Clear cell sarcoma
| Clear cell sarcoma | |
|---|---|
| Other names | Malignant melanoma of the soft parts |
 | |
| Clear cell sarcoma. Tumor cells with prominent nucleoli and clear cytoplasm are arranged in well-defined nests surrounded by dense fibrous stroma. | |
| Specialty | Oncology |
Clear cell sarcomais a sub-type of a rare form ofcancercalled asarcoma.[1]It is known to occur mainly in thesoft tissuesanddermis.Rare forms were thought to occur in the gastrointestinal tract before they were discovered to be different and redesignated asgastrointestinal neuroectodermal tumors.
Recurrence is common.[2]
Clear cell sarcoma's neoplastic cells express theEWSR1-ATF1fusion gene in a majority of cases or aEWSR1-CREB1,EWSR1-CREM,[3]orEWSR1-DDIT3[4]fusion gene in a small subset of cases (seeFET gene familyof fusion genes).
Clear cell sarcoma of the soft tissues in adults is not related to the pediatric tumor known asclear cell sarcoma of the kidney.[5]
Signs and symptoms
[edit]It presents as a slow growing mass that especially affectstendonsandaponeurosesand it is deeply situated. Patients often perceive it as alumpor 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 theknee,feet andankle.Patients diagnosed with clear cell sarcoma are usually between the ages of 20 and 40.[1]
Pathology
[edit]Despite the name clear cell sarcoma, the tumor cells do not necessarily need to have clearcytoplasm.The lesion has a distinctly nested growth pattern with a mixture ofspindle,epithelioidandtumor giant cells.Approximately two thirds of the tumors containmelaninpigment. Clear cell sarcoma, similar tomelanoma,has consistent positivity forS-100,HMB-45,andMITF.[6]
Diagnosis
[edit]Imaging studies such asX-rays,computed tomography (CT) scans,orMRImay be required to diagnose clear-cell sarcoma together with aphysical exam.Normally abiopsyis also necessary. Furthermore, a chest CT, a bone scan, andpositron emission tomography(PET) may be part of the tests in order to evaluate areas where metastases occur.[7]
Treatment
[edit]Treatment depends upon the site and the extent of the disease. Clear cell sarcoma is usually treated withsurgeryin the first place in order to remove the tumor. The surgical procedure is then followed byradiationand sometimeschemotherapy.[8]Few cases of clear cell sarcoma respond tochemotherapy.[1]Several types oftargeted therapythat may be of benefit to people with clear cell sarcoma are currently under investigation.[1]
Prognosis
[edit]When the tumor is large and there is presence ofnecrosisand local recurrence, the prognosis is poor. Presence of metastasis occurs in more than 50% cases and the common places of its occurrence are thebone,lymph nodeandlungs.[9][10]Five-year survival rates,which are reported to be between 50 and 65%, can be misleading because the disease is prone to late metastasis or recurrence. Ten and twenty-year survival rates are 33% and 10%, respectively.[1]
See also
[edit]- Melanoma
- Clear-cell sarcoma of the kidney
- List of cutaneous conditions
- Hyalinizing clear cell carcinoma- has the same chromosomal translocation
References
[edit]- ^abcdeBarry, G; Nielsen, T (June 2012)."Clear Cell Sarcoma of Soft Tissue".ESUN.9(3).Retrieved15 June2012.
- ^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.PMID17192915.S2CID27122422.
- ^Argani P, Harvey I, Nielsen GP, Takano A, Suurmeijer AJ, Voltaggio L, Zhang L, Sung YS, Stenzinger A, Mechtersheimer G, Dickson BC, Antonescu CR (November 2020)."EWSR1/FUS-CREB fusions define a distinctive malignant epithelioid neoplasm with predilection for mesothelial-lined cavities".Modern Pathology.33(11): 2233–2243.doi:10.1038/s41379-020-0646-5.PMC7584759.PMID32770123.
- ^Flucke U, van Noesel MM, Siozopoulou V, Creytens D, Tops BB, van Gorp JM, Hiemcke-Jiwa LS (June 2021)."EWSR1-The Most Common Rearranged Gene in Soft Tissue Lesions, Which Also Occurs in Different Bone Lesions: An Updated Review".Diagnostics.11(6): 1093.doi:10.3390/diagnostics11061093.PMC8232650.PMID34203801.
- ^Poppe B, Forsyth R, Dhaene K, Speleman F.Soft tissue tumors: Clear cell sarcoma.Atlas Genet Cytogenet Oncol Haematol. November 2002.
- ^"Clear cell sarcoma".Pathology Outlines.Retrieved26 May2015.
- ^"Diagnosis".Mayo Clinic.Retrieved2010-06-29.
- ^"Treatment".Mayo Clinic.Retrieved2010-06-29.
- ^"Clear Cell Sarcoma of Tendons and Aponeuroses".Retrieved2010-06-29.
- ^Alhatem, Albert (2020)."Primary Cutaneous Clear Cell Sarcoma, Clinical Outcome With Sentinel Lymph Nodes Status".Am J Clin Pathol.153(5): 799–810.doi:10.1093/ajcp/aqaa009.PMID32157275.