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Soft Tissue Sarcomas
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2020
Thomas F. DeLaney, David C. Harmon, Karol Sikora, Francis J. Hornicek
Clear cell sarcoma has similarities to malignant melanoma, although cytogenetically the tumors are distinct. Clear cell sarcomas often exhibit a translocation at t(12;22)(q13–14;q12), which is not seen in malignant melanoma. Trisomy of chromosome 8 is also observed in clear cell sarcoma. Alveolar soft part sarcoma is another tumor that also exhibits a specific chromosomal translocation t(X;17) (p11;q25), which creates the fusion product TFE–ASPL.
Sarcomas
Published in E. George Elias, CRC Handbook of Surgical Oncology, 2020
Clear cell sarcoma of tendons and aponeurosis — is a rare tumor that occurs in young adults. It is usually located in the distal portion of the extremities. They are slow growing but persistent tumors that have a high tendency for local recurrences if not radically excised by amputation.
Skin and soft tissue
Published in Tor Wo Chiu, Stone’s Plastic Surgery Facts, 2018
Predictor of lymph node recurrence Histopathological tumour type – epithelioid sarcoma, RMS and clear cell sarcoma
Malignant Perivascular Epitheloid Cell Tumor with an Unusual Immunophenotype in a Ten-Year-Old Child
Published in Fetal and Pediatric Pathology, 2019
Nives Jonjic, Jelena Roganovic, Nedeljka Glavan, Ita Hadzisejdic, Irena Seili-Bekafigo
The major differential diagnosis in the current case includes clear cell sarcoma since it occurs mainly in the soft tissues and dermis, predominantly in adolescents and young adults, and histologically is characterized by nested growth pattern and a mixture of spindled, epitheloid cells. However, clear cell sarcoma shows consistent positivity for S-100, HMB-45, and MITF, and negativity for myoid markers. Alveolar soft part sarcoma, characterized by an organoid pattern and a sinusoidal-type vasculature, as well as melanoma, may be confused with PEComa. Alveolar soft part sarcoma is negative for specific melanocytic markers while melanoma, similar to clear cell sarcoma, shows positivity for S-100, HMB-45 and MITF. Therefore, S-100 negativity in PECOMA can be useful in the differential diagnosis of malignant melanoma, even though up to 11% of PEComas express S-100 as well [7]. For the diagnosis of PEComa it is very important to include the negative history for melanoma, perivascular arrangement of tumor cells, immunoreactivity for myoid markers and absence of specific EWSR1/ATF1 fusion transcript resulting from t(12;22) (q13;q12) in clear cell sarcoma.
The place of trabectedin in the treatment of soft tissue sarcoma: an umbrella review of the level one evidence
Published in Expert Opinion on Orphan Drugs, 2019
Pavlina Andreeva-Gateva, Shenol Chakar
Most studies on metastatic STS chemotherapy are hindered by the mixing of different histological types in the analysis of the results, making it difficult to assess the clinical activity of any treatment according to histology. These data have led to the concept of histologically guided treatment [57] instead of using a uniform treatment for all STS. For example, synovial sarcomas and myxoid/round cell liposarcomas are some of the most sensitive to chemotherapy when using a specific agent [58]. In particular, the myxoid/round cell liposarcomas are sensitive to doxorubicin-based chemotherapy, and synovial sarcomas are sensitive to alkylating agents such as ifosfamide [59]. Conversely, other types such as clear cell sarcoma and fibromyxoid sarcoma have a weaker response to conventional therapy with an anthracycline and ifosfamide-based chemotherapy. It is known that gastrointestinal stromal tumors (GIST) are highly resistant to the standard cytotoxic agents [60].
Metastatic TFE3-overexpressing renal clear cell carcinoma with dense granules: a histological, immunohistochemical, and ultrastructural study
Published in Ultrastructural Pathology, 2018
Shoujun Chen, Elba A. Turbat-Herrera, Guillermo A. Herrera, Meghna Chadha, Rodney E. Shackelford, Eric X. Wei
Clear cell RCC is the most common histologic variant of RCC. The clear cytoplasm is created by the rich lipid and glycogen content due to the deficient glycogenolysis and lipolysis.5 The lipid content is comparable to that of the proximal convoluted tubules,6 which is considered the cell origin or line of differentiation of the clear cell RCC variant. Accordingly, the tumor cells of the RCC exhibit tubular differentiation with tumor cells arranged around microlumens demarcated by basal lamina. Tumor cells often harbor abundant lipid vacuoles and mono-particulate glycogen, characteristic features as in this reported case. Adult clear cell sarcoma of the kidney may be suggested based on morphological and immunostaining patterns. However, in the clear cell sarcoma of the kidney, the electron microscopy normally demonstrates that the “clear” cell appearance is due to the extracellular matrix instead of cytoplasmic glycogen or droplets as shown in this case.