Observational Study in Localized Osteosarcoma

Osteosarcoma is the most frequent primary malignant bone tumor in children and adolescents and is considered a rare disease.

The overall incidence of OS is 0.2-3 / 100,000 inhabitants per year with a bimodal distribution by age, with a first peak in adolescence.

Currently, the treatment strategy for localized OS includes courses of neo-adjuvant chemotherapy based on 3 agents: Adriamycin (ADM), cisplatin (CDP) and high dose methotrexate (HDMTX) followed by surgical removal of the primary tumor and subsequent adjuvant chemotherapy treatment.

The MAP (Methotrexate, Adriamycin, cisPlatin) regimen, with or without ifosfamide (IFO) and etoposide (ETO), is considered the "gold standard" for the treatment of localized osteosarcoma.

Given the rarity of the disease, it is considered appropriate to collect information prospectively on the diagnosis, chemotherapy and surgical treatment and outcomes on patients treated by the network of AIEOP and ISG Centers according to the recommendations on the treatment of localized OS AIEOP/ISG OS 2021.

This study will be active up to the next interventional prospective protocol aimed at the therapy of high-grade, non-metastatic OS.

Inclusion criteria

• Diagnosis of high grade OS of the extremities
• Age at diagnosis ≤ 40 years at the time of diagnosis
• Localized disease (skip metastases are allowed)
• Adequate organ function according the AIEOP/ISG OS 2021 recommendation
• Patients or parents oe guardians of minors who have given their written informed consent to participate in the study

Exclusion criteria

• Presence of metastases
• Diagnosis of periosteal OS, of parosteal OS or secondary OS
• Any medical condition which can not allowed the use of the treatments recommended by AIEOP/ISG OS 2021