Clinical experience and treatment strategy of leiomyosarcoma originating from the renal vein

BACKGROUND: Leiomyosarcoma originating from the renal vein (RVLMS) is extremely rare. RVLMS lacks specific clinical manifestations and specific imaging features. This article discusses the epidemiological characteristics and diagnostic difficulties of RVLMS, as well as imaging features, differential diagnosis, treatment strategy, and prognostic factors of this disease. METHOD: A case of RVLMS at our center, and 55 cases from the literature based on the PubMed search. RESULTS: Total operation time was 224 min, and total blood loss during the surgery was 200 ml. Resected tumor was irregular in shape, with negative margins. On the 6th day after the operation, the drainage tube was removed, and the patient was discharged from the hospital. Postoperative pathological results confirmed the renal vein leiomyosarcoma: spindle cell sarcoma, diffuse severe atypia, S-100 (-), SMA ( +), desmin ( +), CD34 (−), CD99 ( +). Twenty-seven months after the surgery, the patient is alive, and without local recurrence or distant metastases. CONCLUSION: Unspecific clinical manifestations and imaging features make the diagnosis of RVLMS difficult. Most patients are diagnosed intra-operatively or following postoperative pathology. Differential diagnosis with paraganglioma (PG) and retroperitoneal sarcoma (RPS) should be made. Early and complete resection is considered as the first choice of treatment, and whether to preserve the kidney is based on the patient's condition. RVLMS is highly malignant, and may recur locally or metastasize to distant locations; therefore, adjuvant therapy and regular follow-up should be carried out after surgery.