Abstract

A 64 year old woman was referred to hospital with complaints of abdomen right side tightness with discomfort since one month. Computed tomography scan of abdomen investigation revealed large retroperitoneal mass measuring 18.9*12.1cms displacing the inferior vena cava right kidney and renal vessels in right hypochondriac and lumbar regions of the abdomen with large fat components. She underwent surgery which involves the resection of tumor mass with non-affected surgical margins. These tumors tend to be resistant to radiotherapy or chemotherapy. Among the most important prognostic factor related to survival is surgery with non-affected margins. A review on etiology, pathophysiology, pathological classification and grading is explained in literature review.