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At least 37 records · Page 2Linked to original sources
PubMed2003

Cytogenetic comparison between clear cell sarcoma and a case of acral clear cell melanoma.

BACKGROUND: Clear cell melanoma is a recently described variant of melanoma characterized by prominent clear cell features similar to clear cell sarcoma. PATIENTS AND METHODS: The present report describes a deeply invasive case of clear cell melanoma clinically, histologically, immunohistochemically, ultrastructurally and with the FISH technique and compares these findings with those in clear cell sarcoma. RESULTS: A 68-year-old male presented with an ulcerated, deeply invasive melanoma on the heel, corresponding to Clark level V, with a maximum tumour thickness of more than 14 mm; follow-up revealed inguinal lymph-node and lung metastases. Apart from characteristic histological features of melanoma with intraepidermal involvement, the lesion was immunohistochemically positive for 5100 protein, with HMB45 and A103 (anti-human Melan-A/MART-1), and ultrastructurally showed melanosomes. In deeply invasive parts, the lesion was indistinguishable from clear cell sarcoma, exhibiting nests, fascicles and sheets of pale-staining epithelioid to spindle-shaped cells ('clear cells') with scattered Touton-like multinucleated giant cells. Cytogenetic analysis using the FISH technique revealed a 12q13 chromosomal break in the vicinity of the ATFI gene but without ATF1 disruption. CONCLUSION: These findings may indicate the possible involvement of the ATF1 gene in clear cell melanoma tumorigenesis, similar to clear cell sarcoma with classical t(12;22) translocation.

Activating Transcription Factor 1↗
PubMed2005

Osteoclast-rich tumor of the gastrointestinal tract with features resembling those of clear cell sarcoma of soft parts.

Clear cell sarcoma is a high-grade sarcoma with morphological features resembling those of malignant melanoma. An osteoclast-rich tumor of the gastrointestinal tract with features resembling those of clear cell sarcomas of soft parts is very rare. Herein, we report an unusual stomach tumor with microscopic and immunohistochemical characteristics of an osteoclast-rich tumor of the gastrointestinal tract with features resembling those of clear cell sarcomas of soft parts. The tumor cells were predominantly oval, admixed with some round and spindle elements arranged in nests and fascicles, and admixed with scattered osteoclast-like multinucleated giant cells. Neoplastic cells were positive for S-100 protein, and osteoclast-like multinucleated giant cells were immunoreactive to CD68. The unusual morphology of the tumor caused significant diagnostic difficulties. The differential diagnosis included gastrointestinal stromal tumor, primary or metastatic melanoma, and epithelioid malignant peripheral nerve sheath tumor. To the best of our knowledge, this is possibly the second description of an osteoclast-rich tumor of the gastrointestinal tract with features resembling those of clear cell sarcomas of soft parts.

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↗
PubMed2006

Constitutive activation of neuregulin/ERBB3 signaling pathway in clear cell sarcoma of soft tissue.

Clear cell sarcoma of soft tissue (CCSST) represents a highly malignant tumor of the musculoskeletal system that is characterized by the chromosomal translocation t(12;22)(q13;q12) of the Ewing sarcoma gene (EWSR1) and activating transcription factor 1 (ATF1). In a former microarray expression study, we identified ERBB3, a member of the epidermal growth factor receptor (EGFR) family, as a promising new diagnostic marker in the differential diagnosis of CCSST. Here we show that, besides ErbB3, all CCSST cell lines (n = 8) also express the ErbB2 receptor or the ErbB4 receptor, representing an adequate coreceptor of ErbB3. The phosphorylation status of ErbB3 revealed these receptor pairs to be either constitutively activated in CCSST cells with high neuregulin-1 (NRG1) expression (n = 4) or activatable by exogenic NRG1 in cells showing low amounts of NRG1 mRNA (n = 4). Exogenous NRG1 stimulated the growth of a subset of CCSST cells but did not affect the kinetics of another subset. This difference was not strictly dependent on endogenous NRG1 expression; however, the growth-inhibiting effect of the pan-ErbB tyrosine kinase inhibitor CI-1033 or PD158780 clearly correlated with NRG1 expression indicating an autocrine growth stimulation loop which may constitute an interesting target of new therapeutic strategies in this tumor entity.

