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Osteosarcoma

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Disease definition

Osteosarcoma is a primary malignant tumour of the skeleton characterised by the direct formation of immature bone or osteoid tissue by the tumour cells.

ORPHA:668

Classification level: Disorder

Synonym(s):
  • Osteogenic sarcoma

Prevalence: 1-9 / 100 000

Inheritance: Not applicable

Age of onset: Childhood

ICD-10: C41.9

ICD-11: 2B51.Y 2B51 2B51.0 2B51.1 2B51.2

OMIM: 259500

UMLS: C0029463

MeSH: D012516

GARD: 7284

MedDRA: 10031291

Summary
Epidemiology

Classic osteosarcoma is a rare (0.2% of all malignant tumours) highly malignant tumour, with an estimated incidence of 3 cases/million population/year.

Clinical description

Osteosarcoma arises predominantly in the long bones and rarely in the soft tissues. The age at presentation ranges from 10 to 25 years of age.

Diagnostic methods

Plain radiographs, computed tomography, magnetic resonance imaging, angiography and dynamic bone scintigraphy are used for diagnosis, evaluation the extent of tumour involvement and for making decisions about the type of operation and, if necessary, the type of reconstruction required.

Management and treatment

In the past, all patients with osteosarcoma were treated by amputation but the cure rate was under 10% and almost all patients died within a year from diagnosis. At present, the percentage of patients cured varies between 60% and 70% for localised osteosarcoma at onset (80% of cases) treated in specialised bone tumour centres with pre- and postoperative chemotherapy associated with surgery. Surgery is conservative (limb salvage) in more than 90% of patients.

Prognosis

Prognosis is more severe (cure rate about 30%) for tumours located in the axial skeleton and in patients with metastasis at onset.

Last update: January 2007 - Expert reviewer(s): Dr Piero PICCI
A summary on this disease is available in Français (2007) Español (2007) Deutsch (2007) Italiano (2007) Português (2024) Nederlands (2007) Polski (2024) čeština (2024)
Detailed information
General public
Article for general public
English (2017) - PDQ Cancer Inf Sum
Guidelines
Clinical practice guidelines
English (2018) - Ann Oncol Logo ERN
English (2021) - Ann Oncol Logo ERN
English (2020) - Eur J Hum Genet Logo ERN
Disease review articles
Review article
Deutsch (2023) - Onkopedia
English (2017) - PDQ Cancer Inf Sum

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