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Renal angiomyolipomas (AML) are the most common benign renal tumors. By containing an important amount of fat tissue they are easily identifiable on CT and MRI images, thus no biopsy should be needed for a diagnosis. However there is a small percentage of AMLs that have very little fat tissue and are very difficult to differentiate from renal cell carcinoma. Along fat tissue, they also contain smooth muscle and blood vessels. Renal AMLs can occur as a sporadic finding, or more frequently in association with tuberous sclerosis complex (TSC) or pulmonary lymphangioleiomyomatosis (LAM). These tumors are usually an incidental finding on imaging tools, but sometimes a serious presentation is rupture and hemorrhage, which can easily evolve to shock. Therapeutical management vary from active surveillance to surgical removal, and it needs to be individualized for every patient and according to the clinical presentation.

According to literature the primary retroperitoneal malignant pathology is rare, representing less than 0.5% of all malignancies. In spite of its low incidence, this pathology often proves challenging in terms of diagnosis and treatment. The most com¬mon retroperitoneal malignancy is represented by lymphoma that accounts for approximately 33 % of the retroperitoneal malignancies. Other frequent retroperitoneal tumors are sarcomas: liposarcomas, leiomyosarcomas and malignant fibrous histiocytoma (in order of incidence). We have analyzed the existing data regarding the most common primary retroperitoneal tumors in order to realize a brief classification and their differential diagnosis considering their clinical, imagistic, histopathological and molecular characteristics. The technological developments that have been made over the years in terms of imaging investigations, as well as in biomelecular and cytogenetic studies have offered new possibilities for of assessing a retroperitoneal mass in order achieve more precise informations that can guide physicians to better distinguish between different types of retroperitoneal tumors and therefore their therapeutical protocol.