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Metastatic Perivascular Epithelioid Cell Tumor Of The Kidney: A Case Report With Emphasis On Response To The Tyrosine-Kinase Inhibitor Sunitinib

Authors AlAzab RS, Alorjani MS, Sahawneh FE, Al-Sukhun S

Received 5 August 2019

Accepted for publication 24 October 2019

Published 13 November 2019 Volume 2019:11 Pages 311—317

DOI https://doi.org/10.2147/RRU.S226005

Checked for plagiarism Yes

Review by Single-blind

Peer reviewer comments 2

Editor who approved publication: Dr Jan Colli


Rami S AlAzab,1 Mohammed S Alorjani,2 Firas E Sahawneh,1 Sana Al-Sukhun3

1Urology Division, Department of Surgery, Faculty of Medicine, Jordan University of Science and Technology, Irbid, Jordan; 2Department of Pathology and Microbiology, Faculty of Medicine, Jordan University of Science and Technology, Irbid, Jordan; 3Oncology Practice, Hyatt Medical Center, Amman, Jordan

Correspondence: Rami S AlAzab
Urology Division, Department of Surgery, Faculty of Medicine, Jordan University of Science and Technology, P.O. Box 3030, Irbid 22110, Jordan
Tel +962799530333
Fax +962 2 7095010
Email rsazab@just.edu.jo

Abstract: Several case reports have provided most of the information available on perivascular epithelioid cell tumors, but with no consensus regarding diagnosis or treatment paradigms. Sunitinb is a VEGFR multitargeted tyrosine-kinase inhibitor that is regarded as a first-line treatment of clear cell–type metastatic renal-cell carcinoma after cytoreductive surgery. Our case was a 29-year-old male who presented with a metastatic left renal tumor and underwent left partial nephrectomy followed by adjuvant tyrosine kinase–inhibitor treatment. We report this case to present the peculiarity of this rare pathological variant, its clinical and diagnostic features, and challenges regarding treatment options, since the response of this rare tumor to adjuvant tyrosine kinase–inhibitor therapy has not been well described.

Keywords: PEComa, tyrosine kinase, renal-cell carcinoma, sarcoma, everolimus

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