@Article{cju.2025.063279,
AUTHOR = {Priya Dave, Ghizlane Yaakoubi, Justin Loloi, Niraj Shenoy, Ahmed Aboumohamed},
TITLE = {Long-term follow-up of metastatic renal myoepithelial carcinoma: case report and literature review},
JOURNAL = {Canadian Journal of Urology},
VOLUME = {32},
YEAR = {2025},
NUMBER = {3},
PAGES = {167--172},
URL = {http://www.techscience.com/CJU/v32n3/62612},
ISSN = {1488-5581},
ABSTRACT = { <b>Background:</b> Primary renal myoepithelial carcinoma is an exceptionally rare malignancy with limited data on optimal treatment, particularly in metastatic settings. <b>Case Description:</b> In 2020, Shenoy reported a dramatic response in a case of metastatic myoepithelial carcinoma with Ewing sarcoma breakpoint region 1-POU class 5 homeobox 1 (EWSR1-POU5F1) fusion arising from the left kidney using the Ewing Sarcoma vincristine, doxorubicin, cyclophosphamide/ifosfamide, etoposide (VDC/IE) chemotherapy regimen. Ten months post-treatment, the patient showed ~90% reduced disease burden on imaging. Subsequent treatment included consolidation vincristine, cyclophosphamide/ifosfamide, etoposide (VC/IE) chemotherapy, surgical resection of the remnant tumor, and follow-up imaging. <b>Conclusion:</b> The patient has been disease-free for 44 months off treatment and 5 years post-treatment initiation. To our knowledge, this is the first report of long-term disease-free survival in metastatic primary renal myoepithelial carcinoma. We also review the literature on this rare disease.},
DOI = {10.32604/cju.2025.063279}
}