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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.
Synchronous renal cell carcinomas (RCC) and angiomyolipomas (AML) occurring in the same kidney are rare. Cases in the setting of tuberous sclerosis (TS) have been reported in the literature. However, the association of these tumors in the same kidney without TS is even more rare. We report here a case of a clear cell renal cell carcinoma (CCRCC) associated with an AML in the same kidney in a 42 years old female lacking the TS diagnostic criteria. The patient underwent a radical nephrectomy. Six months after surgery, the patient is healthy without signs of tumor recurrence or distant metastasis.
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