Clinical Medicine Insights: Oncology 2013:7 257–262 doi: 10.4137/CMO.S12243 This article is available from http://www.la-press.com. © the author(s), publisher and licensee Libertas Academica Ltd. This is an open access article published under the Creative Commons CC-BY-NC 3.0 license. Open Access Full open access to this and thousands of other papers at http://www.la-press.com. Clinical Medicine Insights: Oncology CASe RepORT Clinical Medicine Insights: Oncology 2013:7 257 primary Monophasic synovial sarcoma of the Kidney: A case Report and Review of Literature Henrique Lopes 1 , Caio A.D. pereira 1 , Luís e.R. Zucca 1 , Sérgio V. Serrano 1 , Sandra R.M. Silva 2 , Marjori L. Camparoto 3 and Flavio M. Cárcano 1 1 Department of Medical Oncology, Barretos Cancer Hospital, Barretos, Sp, Brazil. 2 Department of pathology, Barretos Cancer Hospital, Barretos, Sp, Brazil. 3 Department of Cytogenetics, Center of Research on Molecular Oncology, Barretos Cancer Hospital, Barretos, Sp, Brazil. Corresponding author email: fmcmed@globo.com Abstract: Primary synovial sarcoma (SS) of the kidney is a rare neoplasm and its presenting features are similar to other common renal tumors, making early diagnosis diffcult. To date, few cases have been reported in the literature. Primary renal SSs can exist in either a monophasic or a biphasic pattern, the former being more common and tending to have a better prognosis than the biphasic variant. Herein we describe a case of primary renal SS that was diagnosed based on histopathology and immunohistochemistry after radical nephrectomy. Fusion gene product analysis was also done by FISH and RT-PCR. Patient follow-up and literature review are presented, focused on systemic therapy. We highlight that these tumors should be correctly diagnosed as clinical results and specifc treatment are distinct from primary epithelial renal cell carcinoma. Adjuvant chemotherapy should be tailored for each patient in the management of disease, although its role still remains unclear. Keywords: synovial sarcoma, kidney neoplasms, SYT-SSX fusion protein, chemotherapy