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Clear-Cell Sarcoma 

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  1. Open Access 01-12-2024 | Clear-Cell Sarcoma | OriginalPaper

    Navigating diagnostic challenges—distinguishing malignant melanoma and clear cell sarcoma of soft tissues: a case report and review of the literature

    In the context of encountering a melanocytic differentiated tumor, pathologists are confronted with the imperative task of discerning potential diagnoses, with clear cell sarcoma of soft tissues (CCSST) being among the considerations related to …

  2. 01-12-2024 | Clear-Cell Sarcoma | ReviewPaper

    A Comparison of Clear Cell Sarcoma to Jaw and Salivary Tumors Bearing EWS Fusions

    Rare tumors are typically difficult to study due to the limited availability of tissue, both preserved and fresh. As such, it is critical that researchers working on rare tumors communicate and disseminate any information broadly. World-wide …

  3. Open Access 01-12-2024 | Nephroblastoma | OriginalPaper

    CT radiomics to differentiate between Wilms tumor and clear cell sarcoma of the kidney in children

    Clear cell sarcoma of the kidney (CCSK) is a rare renal malignancy, accounting for approximately 2–5% of pediatric renal tumors, ranking second in prevalence after Wilms tumor (WT). Typically, CCSK manifests between the ages of 2 and 4 years [ 1 …

  4. Open Access 01-12-2023 | Metastasis | OriginalPaper

    Targeting epigenetic features in clear cell sarcomas based on patient-derived cell lines

    Clear cell sarcomas (CCSs) are an extremely rare type of soft-tissue saromas arising from cells of the neural crest and occurring primarily in young adults. Patients with CCSs report a palpable tumor mass for months or years. Tumors larger than 5 …

  5. 16-09-2023 | Clear-Cell Sarcoma | BriefCommunication

    Melanin-targeted [18F]-PFPN PET imaging may shed light for clear cell sarcoma

    To the best of our knowledge, this is the first investigation into the feasibility of melanin-targeted imaging in patients with CCS. We report [ 18 F]-PFPN PET imaging in three cases with pathologically confirmed CCS. A total of 56 lesions were …

  6. Open Access 01-12-2022 | Nephrectomy | OriginalPaper

    Clear cell sarcoma of the kidney with inferior vena cava thrombus: a case report

    Clear cell sarcoma of the kidney (CCSK) is an uncommon pediatric renal malignant neoplasm (representing approximately 2.8% of all primary renal tumors in children) [ 1 ]. CCSK is most common in children between 2 and 3 years old, with a slight …

  7. Open Access 29-04-2023 | Kidney Cancer | OriginalPaper

    STING is a prognostic factor related to tumor necrosis, sarcomatoid dedifferentiation, and distant metastasis in clear cell renal cell carcinoma

    STING is a molecule involved in immune reactions against double-stranded DNA fragments, released in infective and neoplastic diseases, whose role in the interactions between immune and neoplastic cells in clear cell renal cell carcinoma has not …

  8. Open Access 01-12-2019 | Clear-Cell Sarcoma | OriginalPaper

    Clear cell sarcoma of the kidney in a 62-year-old patient presenting with generalized pruritus

    Clear cell sarcoma of the kidney (CCSK) is the second most common renal tumor in children following Wilms’ tumor. The average age at diagnosis of CCSK is 3 years old. The tumor is prone to metastasize to bone, brain, and soft tissue [ 1 ]. The …

  9. Open Access 01-12-2019 | Clear-Cell Sarcoma | OriginalPaper

    Prognostic analysis of surgically treated clear cell sarcoma: an analysis of a rare tumor from a single center

    Clear cell sarcoma (CCS), which was first described by Enzinger in 1965 [ 1 ], is a rare malignant tumor mainly involving tendons and aponeuroses in young adults. CCS is often misdiagnosed as malignant melanoma due to similar clinical and …

  10. 01-02-2022 | Metastasis

    Clinical, pathological, and genetic profile of clear cell sarcoma-like tumour of jejunum: report of a rare aggressive tumour of small bowel

    Clear cell sarcoma-like tumour of the gastrointestinal tract (CCSLGT) is a rare entity which has been recently described as late as 2003. Only around 70 cases have been reported in the English literature till date. CCSLGT is mostly seen in young …

