Summary
Definition
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 disease
- radiation therapy
- Rothmund-Thomson syndrome
- familial retinoblastoma syndrome
- Li-Fraumeni syndrome
- fibrous dysplasia
- chemotherapy
- male sex
Diagnostic investigations
1st investigations to order
- conventional radiographs
- bone biopsy
- magnetic resonance imaging (MRI)
- computed tomography (CT)
- CT thorax
- bone scan
- whole body positron emission tomography-computed tomography (PET-CT)
- complete blood count
- serum alkaline phosphatase
- serum lactate dehydrogenase
Treatment algorithm
low-grade disease at presentation
high-grade nonmetastatic 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 sarcoma
- Chondrosarcoma
- Malignant fibrous histiocytoma
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