Atypical Presentation of Renal Leiomyosarcoma: A Case Report

Primary renal sarcomas are rare comprising from 0.8 to 2.7% of adult renal tumours. They cannot be distinguished clinically or radiologically from the more common renal cell carcinomas. Leiomyosarcoma is the most common histological subtype accounting for around 50-60% of renal sarcomas. Leiomyosarcomas arise from the renal capsule, renal vein or the renal pelvis. They present with nonspecific clinical symptoms and signs and usually have a dismal outcome. Rarely atypical presentations such as acute tumour rupture and hematuria are encountered. We report a 27-year-old woman who presented with malignant hypertension. The hypertension was controlled emergently with antihypertensive agents. In view of the young age, a thorough workup was conducted to identify the cause of hypertension. Imaging studies revealed a solid enhancing renal tumour compressing the renal vasculature. As the staging workup did not reveal any evidence of metastases, the patient underwent right-sided open radical nephrectomy after adequate control of hypertension. The histological examination uncovered the renal tumour to be a leiomyosarcoma. Postoperative period was uneventful and she was doing well on one year follow up. Surprisingly the blood pressure normalised postoperatively and the patient was weaned off of her antihypertensive medications. This case is presented to highlight the atypical acute presentation of primary renal leiomyosarcoma with relatively good prognosis. Timely diagnosis and meticulous surgical resection improved the prognosis of this aggressive renal malignancy.