Open Access

CASE REPORT

Long-term follow-up of metastatic renal myoepithelial carcinoma: case report and literature review

Priya Dave¹, Ghizlane Yaakoubi², Justin Loloi¹, Niraj Shenoy^3,*, Ahmed Aboumohamed^1,*

1 Department of Urology, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, NY 10467, USA
2 Medicine, Mohammed VI University Hospital, Faculty of Medicine of Oujda, Mohammed First University, Oujda, 60000, Morocco
3 Department of Medicine (Hematology/Oncology), Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA

* Corresponding Authors: Niraj Shenoy. Email: ; Ahmed Aboumohamed. Email:

Canadian Journal of Urology 2025, 32(3), 167-172. https://doi.org/10.32604/cju.2025.063279

Received 10 January 2025; Accepted 06 May 2025; Issue published 27 June 2025

Abstract

Background: Primary 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. Conclusion: 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.

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Supplementary Material

Supplementary Material File

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