Clear-cell sarcoma

Clear-cell sarcoma
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.
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]

Pathology

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.

Diagnosis

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]

Signs and symptoms

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]

Prognosis

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

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]

See also

• Melanoma
• Clear-cell sarcoma of the kidney
• List of cutaneous conditions

References

1. ^ James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN 0-7216-2921-0. 
2. ^ Xu GG, Chong YL, Cheong MO (July 2007). "Clear cell sarcoma of the rectus sheath". Singapore Med J 48 (7): e203–5. PMID 17609816. http://smj.sma.org.sg/4807/4807cr7.pdf. 
3. ^ Kawai A, Hosono A, Nakayama R, et al. (January 2007). "Clear cell sarcoma of tendons and aponeuroses: a study of 75 patients". Cancer 109 (1): 109–16. doi:10.1002/cncr.22380. PMID 17133413. 
4. ^ Malchau SS, Hayden J, Hornicek F, Mankin HJ (May 2007). "Clear cell sarcoma of soft tissues". J Surg Oncol 95 (6): 519–22. doi:10.1002/jso.20730. PMID 17192915. 
5. ^ Hisaoka M, Ishida T, Kuo TT, et al. (March 2008). "Clear cell sarcoma of soft tissue: a clinicopathologic, immunohistochemical, and molecular analysis of 33 cases". Am. J. Surg. Pathol. 32 (3): 452–60. doi:10.1097/PAS.0b013e31814b18fb. PMID 18300804. 
6. ^ Poppe B, Forsyth R, Dhaene K, Speleman F. Soft tissue tumors: Clear cell sarcoma. Atlas Genet Cytogenet Oncol Haematol. November 2002.
7. ^ "Melanoma Skin Cancer". http://www.tanningmymelanoma.com/melanomaskin/. Retrieved 2010-06-29. 
8. ^ "Diagnosis". http://www.mayoclinic.org/soft-tissue-sarcoma/diagnosis.html. Retrieved 2010-06-29. 
9. ^ "Research Notes on Clear Cell Sarcoma". http://organizedwisdom.com/Clear_Cell_Sarcoma. Retrieved 2010-06-29. 
10. ^ "Clear Cell Sarcoma of Tendons and Aponeuroses". http://www.histopathology-india.net/CCS.htm. Retrieved 2010-06-29. 
11. ^ "Treatment". http://www.mayoclinic.org/soft-tissue-sarcoma/treatment.html. Retrieved 2010-06-29. 

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