title: Advances in the management of osteosarcoma.
abstract: Osteosarcoma, a bone cancer most commonly seen in adolescents and young adults, is usually a high-grade malignancy characterized by a very high risk for the development of pulmonary metastases. High-grade osteosarcomas are usually treated by preoperative and postoperative chemotherapy and surgery, with a very limited number of active agents available. Rarer lower-grade variants such as parosteal and periosteal osteosarcoma or low-grade central osteosarcoma are treated by surgery only. Imaging to search for possible metastases focuses on the lung. Computed tomography is the most sensitive method but cannot reliably distinguish small metastases from benign lesions. Advances of local imaging and surgical reconstruction now allow the use of limb-salvage in an ever-increasing proportion of patients. While still troubled by complications, non-invasive endoprosthesis-lengthening mechanisms have led to an increased uptake of limb-salvage, even for young, skeletally immature patients. Radiotherapy is employed when osteosarcomas cannot be removed with clear margins, but very high doses are required, and both proton and carbon-ion radiotherapy are under investigation. Unfortunately, the past 30 years have witnessed few, if any, survival improvements. Novel agents have not led to universally accepted changes of treatment standards. In patients with operable high-grade osteosarcomas, the extent of histological response to preoperative chemotherapy is a significant predictive factor for both local and systemic control. Attempts to improve prognosis by adapting postoperative treatment to response, recently tested in a randomized, prospective setting by the European and American Osteosarcoma Study Group, have not been proven to be beneficial. Many agree that only increased knowledge about osteosarcoma biology will lead to novel, effective treatment approaches and will be able to move the field forward.
authors.affiliation: Klinikum Stuttgart - Olgahospital, Zentrum für Kinder-, Jugend- und Frauenmedizin; Pädiatrie 5 (Onkologie, Hämatologie, Immunologie), Kriegsbergstrasse 62, Stuttgart Cancer Center, Germany.
authors.forename: Stefan S
authors.initials: SS
authors.lastname: Bielack
authors.affiliation: Klinikum Stuttgart - Olgahospital, Zentrum für Kinder-, Jugend- und Frauenmedizin; Pädiatrie 5 (Onkologie, Hämatologie, Immunologie), Kriegsbergstrasse 62, Stuttgart Cancer Center, Germany.
authors.forename: Stefanie
authors.initials: S
authors.lastname: Hecker-Nolting
authors.affiliation: Klinikum Stuttgart - Olgahospital, Zentrum für Kinder-, Jugend- und Frauenmedizin; Pädiatrie 5 (Onkologie, Hämatologie, Immunologie), Kriegsbergstrasse 62, Stuttgart Cancer Center, Germany.
authors.forename: Claudia
authors.initials: C
authors.lastname: Blattmann
authors.affiliation: St. Anna Children's Hospital, Department of Paediatrics, Medical University Vienna and Children's Cancer Research Institute CCRI, Vienna, Austria.
authors.forename: Leo
authors.initials: L
authors.lastname: Kager
keywords: chemotherapy; imaging; osteosarcoma; radiotherapy; surgery