Cases reported "Sarcoma, Clear Cell"

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1/29. 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.
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ranking = 1
keywords = malignant melanoma, melanoma
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2/29. 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.
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ranking = 1
keywords = malignant melanoma, melanoma
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3/29. 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.
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ranking = 1.3119491960894
keywords = malignant melanoma, melanoma
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4/29. Clear cell variant of malignant melanoma of the uterine cervix: a case report and review of the literature.

    BACKGROUND: A rare variant of malignant melanoma of the uterine cervix mimicking clear cell carcinoma or clear cell sarcoma is described. CASE: A 33-year-old Japanese woman was admitted to the hospital complaining of genital discharge and lower back pain. The stage was FIGO IIB and radical hysterectomy and pelvic lymphadenectomy were done. Pathological examination, immunohistochemical studies of melanin granules, and molecular analysis of the EWS/ATF-1 fusion gene were also done. A diffuse proliferation of amelanotic clear cells was detected in the uterine cervix. Tumor cells were positive for HMB 45, Melan-A (MART-1), and S-100 protein and negative for epithelial markers. The EWS/ATF-1 fusion gene was not detected. CONCLUSION: This is apparently the first report of a case of clear cell melanoma of the uterine cervix. Despite its rarity, this variant of malignant melanoma should be considered when diagnosing clear cell neoplasms of the uterine cervix.
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ranking = 6.3119491960894
keywords = malignant melanoma, melanoma
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5/29. Malignant melanoma of soft tissues (clear cell sarcoma) of the foot. Is MRI able to perform a specific diagnosis? Report of one case and review of the radiological literature.

    The magnetic resonance (MR) findings in malignant melanoma of soft tissues, also called clear cell sarcoma of tendons and aponeuroses, have been described as a focal abnormality with a specific MR pattern of increased signal intensity (relative to normal muscle) on T1 weighted sequences and variably decreased signal intensity on T2 weighted sequences (relative to surrounding fat). We have reported here a case of malignant melanoma of soft tissues of the foot, studied with ultrasonography (US) and MR, in which MR showed T1-hypointensity, T2-hyperintensity and marked gadolinium uptake. We have described the relationship between the intracytoplasmic melanin amount of and these atypical MR findings.
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ranking = 3.2477967843575
keywords = malignant melanoma, melanoma
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6/29. Newly established clear cell sarcoma (malignant melanoma of soft parts) cell line expressing melanoma-associated Melan-A antigen and overexpressing C-MYC oncogene.

    Clear cell sarcoma (CCS), malignant melanoma of soft parts, is a rare malignant tumor with a poor prognosis. In this study, a CCS cell line, designated MP-CCS-SY, was established from a metastatic tumor of a 17-year-old Japanese girl that originated in the left achilles tendon. A small number of melanosomes were detected in the cytoplasm by electron microscopy. The melanosomes immunoreacted with two melanoma-associated antibodies, HMB45 and Melan-A. A Western blot demonstrated the existence of a Melan-A antigen in this cell line. Although a t(12;22)(q13;q12), which is characteristic of CCS, was not identified by a chromosomal analysis with conventional banding techniques, fluorescence in situ hybridization analysis with painting probes of chromosomes 12 and 22 revealed the insertion of a chromosome 12 fragment into one of the long arms of chromosome 22. The chimeric EWS/ATF1 transcript was detected by the reverse transcriptase polymerase chain reaction. Extra copies and structural abnormalities of chromosome 8 were observed. Overexpression of c-myc mRNA was detected by Northern blot analysis and may have a role in malignant progression of CCS. The availability of this MP-CCS-SY cell line will help to understand the molecular biology of this malignancy and should be useful as a tool for developing an immunotherapy.
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ranking = 6.5597459804469
keywords = malignant melanoma, melanoma
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7/29. 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.
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ranking = 1.9358475882681
keywords = malignant melanoma, melanoma
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8/29. Clear cell sarcoma (malignant melanoma of soft parts) presenting as cervical myelopathy.

    A 47-year-old woman presented with a 2 year history of progressive cervical myelopathy. MRI revealed a lesion anterior to the cord at the level of C1/C2. This was excised and found to be a clear cell sarcoma, a tumour not previously reported at this site.
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ranking = 4
keywords = malignant melanoma, melanoma
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9/29. Malignant melanoma of the soft parts showing calciphylaxis.

    calciphylaxis is a poorly understood and highly morbid syndrome of vascular calcification and skin necrosis. hypercalcemia, hyperphosphatemia, and secondary hyperparathyroidism are the factors implicated in the pathogenesis of calciphylaxis, which is generally identified in patients with hyperparathyroidism secondary to end-stage renal disease. It has also been observed in primary hyperparathyroidism, end-stage liver disease, and rheumatoid arthritis, in the absence of renal disease. There are few case reports of calciphylaxis occurring in hypercalcemia of malignancy. An unusual case is reported of calciphylaxis associated with malignant melanoma of the soft parts in the absence of renal or parathyroid disease. This is the first reported case of this soft tissue sarcoma showing calciphylactic changes.
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ranking = 2.2477967843575
keywords = malignant melanoma, melanoma
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10/29. Complete remission of metastatic clear cell sarcoma with DAV chemotherapy.

    We report the first case of metastatic clear cell sarcoma with dramatic response to DAV treatment (DTIC ACNU VCR). Clear cell sarcoma of tendons and aponeuroses, or malignant melanoma of soft parts, is a rare tumour that occurs predominantly in the extremities of young adults. It tends to recur locally or metastasize and the prognosis is poor. Although the importance of surgery has been established, the role of adjuvant chemotherapy has yet to be determined. DAV should be considered as a first-line palliative treatment in disseminated disease as well as adjuvant therapy after surgery of primary clear cell sarcoma.
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ranking = 1
keywords = malignant melanoma, melanoma
(Clic here for more details about this article)
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