metastases (4 of 14 cases). The prognosis is usually particularly poor. The exact histogenesis remains obscure, but the results of special stains, support origin from migrated neural crest cells: melanin is visualized in 2 out of 12 tumors in which Fontana-Masson is performed. S 100 protein, a neuroectodermal marker, is positive by immunoperoxidase technique (PAP) in 6 of 10 tested cases. A better diagnostic approach is allowed with the help of ultrastructural study performed in 3 cases. These tumors must be separated from synovialosarcomas. Because the histogenesis is not actually sure, it is preferable to call them "soft tissue clear cell sarcomas".

Adolescent↗
PubMedDate not supplied

Primitive pelvic sarcoma resembling clear cell sarcoma of kidney.

Clear cell sarcoma of kidney (CCSK) is an aggressive childhood renal tumor of unknown histogenesis that has not been reported to occur outside the kidney. The article describes an extrarenal neoplasm arising in the pelvic soft tissues of a 13-year-old boy that was composed predominantly of uniform mesenchymal cells with optically clear cytoplasm supported by an arborizing network of small blood vessels, which was indistinguishable in appearance from CCSK. The electron microscopic findings, although nonspecific, were essentially identical to those of CCSK, with tumor cells displaying fine chromatin, electron-lucent cytoplasm, and intercellular collagen but no evidence of tissue-specific differentiation. Immunocytochemical studies showed positivity for vimentin but negative results for desmin, myoglobin, cytokeratin, epithelial membrane antigen, S-100 protein, neuron-specific enolase and factor VIII-related antigen. Tumor cells were also nonreactive with Ulex lectin. This unusual pelvic tumor and CCSK may both derive from primitive mesenchymal cells and may represent phenotypic but not necessarily histogenetic analogs.

Adolescent↗
PubMed1984

Ultrastructural similarities and differences of synovial sarcoma, epithelioid sarcoma, and clear cell sarcoma of the tendons and aponeuroses.

The ultrastructural features of synovial sarcoma, epithelioid sarcoma, and clear cell sarcoma of the tendons and aponeuroses were compared to identify differential markers and similarities. A continuous spectrum of modulation of morphologic features of synovial and epithelioid sarcomas was observed. Biphasic synovial sarcoma with pseudoglandular and stromal components represents one extreme of this spectrum. The gradual disappearance of the pseudoglands and the formation of nests of epithelial-like cells, which are characteristic of epithelioid sarcoma, were observed. The cells of clear cell sarcoma, as well as those of synovial and epithelioid sarcomas, form epithelial-like islands; however, the presence of premelanosomes in the former is a feature of neural crest derivatives.

Humans↗
PubMed1985

[Pigment-free clear-cell sarcoma].

Clear-cell sarcoma of tendons and aponeuroses is an infrequent soft tissue sarcoma. Although this sarcoma is now accepted as a clinico-pathological entity and is well-defined clinically, questions on the histogenesis are still under discussion. Because of the possible presence of melanin pigment the idea was put forward that these tumors may be soft tissue melanomas. The authors studied a clear-cell sarcoma without melanin pigment and found at the ultrastructural level that the tumor cells often showed a basal lamina-like material in the vicinity of their cell membranes and were inclined to wrap around extracellular structures. They were in part connected to another by desmosome-like junctions and occasionally contained membrane-bound dense bodies resembling neurosecretory-type granules. Pinocytosis and glycogen were lacking. The results of electron microscopic examination showed this tumour to be closer to the malignant schwannoma rather than melanoma: histogenetically it is obviously linked to the neural crest.

Adult↗
PubMedDate not supplied

Concurrent chemoimmunotherapy in metastatic clear cell sarcoma: a case report.

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.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2002

Clear cell sarcoma of the stomach.

AIM: Clear cell sarcoma is a high-grade sarcoma with morphological features resembling malignant melanoma. This tumour is reported to display a characteristic distribution pattern nearly always involving the extremities. We report the first case of clear cell sarcoma of the stomach. METHODS AND RESULTS: A 30-year-old male developed a huge tumour of the stomach, which at first glance could be considered as a poorly differentiated carcinoma. Immunohistochemical and ultrastructural examinations were consistent with a diagnosis of metastatic melanoma. However, cytogenetic examination revealed a t(12;22) translocation, specific for clear cell sarcoma. This was confirmed by fluorescence in-situ hybridization. CONCLUSION: Making a reliable diagnosis of clear cell sarcoma of the stomach requires cytogenetic or molecular diagnostic investigations, particularly to rule out metastatic melanoma. This diagnosis avoids an unnecessary search for a primary melanoma.

Adult↗
PubMed1991

Complete remission of metastasised clear cell sarcoma of tendons and aponeuroses.

