Related Experiment Video
Updated: Dec 29, 2025
A Syngeneic Mouse Model of Metastatic Renal Cell Carcinoma for Quantitative and Longitudinal Assessment of Preclinical Therapies
Published on: April 12, 2017
Metastatic Perivascular Epithelioid Cell Tumor Of The Kidney: A Case Report With Emphasis On Response To The
Rami S AlAzab1, Mohammed S Alorjani2, Firas E Sahawneh1
1Urology Division, Department of Surgery, Faculty of Medicine, Jordan University of Science and Technology, Irbid, Jordan.
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.
Insights
This case report details a rare perivascular epithelioid tumor in a young male, highlighting diagnostic and treatment challenges. It examines the tumor's response to tyrosine kinase-inhibitor therapy, a poorly documented area for this condition.
Area of Science:
- Oncology
- Pathology
Background:
- Perivascular epithelioid cell (PEComa) tumors are rare neoplasms with limited documented cases, leading to diagnostic and therapeutic uncertainties.
- Sunitinib, a tyrosine kinase inhibitor, is a standard treatment for metastatic renal cell carcinoma, but its efficacy in PEComas is not well-established.
Observation:
- A 29-year-old male presented with a metastatic left renal tumor, diagnosed as a rare PEComa variant.
- The patient underwent left partial nephrectomy followed by adjuvant tyrosine kinase-inhibitor (TKI) therapy.
Findings:
- This report describes the clinical and diagnostic features of this rare PEComa.
- It specifically addresses the challenges in managing PEComas and presents the observed response to adjuvant TKI therapy, which is not well-documented in existing literature.
Implications:
- This case contributes to understanding the clinical behavior of rare PEComas.
- It underscores the need for further research into optimal treatment strategies for PEComas, particularly regarding the role of targeted therapies like TKIs.
Related Concept Videos
02:57Targeted Cancer Therapies
There are several types of targeted therapies against...
02:30Metastasis
Epithelial-to-Mesenchymal Transition
The epithelial-to-mesenchymal transition or EMT is a developmental process commonly observed in wound healing, embryogenesis, and cancer metastasis. EMT is induced by transforming growth factor-beta (TGF-β) or receptor tyrosine kinase (RTK) ligands, which further...