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At least 19 recordsLinked to original sources
PubMed2005

Myxoid clear cell sarcoma.

Clear cell sarcoma is a rare soft-tissue tumor presenting typically in the extremities of young adults. It has been also known as malignant melanoma of the soft parts because of the presence of melanin and cytoplasmic melanosomes. However, clear cell sarcoma is, at present, usually considered as a unique lesion because the t(12;22)(q13;q12) translocation is present only in clear cell sarcoma. Myxoid malignant melanoma is now a well-recognized morphologic variant of malignant melanoma. However, a myxoid variant of clear cell sarcoma has not been well described yet. We report a case of myxoid clear cell sarcoma occurring on the heel in a 22-year-old man. The tumor was composed of nests and fascicles of oval to fusiform cells with clear to pale eosinophilic cytoplasm, often separated by fibrous septa. The tumor cells were reactive for S-100 protein, HMB-45, and MART-1. Variably sized cysts lined by one or several layers of tumor cells were observed. Alcian blue and mucicarmine stains demonstrated prominent mucin deposition in the tumor stroma and especially in the lumen of the cysts. Fluorescence in situ hybridization for the Ewing sarcoma gene showed rearrangement in nearly all of the neoplastic cells.

Adult↗
PubMed2006

Sentinel node biopsy for clear cell sarcoma.

Clear cell sarcoma (CCS), also known as clear cell sarcoma of tendons and aponeuroses or malignant melanoma of soft tissue, is a rare malignant tumor and is histogenitically related to melanoma. The aim of this study was to describe our experience with the sentinel node (SN) procedure for CCS patients and to discuss the potential value of this technique for CCS patients. Five patients with a subcutaneous CCS, who underwent an SN procedure, are described. Two patients had positive SNs, with additional tumor positive nodes in both lymph node dissection specimens. Only the patients with tumor positive SNs developed recurrent disease during an average follow-up of 33 months. None of the negative SN patients developed recurrent disease and all were alive after an average follow-up of 39 months. SN status seems to predict additional nodal involvement and recurrent disease as well as survival. The SN procedure might be a useful and accurate staging procedure in CCS patients, comparable to the situation in melanoma.

Adult↗
PubMed2003

The management of clear cell sarcoma.

Clear cell sarcoma is a rare soft tissue tumour, constituting approximately 1% of all soft tissue sarcomas. Prognosis is reported to be poor due to the great propensity to metastasise regionally and distantly. In this paper, we report the surgical experience of two university hospitals. Both disease-free and overall survival after resection of clear cell sarcoma in this limited study were excellent, which may be explained by relatively small tumour size in seven out of eight patients and adjuvant radiation treatment. The current treatment for clear cell sarcoma is wide local tumour excision, with adjuvant radiation therapy for resection margins of less than 1 mm.

Adolescent↗
PubMedDate not supplied

Sentinel lymph node biopsy reveals a positive popliteal node in clear cell sarcoma.

Clear cell sarcoma of the tendons and aponeuroses is an aggressive, rare soft tissue tumor with frequent metastases to regional lymph nodes. Sentinel lymph node biopsy, which has dramatically changed the management of melanoma, was used for clear cell sarcoma for an evaluation of popliteal and groin lymph node status. Although all isosulfan blue-stained groin lymph nodes were negative for malignancy, a popliteal lymph node was positive. Adjuvant 50 Gy of radiotherapy to the popliteal node might have been effective for local control for one year.

Adolescent↗
PubMed2003

The melanocyte inducing factor MITF is stably expressed in cell lines from human clear cell sarcoma.

Clear cell sarcoma (CCS) is associated with the EWS/ATF1 oncogene that is created by chromosomal fusion of the Ewings Sarcoma oncogene (EWS) and the cellular transcription factor ATF1. The melanocytic character of CCS suggests that the microphthalmia-associated transcription factor (Mitf), a major inducer of melanocytic differentiation, may be miss-expressed in CCS. Accordingly, we show that the mRNA and protein of the melanocyte-specific isoform of Mitf (Mitf-M) are present in several cultured CCS cell lines (Su-ccs-1, DTC1, Kao, MST-1, MST-2 and MST-3). The above cell lines thus provide a valuable experimental resource for examining the role of Mitf-M in both CCS and melanocyte differentiation. Melanocyte-specific expression of Mitf-M is achieved via an ATF-dependent melanocyte-specific cAMP-response element in the Mitf-M promoter, and expression of Mitf-M in CCS cells suggests that EWS/ATF1 (a potent and promiscuous activator of cAMP-inducible promoters) may activate the Mitf-M promoter. Surprisingly, however, the Mitf-M promoter is not activated by EWS/ATF1 in transient assays employing CCS cells, melanocytes or nonmelanocytic cells. Thus, our results indicate that Mitf-M promoter activation may require an appropriate chromosomal context in CCS cells or alternatively that the Mitf-M promoter is not directly activated by EWS/ATF1.

Blotting, Western↗
PubMed2004

Alterations of the p16INK4a/p14ARF pathway in clear cell sarcoma.

