AlAzab Rami S, Alorjani Mohammed S, Sahawneh Firas E, Al-Sukhun Sana
Urology Division, Department of Surgery, Faculty of Medicine, Jordan University of Science and Technology, Irbid, Jordan.
Department of Pathology and Microbiology, Faculty of Medicine, Jordan University of Science and Technology, Irbid, Jordan.
Res Rep Urol. 2019 Nov 13;11:311-317. doi: 10.2147/RRU.S226005. eCollection 2019.
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.
几例病例报告提供了关于血管周上皮样细胞瘤的大部分现有信息，但在诊断或治疗模式方面尚未达成共识。舒尼替尼是一种VEGFR多靶点酪氨酸激酶抑制剂，被视为减瘤手术后透明细胞型转移性肾细胞癌的一线治疗药物。我们的病例是一名29岁男性，患有左肾转移性肿瘤，接受了左肾部分切除术，随后接受辅助酪氨酸激酶抑制剂治疗。我们报告此病例，以展示这种罕见病理变体的特殊性、其临床和诊断特征以及治疗选择方面的挑战，因为这种罕见肿瘤对辅助酪氨酸激酶抑制剂治疗的反应尚未得到充分描述。
