ReviewThe Canadian journal of urology2025
Long-term follow-up of metastatic renal myoepithelial carcinoma: case report and literature review.
Review in The Canadian journal of urology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
backgroundPrimary renal myoepithelial carcinoma is an exceptionally rare malignancy with limited data on optimal treatment, particularly in metastatic settings. CASE DESCRIPTION: 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.
conclusionThe 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.
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