9044/3 Clear cell sarcoma, NOS



Definitions

Connective, subcutaneous and other soft tissues
ICD-O-3 topography code: C49
ICD10: C49
           

A rare malignant neoplasm with melanocytic differentiation characterized by the presence of polygonal or spindle shaped clear cells. This sarcoma usually affects the tendons and aponeuroses and is associated with a poor prognosis due to recurrences and metastases.



Kidney
ICD-O-3 topography code: C64
ICD10: C64
           

A rare malignant neoplasm with melanocytic differentiation, histologically characterized by the presence of polygonal or spindle shaped clear cells. This sarcoma usually affects the tendons and aponeuroses and is associated with a poor prognosis due to recurrencies and metastases 1
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



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Clear cell sarcoma of the kidney (CCSK) is a rare paediatric tumour that frequently metastasizes to bone. CCSKs are unicentric, typically large, unencapsulated masses centred in the renal medulla. They are characterized by nests or cords of cells separated by fibrovascular septa. The tumours are circumscribed but typically infiltrative at their borders.
The mean age at diagnosis is 36 months, with a male to female ratio of 2:1. Survival has increased due to improved therapy protocols, but metastases may occur as late as 10 years after initial diagnosis
2
 
Eble JE, Sauter G, Epstein JI, Sesterhenn IA (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Urinary System and Male Genital Organs.
3rd Edition
IARC Press: Lyon 2004



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