  11. 01-10-2021 | Fluorescence in Situ Hybridization | BriefCommunication

    Clear cell tumor with melanocytic differentiation and MITF-CREM translocation: a novel entity similar to clear cell sarcoma

    The presence of melanocytic differentiation in tumors of non-melanocyte origin is uncommon and is typically associated with the overexpression of MITF, the master regulator of melanin synthesis, or another member of the MIT/TFE3 family. In clear

  12. 01-12-2021 | Computed Tomography

    Primary clear cell sarcoma of the femur: a unique case with RT-PCR and direct sequencing confirmation of EWSR1/ATF1 fusion gene

    Clear cell sarcoma (CCS) was first described by Enzinger in 1965 [ 1 ]. It is a malignant soft tissue tumor arising from tendons and aponeuroses. CCS has limited treatment options because effective radiotherapy and chemotherapy regimens have not …

  13. 01-06-2021 | Clear-Cell Sarcoma | BriefCommunication

    Clear cell sarcoma-like/malignant gastrointestinal neuroectodermal tumor of the tongue: a clinicopathologic and molecular case report

    Malignant gastrointestinal neuroectodermal tumor (M-GNET) and clear cell sarcoma (CCS) of soft tissue represent closely related, extremely rare, malignant mesenchymal neoplasm of uncertain differentiation. Both entities are characterized …

  14. 01-02-2021 | osteosarcoma | BriefCommunication

    Mixed Chromophobe and Clear Cell Renal Cell Carcinoma with Heterologous Sarcomatoid Differentiation—Radiological Pathological Correlation

    Renal cell carcinoma (RCC) is the most common malignancy of the kidney and has multiple subtypes. The sarcomatoid variety is considered a form of dedifferentiated carcinoma, and when present, it is associated with significant decrease in patient …

  15. 01-09-2020 | Clear-Cell Sarcoma | OriginalPaper

    Clear Cell Sarcoma of Sigmoid Colon: a Rare Malignancy with Common Clinical Manifestations

    The most common variety of colonic malignancy is adenocarcinoma, which accounts for 95% of all malignant neoplasms. Mesenchymal neoplasm consists of 0.5–3.0% of all colonic tumors, with gastrointestinal stromal tumor (GIST) being the commonest …

  16. 01-09-2019 | OriginalPaper

    Clear Cell Sarcoma-Like Tumor of the Gastrointestinal Tract

    Clear cell sarcoma-like tumor of the gastrointestinal tract (CCSLGT) is a relatively recently described neoplasm usually arising from wall of the small intestine [ 1 – 6 ]. Because of the rarity of CCSLGT as well as its overlapping histomorphologic …

  17. Open Access 01-12-2019 | Clear-Cell Sarcoma | OriginalPaper

    Expression of cyclin D1 in clear cell sarcoma of kidney. Is it useful in differentiating it from its histological mimics?

    Clear cell sarcoma of kidney (CCSK) is an uncommon mesenchymal renal tumor of uncertain histogenesis which comprises approximately 3 to 4% of malignant pediatric renal neoplasms. It occurs in young children and mean age at diagnosis is …

  18. Open Access 01-12-2018 | OriginalPaper

    Detection of specific gene rearrangements by fluorescence in situ hybridization in 16 cases of clear cell sarcoma of soft tissue and 6 cases of clear cell sarcoma-like gastrointestinal tumor

    Clear cell sarcoma of soft tissue (CCSST) is a malignant mesenchymal tumor that mostly affects young adults and tends to affect the lower extremities, close to the tendon and aponeuroses [ 1 ]. Histologically, CCSSTs have epithelioid tumor nests …

  19. 01-09-2018 | OriginalPaper

    The epidemiology and survivorship of clear cell sarcoma: a National Cancer Database (NCDB) review

    Clear cell sarcoma (CCS) is a rare melanocytic soft tissue tumor involving tendons and aponeuroses, first characterized by Enzinger ( 1965 ). The true incidence is unknown; however, it is estimated that CCS accounts for approximately 1% of soft …

  20. Open Access 01-12-2017 | OriginalPaper

    Complete response of mediastinal clear cell sarcoma to pembrolizumab with radiotherapy

    Clear cell sarcoma, originally called “malignant melanoma of soft parts” due to its melanocytic differentiation, is a rare, aggressive soft tissue sarcoma usually arising in the lower extremities at 20–40 years of age with only three mediastinal …

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