Alleles↗
PubMed2007

Clear cell sarcoma of soft tissues.

Clear cell sarcoma (CCS) is a rare but highly malignant tumor of soft tissues often appearing as a small tender mass in the deep tissues of the distal extremities. We have studied 17 patients with such lesions treated since 1986 and have a high incidence of local recurrence and metastasis with a survival rate of only 47% despite surgery and for many of the patients, adjuvant therapy. The purpose of this article is to warn the readers of the dangers related to treating this seemingly benign lesion and urge them to perform wide surgery and utilize adjuvant therapy.

Adolescent↗
PubMed2004

Clear cell sarcoma: an institutional review.

Clear cell sarcoma of the tendons and aponeuroses (CCSTA) is an aggressive, rare soft-tissue tumor representing approximately 1 per cent of all soft tissue sarcomas. Although it appears to be histogenetically related to melanoma, its clinical behavior resembles soft tissue sarcoma with a propensity for lymph node metastases. We report our experience at a tertiary referral center. Eight cases of CCSTA evaluated at the University of Illinois at Chicago's Medical Center between 1980 and 2002 were retrospectively reviewed. Patient data analyzed included age, gender, anatomic location, size of tumor, development of local, regional, and distant recurrence, and patient status at last follow-up. Local recurrence did not develop in any patient. Metastases developed in three patients with median time to presentation of metastases of 23 months (range, 8-36 months) from the time of initial diagnosis. Two of these three patients died of their disease with an average interval until death of 22 months from the time of initial diagnosis. The disease-free 2- and 5-year survivals were 68 per cent and 50 per cent, respectively. The overall 2- and 5-year survivals were 86 per cent and 68 per cent, respectively. CCSTA is an aggressive tumor of the soft tissues. These tumors present a high risk for development of distant disease and therefore warrant aggressive surgical management and treatment. Neither aggressive multiagent chemotherapy nor radiotherapy appeared to have an impact on outcome.

Adult↗
PubMed2003

Genetic and genetic expression analyses of clear cell sarcoma of the kidney.

Clear cell sarcoma of the kidney (CCSK) represents a significant diagnostic and clinical challenge. In search of diagnostically useful or biologically significant genetic abnormalities, we screened 30 CCSKs from the National Wilms Tumor Study Group. Genetic gains and losses were analyzed using comparative genomic hybridization; loss of heterozygosity at 11p15 was studied using microsatellite analysis. Loss of imprinting (LOI) was studied using allele-specific expression or methylation analysis at the ApaI polymorphic site for IGF2, AluI and RsaI sites for H19, and Cfo I site for SNRPN. Comparative genomic hybridization analysis revealed quantitative abnormalities in only 4 of 30 CCSKs. Two showed gain of 1q, one also showed loss of 10q, and the other also showed loss of terminal 4p. The other two cases demonstrated chromosome 19 loss and chromosome 19p gain, respectively. All 22 cases informative for 11p15 showed retention of both alleles. Of 14 CCSKs informative for IGF2, six showed biallelic expression; all three CCSKs informative for H19 exhibited monoallelic expression. The normal imprint pattern was present in all six CCSKs analyzed for SNRPN methylation. These data demonstrate an absence of consistent genetic gains or losses in CCSKs using these methods. The high frequency of LOI for IGF2 in CCSKs (43%) is comparable to that reported in Wilms tumors. The retention of imprinting at the SNRPN and H19 loci confirm that LOI is not a ubiquitous epigenetic change. This suggests that IGF2, a potent growth factor, may play a role in the development or progression of CCSK.

