| Clear-cell sarcoma | |
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| Classification and external resources | |
Clear cell sarcoma. Tumor cells with prominent nucleoli and clear cytoplasm are arranged in well-defined nests surrounded by dense fibrous stroma. |
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| ICD-O: | 9044/3 |
| MeSH | D018227 |
Clear-cell sarcoma (also known as "Soft-tissue melanoma," and "Melanoma of the soft parts"[1]:696. However, these older names are misnomers, as clear cell sarcoma is biologically distinct from melanoma.) is a rare form of cancer.[2] It is a type of sarcoma.[3]
Recurrence is common.[4]
It has been associated with a EWSR1-ATF1 fusion gene.[5]
Clear cell sarcoma of the soft tissues in adults is not related to the pediatric tumor known as clear cell sarcoma of the kidney.[6]
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Under a microscope these tumors show some similarities to traditional skin melanomas, [7] and are characterized by solid nests and fascicles of tumor cells with clear cytoplasm and prominent nucleoli. The clear cell sarcoma has a uniform and distinctive morphological pattern which serves to distinguish it from other types of sarcoma.
Imaging studies such as X-rays, computed tomography scans, or MRI may be required to diagnose clear-cell sarcoma together with a physical exam. Normally a biopsy is also necessary. Furthermore, a chest CT, a bone scan and positron emission tomography (PET) may be part of the tests in order to evaluate areas where metastasizes occurs. [8]
It presents as a slow growing mass that especially affects tendons and aponeuroses and it is deeply situated. Patients often perceive it as a lump or hard mass. It causes either pain or tenderness but only until it becomes large enough. This kind of tumor is commonly found in the extremities especially around the knee, feet and ankle. It affects people of all ages, including young adults and even children. [9]
When the tumor is large and there is presence of necrosis and local recurrence, the prognosis is poor. Presence of metastasis occurs in more than 50% cases and the common places of its occurrence are the bone, lymphnode and lungs. [10]
Treatment depends upon the site and the extent of the disease. Clear cell sarcoma is usually treated with surgery in the first place in order to remove the tumor. The surgical procedure is then followed by radiation and chemotherapy. [11]
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