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Desmoplastic small-round-cell tumor is an aggressive and rare cancer that primarily occurs as masses in the abdomen.〔Lee YS, Hsiao CH: Desmoplastic small round cell tumor: a clinicopathologic, immunohistochemical and molecular study of four patients. J Formos Med Assoc 2007; 106: 854–860.〕 Other areas affected may include the lymph nodes, the lining of the abdomen, diaphragm, spleen, liver, chest wall, skull, spinal cord, large intestine, small intestine, bladder, brain, lungs, testicles, ovaries, and the pelvis. Reported sites of metatastic spread include the liver, lungs, lymph nodes, brain, skull, and bones. The tumor is classified as a soft tissue sarcoma. It is considered a childhood cancer that predominantly strikes boys and young adults. The disease rarely occurs in females, but when it does the tumors can be mistaken for ovarian cancer. In dogs, mast cell tumors are the most frequent round cell tumor.〔Mackowiak II ''et al'' (2012) E-cadherin in canine mast cell tumors: Decreased expression and altered subcellular localization in Grade 3 tumors. ''Vet J'' 194(3):405-11〕 ==Causes== There are no known risk factors that have been identified specific to the disease. The tumor appears to arise from the primitive cells of childhood, and is considered a childhood cancer. Research has indicated that there is a chimeric relationship between desmoplastic small-round-cell tumor (DSRCT) and Wilms' tumor and Ewing's sarcoma. Together with neuroblastoma and non-Hodgkin's lymphoma, they form the small cell tumors. DSRCT is associated with a unique chromosomal translocation t(11;22)(p13:q12) resulting in an EWS/WT1 transcript that is diagnostic of this tumor. This transcript codes for a protein that acts as a transcriptional activator that fails to suppress tumor growth. The EWS/WT1 translocation product targets ENT4.〔 〕 ENT4 is also known as PMAT. 抄文引用元・出典: フリー百科事典『 ウィキペディア（Wikipedia）』 ■ウィキペディアで「Desmoplastic small-round-cell tumor」の詳細全文を読む スポンサード リンク
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