Clear cell sarcoma (CCS) is a very rare soft tissue sarcoma with a poor prognosis. It has become apparent through immunohistochemical, ultrastructural, and microarray analyses that CCS is a soft tissue melanocytic neoplasm. Alterations in the p16INK4a/p14ARF gene are common in malignant melanoma, which is the prototypical melanocytic neoplasm. In the present study, we performed a clinicopathologic analysis and investigated p16 and cyclin D1 expression by immunohistochemistry in 14 cases. Furthermore, we investigated genetic changes of various tumor suppressor genes and an oncogene, including p16INK4a/p14ARF, p53, beta-catenin, and APC, in 11 cases. The 5-year overall survival rate in all the patients was 33.3%. A high mitotic rate was a significant adverse prognostic factor (P = 0.004). Decreased expression of p16 was observed in 4 (28.6%) of 14 cases. Overexpression of cyclin D1 was observed in 9 cases (64.3%). SSCP analysis followed by DNA direct sequencing revealed point mutations of the p16INK4a gene in 2 of 11 cases (18.2%). In addition, one case with the p14ARF mutation and 2 cases with the p53 mutation were observed. None of the cases harbored mutation of the beta-catenin or APC gene. Homozygous deletion of the p16INK4a/p14ARF gene was detected in one case. Methylation-specific PCR did not reveal hypermethylation of the p16INK4a/p14ARF promoter region in any of the cases. Three cases harbored genetic alterations of the p16INK4a/p14ARF gene (27.3%). All tumors with genetic alterations of the p16INK4a/p14ARF or p53 gene showed a high mitotic rate or tumor necrosis. These alterations were considered to be influential in the poor prognosis of CCS patients.

Adult↗
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↗
PubMed2006

Utility of CD117 immunoreactivity in differentiating metastatic melanoma from clear cell sarcoma.

CONTEXT: Clear cell sarcoma is a malignant soft tissue tumor with melanocytic differentiation. Molecular methods are sometimes necessary to identify the unique t(12; 22)(q13;q12) translocation and differentiate clear cell sarcoma from melanoma. OBJECTIVE: To determine whether CD117 immunoreactivity may be useful in separating melanoma from clear cell sarcoma. DESIGN: We identified 20 tumors listed in our surgical pathology files that were diagnosed as clear cell sarcoma or in which clear cell sarcoma was strongly considered. These were tested for the presence of the t(12;22) translocation by reverse transcriptase/polymerase chain reaction and sequencing from paraffin-embedded tissue. Tumors with a t(12;22) translocation were immunostained with an antibody to CD117 and compared with 16 similarly stained metastatic melanomas. RESULTS: Twelve tumors from 9 patients demonstrated t(12;22). No metastatic melanomas demonstrated t(12;22). None of the 12 clear cell sarcomas showed membrane or cytoplasmic staining for CD117. Conversely, 10 (63%) of 16 metastatic melanomas were, at least focally, positive for CD117; this difference was significant (P < .001). Interestingly, 3 tumors in which clear cell sarcoma was initially considered as a diagnosis, but which lacked t(12;22), were also positive for CD117. CONCLUSIONS: Reverse transcriptase/polymerase chain reaction, performed on paraffin-embedded tissue, is a useful, rapid tool for identifying the presence of t(12;22) in clear cell sarcoma. The CD117 immunoreactivity may prove useful in the differential diagnosis of deep soft tissue or visceral lesions with melanocytic differentiation; positive staining results exclude clear cell sarcoma, but are compatible with metastatic melanoma.

Adult↗
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↗
PubMed2003

Gastroduodenal metastases in a clear cell sarcoma patient.

Clear cell sarcoma is a rare tumor with an unpredictable clinical course and usually poor prognosis. Almost all of the patients will have pulmonary metastases during the clinical course. Other more common metastatic sites are bones, lymph nodes, liver and spleen. Treatment of clear cell sarcoma is primarily surgical aiming to achieve uninvolved margins. Role of chemotherapy, radiotherapy and immunotherapy is controversial and remission of metastases has been reported rarely. In this study we report a case of clear cell sarcoma, with gastroduodenal and pulmonary metastases and remission of these metastases in response to chemotherapy.

Antineoplastic Combined Chemotherapy Protocols↗
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↗
PubMed2006

Tigroid background in fine-needle aspiration cytology of clear cell sarcoma.

Clear-Cell Sarcoma (CCS) is a rare malignant soft tissue tumor of young adults that often metastasizes to the lymph nodes. The fine needle aspirate cytology (FNAC) findings of CCS have been described previously and it is included in the cytological category of polygonal soft tissue tumors. We describe the cytology of a case of CCS, with emphasis on the so called "tigroid background." Though this tigroid background is a nonspecific feature and has been described in various other tumors, to the best of our knowledge, it has not been described in CCS. This feature may be useful in the recognition of CCS.

Adolescent↗
PubMed2003

Primary clear cell sarcoma of bone.

Clear cell sarcoma is a rare soft tissue sarcoma of young adults with melanocytic differentiation. It occurs predominantly in the soft tissue of extremities, typically involving tendons and aponeuroses. Primary clear cell sarcoma of bone is extremely rare. We report a case of primary clear cell sarcoma of the right first metatarsal in a 48-year-old woman and provide a literature review of the entity.

Bone Neoplasms↗
PubMed2004

Primary clear cell sarcoma of rib.

Clear cell sarcoma (malignant melanoma of soft tissues) is a very rare soft tissue neoplasm. It generally arises in tendons and aponeuroses. Although metastasis of malignant melanoma to bone is not uncommon, primary clear cell sarcoma of bone is an extremely rare neoplasm. To our knowledge five cases have been reported in the English literature. We present a case of primary clear cell sarcoma of bone in a 28-year-old woman arising in the left ninth rib. We treated the patient with total excision of the mass and postoperative radiotherapy. The patient is alive and well without local recurrence or distant metastasis at 33 months after surgery.

Adult↗