Hemangiopericytoma (HPC) is an unusual vascular neoplasm. Renal HPC is very rare. Only a few cases of renal HPC with distant metastasis have been described in the literature. In the absence of specific clinical signs and symptoms and imageological findings, histopathology remains the cornerstone of diagnosis. The present case is a 56-year-old female who presented with abdominal lump and pain of 1-month duration. Her contrast-enhanced computed tomography abdomen and chest showed a large well-defined lobulated heterogeneously enhancing soft tissue density lesion arising from the lower pole of the kidney with central calcification. Nodular opacities were noted in both lungs. It was reported as renal cell carcinoma (RCC) with lung metastasis. She underwent left nephrectomy because of the suspicion of RCC. The histopathology along with immunohistochemistry was reported as HPC. This is an additional case of metastatic renal HPC.
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