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osteosarcoma (osteogenic sarcoma)
Classification: (WHO)
- central (medullary)
- conventional central osteosarcoma
- chondroblastic osteosarcoma
- fibroblastic osteosarcoma
- osteoblastic osteosarcoma
- telangiectatic osteosarcoma
- intraosseous well-differentiated (low grade) osteosarcoma
- small cell osteosarcoma
- surface (peripheral)
- parosteal well differentiated (low grade) osteosarcoma
- perosteal osteosarcoma, low to intermediate grade
- high grade surface osteosarcoma
Epidemiology:
- occurs predominantly in children & young adults.
- most common sites are metaphyses of long bones
- distal femur, proximal tibia, proximal humerus
Genetics:
- amplificatoin of MDM2
- implicated genes NANOGP8, OS9, PPIAL4B, PPIAL4, c-MYC, RB1, ADAMTS19, CHEK2
Clinical manifestations:
- typically affect the metaphyses of the long bones
- ~50% arise in the femur
- ~80% of these adjacent to the knee
- usually occur with few symptoms
Special laboratory:
- bone biopsy* prior to surgical intervention for fracture
* biopsy result will guide surgical approach
Radiology:
- spiculated appearance
- Codman triangles
- intramedullary sclerosis
Complications:
- pulmonary metastases from chondroblastic sarcoma [3] (images)
- metaphyseal fracture in adolescent or young adult
Management:
1) see [1]
2) cryosurgery
Interactions
disease interactions
Specific
extraosseous osteogenic sarcoma
osteosarcoma [RB1 mutation]
osteosarcoma [TP53/ARG248GLN]
osteosarcoma [TP53/SER241PHE]
retinoblastoma-related osteosarcoma
General
bone cancer
osteogenic neoplasm
sarcoma
Database Correlations
OMIM 259500
References
- WHO Classification Tumours of Soft Tissue and Bone
Fletcher, Unni & Mertens Eds. IARC Press 2002
- NEJM Knowledge+ Question of the Week. Jan 8, 2018
https://knowledgeplus.nejm.org/question-of-week/4923/
- Mamdani H, Grethlein SJ
Pulmonary Metastases from Chondroblastic Osteosarcoma.
N Engl J Med 2018; 378:1429. April 12, 2018
PMID: 29641960
http://www.nejm.org/doi/full/10.1056/NEJMicm1711874
- National Cancer Institute -
Osteosarcoma/Bone Fibrous Histiocytoma Treatment
http://www.nci.nih.gov/cancertopics/pdq/treatment/osteosarcoma/healthprofessional