1/82. Clear cell sarcoma of soft parts: report of a case primary in the kidney with cytogenetic confirmation.Clear cell sarcoma of soft parts (CCSSP), also known as malignant melanoma of soft parts, is an aggressive tumor that usually presents in soft tissue and very rarely in small bowel. We report a case arising in the kidney of a 20-year-old man which was difficult to distinguish from Wilms' tumor. The tumor metastasized to the liver and lungs, and the patient died of disseminated disease 5 years after his initial presentation. Both the primary and metastatic tumors were composed predominantly of spindle cells with occasional more epithelioid areas that were inconsistently arranged in nests. In both primary and metastatic sites, the tumor surrounded and entrapped normal epithelial elements, mimicking the biphasic appearance of Wilms' tumor. The tumor cells, however, were positive for S-100 protein and HMB45 and negative for keratin and CD99, and cytogenetic analysis revealed a clonal abnormality, translocation t(12;22)(q13;q12), characteristic of CCSSP. This result was verified by fluorescence in situ hybridization on paraffin-embedded tissue, which demonstrated EWS gene-region rearrangement. CCSSP joins a growing list of tumors that typically arise in soft tissue (PNET, solitary fibrous tumor, and infantile/congenital fibrosarcoma), but can also present in the kidney and may be confused with primary renal tumors. awareness of this possibility and the use of ancillary studies. including immunohistochemistry, cytogenetic analysis, and fluorescence in situ hybridization, are important for accurate diagnosis.- - - - - - - - - - ranking = 1keywords = sarcoma (Clic here for more details about this article) |
2/82. Primary clear cell sarcoma of bone: a unique site of origin.Clear cell sarcoma is a rare soft tissue neoplasm, accounting for less than 1% of soft tissue sarcomas. We are presenting a case of a clear cell sarcoma of bone which, to our - knowledge, is the only report of a , primary clear cell sarcoma of bone.- - - - - - - - - - ranking = 1.3333333333333keywords = sarcoma (Clic here for more details about this article) |
3/82. Melanotic peritoneal sarcomatosis originating from clear cell sarcoma.Peritoneal sarcomatosis was found in a 53-year-old male who had a history of resection of clear cell sarcoma (CCS) of the right wrist 7 years previously. Both the previous wrist tumor and the peritoneal disseminants consisted of small, spindle-shaped cells occasionally containing melanophages. Histologic features, histochemical demonstration of argentaffin granules, immunohistochemical reaction with HMB 45, and the demonstration of a chimeric transcript of EWS-ATF-1 established the diagnosis of CCS in the peritoneal tumors. As far as we are aware, this is the first case of a peritoneal sarcomatosis associated with CCS.- - - - - - - - - - ranking = 1.8333333333333keywords = sarcoma (Clic here for more details about this article) |
4/82. Clear cell sarcoma of the abdominal wall with peritoneal sarcomatosis: CT features.Clear cell sarcoma, also called malignant melanoma of soft parts, is an uncommon neoplasm that involves tendons or aponeuroses of the lower extremity. The CT features of a clear cell sarcoma arising from the abdominal wall with later peritoneal dissemination are described. Peritoneal sarcomatosis from soft tissue sarcomas is a very rare condition previously unreported in the radiologic literature. Metastases to peritoneal surfaces must therefore be considered a possible site for systemic dissemination of soft tissue sarcomas.- - - - - - - - - - ranking = 2.1666666666667keywords = sarcoma (Clic here for more details about this article) |
5/82. 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.Clear cell sarcoma of the kidney is a distinct, highly malignant pediatric neoplasm. Its occurrence in adults is extremely rare and the subject of isolated case reports. We present a series of four cases (three males and one female) identified in an adolescent and in young adults (16, 18, 20, and 25 years) with flank mass (three cases), hematuria (two cases), flank pain (two cases), and hypertension (one case). Three patients had stage III disease and one had stage I disease (National Wilms' Tumor Study staging system). All tumors had predominantly or exclusively the classic histology of a monotonous proliferation of uniform small round cells with evenly distributed fine chromatin, although focal microcyst formation (two cases) and spindled architecture (one case) (variant patterns) were also noted. Therapy in all cases consisted of surgery and chemotherapy with or without radiation. Follow-up data (29-202 months) showed distant metastases in all four cases, including the lung (four cases), bone (two cases), and the liver (two cases). Three patients died of disease at 29, 59, and 63 months (mean, 50.3 months), and one patient is alive with no evidence of disease at 202 months. Ultrastructural features included scattered primitive