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. 

This article about a neoplasm is a stub. You can help Wikipedia by expanding it.v · Categories: Neoplasm stubs Melanoma Wikimedia Foundation. 2010. Игры ⚽ Поможем написать реферат Clear-cell ovarian carcinoma Clear-cell squamous-cell carcinoma Look at other dictionaries: Clear-cell sarcoma of the kidney — Classification and external resources ICD O: 8964/3 Clear cell sarcoma of the kidney is a rare type of kidney cancer. Clear cell sarcoma can spread from the kidney to other organs, most commonly the bone, but also including the lungs, brain, and… …   Wikipedia clear cell sarcoma of the kidney — A rare type of kidney cancer. Clear cell sarcoma can spread from the kidney to other organs, most commonly the bone, but also including the lungs, brain, and soft tissues of the body …   English dictionary of cancer terms clear cell sarcoma of kidney — a malignant kidney tumor similar to Wilms tumor but with a poorer prognosis; its cells have pale or clear cytoplasm and are arranged uniformly in vascularized nests. It often metastasizes to bone …   Medical dictionary Clear-cell tumor — can refer to: clear cell sarcoma (including clear cell sarcoma of the kidney) clear cell adenocarcinoma This disambiguation page lists articles associated with the same title. If an …   Wikipedia Clear cell adenocarcinoma — Classification and external resources Micrograph of an ovarian clear cell adenocarinoma. H E stain …   Wikipedia Clear cell adenocarcinoma of the vagina — Classification and external resources ICD O: 8310/3 DiseasesDB 2786 MeSH …   Wikipedia Mast cell sarcoma — Classification and external resources ICD 10 C96.2 ICD 9 202.6 …   Wikipedia Clear-cell ovarian carcinoma — Micrograph of an ovarian clear cell carcinoma. H E stain. Clear cell ovarian tumors are part of the surface epithelial stromal tumor group of ovarian cancers, accounting for 6% of these cancers. Clear cell tumors are also associated with the… …   Wikipedia Sarcoma — Classification and external resources ICD O: M8800/3 MeSH D012509 A sarcoma (from the Greek sarx …   Wikipedia Uterine clear cell carcinoma — Classification and external resources ICD 10 C54.1 ICD 9 182 Uterine clear cell carcinom …   Wikipedia 18+ © Academic, 2000-2024 Contact us: Technical Support, Advertising Dictionaries export, created on PHP, Joomla, Drupal, WordPress, MODx. Mark and share Search through all dictionaries Translate… Search Internet Share the article and excerpts Direct link … Do a right-click on the link above and select “Copy Link”