Fekkar Amal, Elouazzani Hafsa, Jahid Ahmed, Znati Kaoutar, Zouaidia Fouad, Bernoussi Zakia
Department of Pathology, Ibn Sina University Hospital Center, Rabat, Morocco.
Case Rep Pathol. 2021 May 12;2021:6637533. doi: 10.1155/2021/6637533. eCollection 2021.
Primary leiomyosarcomas (LMS) of vascular origin are rare tumors, and more than half of the cases arise in the inferior vena cava (IVC). Primary LMS of the renal vein are extremely rare tumors with only a few cases reported in the literature. Their diagnosis is made only by pathological features. Histologically, they are made of atypical spindle-shaped cells arranged in long intersecting fascicles. Tumor cells stain positive for myogenic markers in immunohistochemistry. Standard treatment consists of radical nephrectomy followed by chemotherapy and/or radiotherapy. Because of insufficient histological data and follow-up, the prognosis factors are not well identified. Overall prognosis of renal vein LMS is poor. We report here an exceptional case of a huge LMS of the right renal vein mimicking a primitive renal cell carcinoma, occurring in a 56-year-old male patient.
原发性血管源性平滑肌肉瘤（LMS）是罕见肿瘤，半数以上病例发生于下腔静脉（IVC）。肾静脉原发性LMS极为罕见，文献中仅报道过少数病例。其诊断仅依据病理特征。组织学上，它们由排列成相互交叉长束状的非典型梭形细胞构成。免疫组化中肿瘤细胞对肌源性标志物呈阳性染色。标准治疗包括根治性肾切除术，随后进行化疗和/或放疗。由于组织学数据和随访不足，预后因素尚未明确界定。肾静脉LMS的总体预后较差。我们在此报告一例罕见病例，一名56岁男性患者发生了巨大的右肾静脉LMS，酷似原发性肾细胞癌。