Clear cell sarcoma of tendons and aponeuroses is a rare disorder which originates from migrated neural crest cells. It tends to local recurrences and dissemination and the prognosis has to be considered as poor. Based on a small series of patients, a wide surgical excision of the primary tumour or amputation are the therapies of choice. Radiotherapy might be of some value as an adjuvant treatment but radiotherapy and chemotherapy are of little value in the treatment of the advanced disease. Because of the lack of treatment alternatives we treated a 40-year-old female patient with disseminated clear cell sarcoma with interferon-alpha 2b (IFN-alpha 2b) perilesionally after several courses of systemic chemotherapy and radiotherapy had failed. After 4 months of therapy the patient came into a complete pathological remission which lasted for 17 months. A relapse of round cell sarcoma on both tumour sites was then noted. This outcome shows that IFN-alpha 2b was able to induce a complete remission in clear cell sarcoma and might have altered the natural course of the disease. IFN-alpha should be studied as adjuvant therapy after surgery of primary clear cell sarcoma and as a first-line palliative treatment in disseminated disease.

Adult↗
PubMedDate not supplied

Clear cell sarcoma arising in the retroperitoneum.

Clear cell sarcoma is a rare soft-tissue neoplasm, arising most commonly in the tendons and aponeuroses of young adults. We report here the first female case of clear cell sarcoma arising in the retroperitoneum with clinical features similar to those of malignant ovarian tumors. Aspects of clinical presentation, histopathologic evaluation, and treatment are described.

Adult↗
PubMed1992

Reciprocal translocation t(12;22)(q13;q13) in clear-cell sarcoma of tendons and aponeuroses.

Clear-cell sarcoma is a rare soft tissue sarcoma that displays certain similarities to malignant melanoma. In this paper we describe the karyotypic findings and in vitro growth characteristics of a short-term-cultured clear-cell sarcoma. The cultured tumor cells had preserved immunohistochemical characteristics and certain ultrastructural features of the primary tumour, including positivity for vimentin, S-100 protein, and a melanoma-associated antigen, supporting the authenticity of the cultured cells. Cytogenetic analysis revealed an abnormal stemline karyotype of 49,XY, -1, +8, +8, +12, +der(1)t(1;?)(p36.1-.3;?), t(12;22)(q13;q13). A similar or identical t(12;22) was recently reported in two of four clear-cell sarcomas. It is suggested that the t(12;22)(q13;q13) is a primary cytogenetic abnormality in clear-cell sarcoma and distinguishes this tumor type from malignant melanoma.

Adult↗
PubMed1994

Establishment and characterization of a clear-cell sarcoma (malignant melanoma of soft parts) cell line.

A clear cell sarcoma (CCS) cell line, designated as NCS-1, was established in monolayer culture from a xenograft line originating from a metastatic CCS. Marked karyotypic aberrations and tumorigenicity in nude mice revealed the malignant derivation of the NCS-1 cell line. These cells contained abundant glycogen and were amelanotic by light microscopy. By electron microscopy, however, melanosomes in various developmental stages were seen, and some of them were partially melanized. The electron microscopic dopa reaction revealed the presence of tyrosinase activity. Enzyme-linked immunoadsorbent assay revealed that NCS-1 cells expressed a 75-kDa glycoprotein which was identified as a marker of highly differentiated melanoma cells. From these results, NCS-1 cells were found to retain both cytochemical and morphological properties of CCS. Application of NCS-1 cells to a panel of monoclonal antibodies recognizing melanocytic differentiation antigens showed that they corresponded approximately to highly differentiated melanoma cells. In conclusion, the present study strongly supports the close relationship between CCS and malignant melanoma.

Adult↗
PubMed1997

Late recurrence of clear cell sarcoma of the kidney.

Clear cell sarcoma of the kidney (CCSK) is a rare pediatric neoplasm with particular propensity for bone metastasis that requires aggressive therapy. We report a patient with CCSK who was misreported as having Wilm's tumour at the time of initial diagnosis and received only minimal therapy. The disease recurred locally-after 8 years, with no evidence of distant metastasis. Important clinical and histologic features of CCSK are described, along with a review of the literature.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1992

Significance of both numerical and structural chromosomal abnormalities in clear cell sarcoma.

We analyzed cytogenetically two cases of clear cell sarcoma. The t(12;22)(q13;q13) hypothesized to be characteristic of clear cell sarcoma was detected in one of the two cases. Although this translocation was not observed in the other case; trisomy of chromosome 22 was, as were other numerical abnormalities. This latter observation has also been described in other reported studies of clear cell sarcoma. These findings confirm the nonrandomness of t(12;22)(q13;q13) in clear cell sarcoma, but also suggest that in its absence other abnormalities of chromosome 22 are significant. Trisomy 8, noted in both cases we report, as well as in previous studies, also appears to play an important role in clear cell sarcoma.

