Summary
Definition
The World Health Organization defines osteosarcoma as a primary osseous malignant neoplasm composed of mesenchymal cells producing osteoid and immature bone, even if only in small amounts.[1][Figure caption and citation for the preceding image starts]: Osteoblastic osteosarcoma of the distal femur (radiograph and photograph of the gross specimen)Personal collections of Dr Michael J. Klein and Dr Luminita Rezeanu [Citation ends].
History and exam
Key diagnostic factors
- childhood and adolescence
- worsening pain over weeks to months
- mass/swelling
Other diagnostic factors
- male sex
- limp
- history of trauma
- limited range of motion
- overlying skin ulceration
Risk factors
- childhood and adolescence
- Paget's disease
- radiotherapy
- Rothmund-Thomson syndrome
- familial retinoblastoma syndrome
- Li-Fraumeni syndrome
- fibrous dysplasia
- chemotherapy
- male sex
Diagnostic investigations
1st investigations to order
- conventional radiographs
- MRI primary tumour
- CT thorax
- radionuclide bone scan
- 18F-fluorodeoxyglucose positron emission tomography-computed tomography (18F-FDG-PET-CT)
- serum alkaline phosphatase
- serum lactate dehydrogenase
Investigations to consider
- CT primary tumour
- bone biopsy
Treatment algorithm
low-grade disease at presentation
high-grade non-metastatic disease at presentation
metastatic disease at presentation
recurrent disease
Contributors
Authors
David Loeb, MD, PhD
Chief
Division of Pediatric Hematology, Oncology, and Marrow & Blood Cell Transplantation
Children's Hospital at Montefiore
Associate Professor, Pediatrics
Associate Professor, Developmental and Molecular Biology
Albert Einstein College of Medicine
Bronx
NY
Disclosures
DL declares that he has no competing interests.
Acknowledgements
Dr David Loeb would like to gratefully acknowledge Dr Luminita Rezeanu and Dr Michael J. Klein, the previous contributors to this topic.
Disclosures
LR and MJK declare that they have no competing interests.
Peer reviewers
Edward Sauter, MD, PhD
Professor of Surgery
Ellis Fischel Cancer Center
University of Missouri
Columbia
MO
Disclosures
ES declares that he has no competing interests.
Rachael Windsor, BSc, MBBS, MSc, MRCPCH
Locum Consultant Paediatric Oncologist
University College Hospital
London
UK
Disclosures
RW declares that she has no competing interests.
Differentials
- Ewing's sarcoma
- Chondrosarcoma
- Malignant fibrous histiocytoma
More DifferentialsGuidelines
- NCCN clinical practice guidelines in oncology: bone cancer
- ACR appropriateness criteria: malignant or aggressive primary musculoskeletal tumor staging and surveillance
More Guidelines
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