Abstract
Periosteal osteosarcoma (PO), an intermediate-grade chondroblastic osteosarcoma (OST) arising from the surface of the bones, is a rare histological subtype among primary bone sarcomas, most commonly diagnosed in young patients. It is characterized by distinct specific radiological and pathological features. The current management strategy is based on several case reports and series, without any solid international recommendations. Most sarcoma experts agree on the crucial role of an optimal complete surgical approach. However, with the paucity of available reports, the role of systemic treatment and its timing remains debatable. With this paper, we will review the available data on the actual impact of chemotherapy in PO patients with emphasis on the radiological, pathological, and therapeutic characteristics of this rare entity.