Adult↗
PubMedDate not supplied

Long survival with metastatic clear cell sarcoma of the Achilles tendon.

Clear cell sarcoma of tendons and aponeuroses is a rare tumor with a predilection for the lower extremities, particularly the foot. Although it is claimed to have a poor prognostic outlook, it is sometimes characterized by a slow clinical course. The authors report a case with a prolonged survival period of 7 years--5 years in the presence of multiple lung metastases.

Achilles Tendon↗
PubMed1983

Acral lentiginous melanoma simulating "clear cell sarcoma of tendon and aponeuroses".

Clear cell sarcoma was closely mimicked in metastatic tumor deposits from two patients with acral lentiginous malignant melanoma. A subcutaneous deposit composed of glycogen-laden spindle cells dominated the presenting clinical picture in one patient. In the other, primary acral melanoma resembled a histiocytic tumor and metastatic tumor simulated clear cell sarcoma. The cases illustrate the pleomorphism that may be encountered in malignant melanoma.

Adult↗
PubMed2000

Comparative genomic hybridization analysis of clear cell sarcoma of the kidney.

BACKGROUND: Clear cell sarcoma of the kidney (CCSK) is a rare malignant pediatric tumor, distinguished from the Wilms tumor by its characteristic histologic features and a more aggressive clinical behavior with a tendency to metastasize to bone. Genetic studies on CCSK are limited and no consistent findings have been reported. PROCEDURE: We examined four cases of CCSK for presence of consistent genetic alterations using comparative genomic hybridization (CGH). This is the first report concerning CGH analysis of CCSK. RESULTS: Three of the tumors showed no chromosome gains or losses. One of the tumors had gains of 1 q and the terminal end of 11 q. CONCLUSIONS: These results are consistent with previous findings of limited chromosomal changes in CCSK karyotypes. Gain of 1 q in CCSK warrants further investigation. Copy number gains of 1 q have been repeatedly demonstrated in soft tissue and bone sarcomas, as well as other tumors, implying the presence of genes involved in tumor development and/or progression.

Child↗
PubMed2002

Molecular genetic characterization of the EWS/ATF1 fusion gene in clear cell sarcoma of tendons and aponeuroses.

Clear cell sarcoma (CCS) is a rare malignant soft tissue tumor particularly associated with tendons and aponeuroses. The cytogenetic hallmark is the translocation t(12;22)(q13;q12) resulting in a chimeric EWS/ATF1 gene in which the 3'-terminal part of EWS at 22q is replaced by the 3'-terminal part of ATF1 at 12q. To date, only 13 cases of CCS have been analyzed for fusion genes at the transcription level, and there is no information about the breakpoints at the genomic level. In the present study, we describe the molecular genetic characteristics of CCS from 10 patients. Karyotypes were obtained from 10 cases, 7 of which showed the characteristic t(12;22). As an initial step in the characterization of the EWS/ATF1 and ATF1/EWS chimeras, we constructed an exon/intron map of the ATF1 gene. The entire ATF1 gene spanned >40 kb and was composed of 7 exons. Intron 3, in which most of the genomic breakpoints occurred, was to a large extent (83%) composed of repetitive elements. RT-PCR amplified EWS/ATF1 cDNA fragments in all patients and ATF1/EWS cDNA fragments in 6 of 10 patients. Four types of EWS/ATF1 chimeric transcript, designated types 1-4, were identified. The most frequent chimeric transcript (type 1) was an in-frame fusion of exon 8 of EWS with exon 4 of ATF1. This was the only chimeric transcript in 5 patients but found together with other variants in 3 tumors. The type 2 transcript of EWS/ATF1, an in-frame fusion of exon 7 of EWS with exon 5 of ATF1, was detected in 4 patients, as the only transcript in 1 case and together with other variants in 3 cases. An in-frame fusion of exon 10 of EWS with exon 5 of ATF1 (type 3) was found in 1 patient as the only transcript, and an out-of-frame fusion of EWS exon 7 with ATF1 exon 7 (type 4) was detected in 1 patient together with type 1 and type 2 transcripts. Sequencing of the amplified ATF1/EWS cDNA fragments showed in 5 patients that ATF1 exon 3 was fused with EWS exon 10, resulting in an out-of-frame chimeric transcript. In 1 case, nt 428 of ATF1 (exon 4) was fused with EWS exon 8; at the junction, there was an insertion of 4 nucleotides, also resulting in an out-of-frame transcript. Genomic extra long PCR and sequence analysis mapped the genomic breakpoints to introns 7, 8 and 9 of EWS and intron 3 and exon 4 of ATF1. While a simple end-to-end fusion was observed in 2 cases, additional nucleotides were found at the junctions in 2 other cases. In addition, topoisomerase I consensus sequences were found close to the junctions, suggesting that this enzyme may participate in the genesis of the EWS/ATF1 fusion.

Adolescent↗