What causes sarcomatoid renal cell carcinoma (SRCC)?

Updated: Mar 11, 2019
  • Author: Ronald J Cohen, MB, BCh, PhD, FRCPA, FFPATH; Chief Editor: Liang Cheng, MD  more...
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Answer

Answer

SRCCs were originally considered to be carcinosarcomas or to be renal sarcomas in cases in which the sarcomatous component had overgrown all epithelial (carcinomatous) components. [17, 18] Then, in 1968, sarcomatoid cells were proposed to arise through transformation of malignant renal epithelial cells; thus, the term sarcomatoid RCC was introduced. [19]

This theory has been confirmed by genetic studies such as analysis of X-chromosome inactivation patterns, which indicated a clonal origin for both the epithelial and sarcomatoid components of CCRCCs with sarcomatoid differentiation. [20]

Currently, the 2004 WHO classification of renal tumors recognizes this transformation as "sarcomatoid change" or "sarcomatoid features" arising within RCC, rather than as a separate histologic entity. [1] Sarcomatoid differentiation usually arises within high-grade RCC, [4, 7] representing a late step in the progression of this tumor type; however, the factors leading to development of sarcomatoid differentiation are unknown.

Etiologic factors associated with development of the underlying renal cell carcinoma (RCC) are most likely similar to those described for RCC in general, which include smoking, obesity, hypertension, and hereditary genetic disorders. [21, 22, 23]


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