Adolescent↗
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↗
PubMed2005

Dual-color, break-apart fluorescence in situ hybridization for EWS gene rearrangement distinguishes clear cell sarcoma of soft tissue from malignant melanoma.

Clear cell sarcoma of soft tissue (malignant melanoma of soft parts) is a soft tissue sarcoma with melanocytic differentiation that typically occurs in the tendons and aponeuroses of young adults. 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. Identification of this translocation distinguishes clear cell sarcoma from histologic mimics, most importantly conventional malignant melanoma. We report our experience with a commercially available, dual-color, break-apart fluorescence in situ hybridization (FISH) probe, which allows detection of EWS (22q12) gene rearrangement in formalin-fixed, paraffin-embedded tissues. Histologically and immunophenotypically well-characterized cases of clear cell sarcoma (n = 10) and malignant melanoma (n = 32) were evaluated with a 22q12 dual-color, break-apart probe (Vysis, Downer's Grove, IL, USA), which spans the known common breakpoints in the EWS gene on chromosome 22 (introns 7-10). Signals from tumor cell nuclei were counted under a fluorescence microscope and the presence of red-green break-apart signals was recorded. Of the clear cell sarcoma cases, seven of 10 showed evidence of an EWS gene rearrangement with a mean of 81.6% positive cells per sample (range: 60-95%). All cases of malignant melanoma (n = 32) showed virtually absent break-apart signals in the EWS gene (less than 4% cells per case). FISH detects EWS gene rearrangement in a substantial proportion of clear cell sarcomas, with excellent specificity. Importantly, EWS FISH is negative in malignant melanoma, a clinically dissimilar tumor, which may closely mimic clear cell sarcoma histologically and immunohistochemically. As the studied probe can be utilized in routinely processed tissue, FISH provides an excellent alternative to reverse-transcriptase polymerase chain reaction in cases where fresh tissue is unavailable.

Diagnosis, Differential↗
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↗
PubMed2006

Mismatch repair protein expression and microsatellite instability: a comparison of clear cell sarcoma of soft parts and metastatic melanoma.

Clear cell sarcoma of soft parts is a rare soft tissue malignancy that shows phenotypic overlap with cutaneous melanoma but can be distinguished by the presence of a t(12;22) translocation. Microsatellite instability (MSI), a variation in the lengths of short repeat DNA segments in the genome, has been implicated in melanoma tumorigenesis, but is rare or absent in clear cell sarcoma. Defects in the mismatch repair (MMR) enzyme complex correlate with MSI in some tumor types, allowing the use of immunohistochemistry for the MMR proteins hMLH1 and hMSH2 to predict the presence of MSI. To determine if the association between MMR defects and MSI extends to clear cell sarcoma, we compared a group of nine clear cell sarcomas to 11 metastatic melanomas on the basis of MSI and the expression of MMR proteins. MSI was studied using fluorescence-based multiplexed PCR of five loci. Immunohistochemistry was evaluated on formalin-fixed paraffin-embedded tissue for hMLH1, hMHS2 and hMSH6. MSI was present in only 1/9 (11%) clear cell sarcoma case and in 8/11 (73%) melanoma cases. 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%). hMSH6 was detected in 7/9 (78%) clear cell sarcomas and 10/11 (91%) of melanomas. Clear cell sarcoma and metastatic melanoma differed significantly with respect to the presence of MSI (P=0.010) and staining for hMLH1 and/or hMSH2 (P=0.014) but not hMSH6 (P=0.57). Mismatch repair, and consequently genomic instability may contribute to tumorigenesis in melanoma but not clear cell sarcoma. Immunostaining for hMLH1 and hMSH2 and MSI analysis may be helpful in the differential diagnosis of large soft tissue or visceral malignancies with melanocytic differentiation.

Adaptor Proteins, Signal Transducing↗
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↗