junctions, short and irregular cytoplasmic extensions, and scant to a moderate amount of mitochondria. Immunohistochemical study (three cases) showed immunoreactivity with vimentin (two cases) and no reaction with cytokeratin, epithelial membrane antigen, S-100 protein, or desmin. Flow cytometric analysis showed diploid dna content in three primary tumors and tetraploidy in one metastatic tumor. The proliferative activity (S-phase fraction) was low to intermediate (mean, 9.8%). Our data suggest that clear cell sarcoma of the kidney in the young adult age group resembles its pediatric counterpart in ultrastructural and immunohistochemical characteristics, proclivity for skeletal and visceral metastasis, dna diploid status with relatively low S-phase, and aggressive clinical course. Clear cell sarcoma of the kidney in adult patients, although rare, must be differentiated from sarcomatoid carcinoma, sarcomas, and round cell tumors because of its unique characteristics in comparison to other renal neoplasms.- - - - - - - - - - ranking = 1.5keywords = sarcoma (Clic here for more details about this article) |
6/82. Tyrosinase gene expression in clear cell sarcoma indicates a melanocytic origin: insight from the first reported canine case.The aim of this study was to characterize a metastasizing soft tissue tumor in a dog, which clinically, grossly and histologically showed a close resemblance to human clear cell sarcoma, a soft tissue variant of malignant melanoma. Ultrastructurally, melanosomes were found, indicating a melanocytic origin of the tumor. Using reverse-transcription polymerase chain reaction, expression of the gene encoding tyrosinase was determined in tumor cells. With this first case of canine clear cell sarcoma, as well as the earlier report from our laboratory on amelanotic melanomas in the cat, we demonstrate that expression of the tyrosinase gene may occur in a broader range of less differentiated melanocytic tumors in different species, including man.- - - - - - - - - - ranking = 1keywords = sarcoma (Clic here for more details about this article) |
7/82. Triple central ray amputation for clear cell sarcoma of the hand.A 16-year-old girl presented for evaluation and management of a recurrent soft-tissue mass in her left hand after excision at another facility. She was given a diagnosis of clear cell sarcoma. She chose a limb salvage procedure over amputation. A triple central ray amputation of her index, middle, and ring fingers was performed. After 3 years, she has reasonable grasp function with her remaining fingers and has chosen not to use a prosthesis. Long-term follow-up is necessary for this tumor, as local recurrence and metastatic disease have been reported frequently.- - - - - - - - - - ranking = 0.83333333333333keywords = sarcoma (Clic here for more details about this article) |
8/82. 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.- - - - - - - - - - ranking = 1keywords = sarcoma (Clic here for more details about this article) |
9/82. Clear cell sarcoma arising in the transverse colon.A case of clear cell sarcoma (CCS) arising in the transverse colon is presented. The tumor consisted of sheets or small nests of epithelioid malignant cells possessing pleomorphic nuclei with one or more prominent nucleoli and ample clear or slightly eosinophilic cytoplasm. Some of the tumor cells contained various amounts of melanin pigments that were confirmed by histochemical and ultrastructural examinations. Immunohistochemical examination showed a positive immunoreactivity for HMB45 and S-100 protein. A metastatic nodule, which was found 9 months after surgery, showed similar histological findings to those of the primary one but lacked melanin pigments. Reverse transcriptase- polymerase chain reaction using total ribonucleic acid obtained from metastatic nodule demonstrated the presence of EWS-ATF-1 fusion gene. Based on these findings, the present case tumor is a CCS of the colon.- - - - - - - - - - ranking = 0.83333333333333keywords = sarcoma (Clic here for more details about this article) |
10/82. Ovarian sarcoma with pathologic features of clear cell sarcoma of the kidney.We report a case of an unusual sarcoma arising in the ovary of an infant girl. Histologically, the tumor was composed of clear, undifferentiated cells set in an arborizing vascular stroma. Immunohistochemical staining was positive only for vimentin. Ultrastructural evaluation demonstrated undifferentiated cells with interdigitating broad cell processes that encompassed irregular electron lucent spaces that contained flocculent extracellular material. light and electron microscopic features of the tumor resembled a clear cell sarcoma of the kidney. Although the cell of origin is unproven, both tumors may arise from primitive mesenchymal cells that may not be restricted to the kidney.- - - - - - - - - - ranking = 1.6666666666667keywords = sarcoma (Clic here for more details about this article) |
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