Clear cell sarcoma of tendons and aponeuroses: A review

University of Missouri - Kansas City, Kansas City, Missouri, United States
Archives of pathology & laboratory medicine (Impact Factor: 2.84). 02/2007; 131(1):152-6. DOI: 10.1043/1543-2165(2007)131[152:CCSOTA]2.0.CO;2
Source: PubMed


Clear cell sarcoma of tendons and aponeuroses, also referred to as malignant melanoma of soft parts, is a rare malignancy derived from neural crest cells. It usually presents in the distal lower extremities of young adults, frequently attached to tendons or aponeuroses. It behaves like a high-grade soft tissue sarcoma and is associated with poor overall survival. Magnetic resonance imaging studies of the lesion reveal T1 hypointensity, T2 hyperintensity, and gadolinium uptake. Grossly, the tumor is usually circumscribed with a histologic pattern of uniform polygonal to fusiform cells with clear to pale eosinophilic cytoplasm divided into variably sized clusters by fibrous septa. Immunohistochemical studies in most cases show that the neoplastic cells are positive with HMB-45 and react with antibody against S100 protein. Most cases show a reciprocal cytogenetic translocation t(12;22)(q13;q12) that creates a unique chimeric fusion EWSR1/ATF1 gene transcript. Metastasis occurs mainly to regional lymph nodes and lungs. Poor prognostic indicators include a tumor size equal to or more than 5 cm, presence of metastasis, and necrosis. The mainstay of treatment is wide excision of the tumor. The use of sentinel lymph node biopsy may become an important procedure in detecting occult regional metastasis and guiding the extent of surgery. The beneficial effects of adjuvant chemotherapy and radiotherapy have not been fully evaluated. This article provides a short overview of the current knowledge of clear cell sarcoma of tendons and aponeuroses.

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    • "This experience is well described in a review of MRI utility in sarcomas by Varma et al. [23]. There are several papers that describe the importance of T2-weighted MRI in clinical sarcoma diagnosis and treatment MR as evidenced by the following 21 publications, many of which performed a radiological/pathological correlation and specifically describe the tumor boundaries and rims [1] [2] [3] [4] [5] [6] [8-22]. The boundary changes are likely caused by edema and other processes and are noted in the papers below. "

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    • "Clear cell sarcoma (CCS) is an aggressive neoplasm of uncertain histogenesis, accounting for 1% of soft tissue sarcomas. The deep soft tissues of the distal extremities are most frequently involved, often in association with tendons and aponeuroses [7]. Unlike most sarcomas, CCS has a high propensity for lymph nodes metastasis. "
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    ABSTRACT: The group of small blue round cell tumors encompasses a heterogeneous group of neoplasms characterized by primitive appearing round cells with few distinguishing histologic features. We report the case of a small blue round cell tumor with an EWS gene rearrangement detected by fluorescent in situ hybridization (FISH) analysis that mimicked Ewing sarcoma, but with unusual histology and immunohistochemical features. Multi-color karyotyping identified the presence of a t(2;22)(q34;q12) that was initially expected to represent a variant EWSR1-FEV translocation. After an extensive workup, the lesion is considered to represent a clear cell sarcoma harboring an EWSR1-CREB1 fusion transcript. This case appears to represent a rare variant of clear cell sarcoma arising in peripheral soft tissues with unusual histology and unique immunophenotype. In this circumstance, FISH for all EWSR1 translocation partners or RT- PCR for a spectrum of possible transcript variants is critically important for diagnosis, since cytogenetic analysis or clinical FISH assay using only commercial EWSR1 probes will be misleading.
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