Primary Renal Angiosarcoma Mimicking Renal Cell Carcinoma: A Case Report

Abstract

Primary renal angiosarcoma is an exceedingly rare malignancy of the kidney. It usually presents in the elderly with metastatic disease and dismal prognosis. Treatment protocols are not standardized for this very rare renal malignancy. We report the case of a young man who was incidentally diagnosed with primary renal angiosarcoma. Preoperative imaging was suggestive of renal cell carcinoma; however, postoperative histopathological examination confirmed it to be an angiosarcoma. The patient was treated with surgical excision followed by adjuvant chemotherapy and is doing well at one-year follow-up.

Keywords: adjuvant chemotherapy; primary renal angiosarcoma; radical nephrectomy; renal cell carcinoma.

Figure 1. Contrast-enhanced computed tomography (CT) of the abdomen showing an enhancing heterogeneous tumor occupying the right kidney (white arrow)

Figure 2. Computed tomography (CT) of the chest showing an absence of pleural or lung metastases

Figure 3. Clinical photograph of the radical nephrectomy specimen opened along the lateral renal border showing tumor involving the upper pole and interpolar region. The tumor is composed of focal fleshy (white arrow) and necrotic areas (yellow arrow) with vascular spaces

Figure 4. Histopathological image demonstrating the tumor with hemorrhagic areas (yellow arrow) and vascular spaces lined by cells with pleomorphic and mitotically active nuclei (hematoxylin and eosin stain, 200x magnification)