High-grade surface osteosarcoma
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High-grade surface osteosarcomas are sporadic and high-grade malignant osteoid-forming tumors emerging from the bony surfaces similar to parosteal and periosteal osteosarcomas.
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High-grade surface osteosarcomas are very rare and constitute <1% of all osteosarcomas. There seems to be a peak incidence in the 2nd and 3rd decade of life and a male preference 1-5.
The diagnosis of high-grade surface osteosarcomas is based on a combination of typical radiographic features with the histology of high-grade osteosarcoma.
Diagnostic criteria according to the WHO classification of soft tissue and bone tumors (5th edition) 1:
imaging features of a bone tumor
histological finding of a high-grade osteosarcoma
origin from the surface of the bone
no significant intraosseous part
The most common complaints are swelling and pain 1. On rare occasions, there is joint stiffness or even no symptoms at all 3,4.
High-grade surface osteosarcomas are highly malignant osteosarcomas growing from the fibrous periosteal surfaces and are one of the three subtypes of surface osteosarcoma together with parosteal osteosarcoma and periosteal osteosarcoma 1-3.
The tumor has been found in the following locations 1-5:
Macroscopically high-grade surface osteosarcomas are of variable appearance mostly dependent on the type of generated extracellular matrix. The bulk of the tumor is usually located outside of the bone with infiltration and extension of the periosteum inwards and erosion of the underlying cortex 1.
Microscopic features of high-grade surface osteosarcomas are those of conventional high-grade osteosarcoma 1.
Irrelevant for diagnosis 1.
General imaging features of high-grade surface osteosarcomas are the following 1,2,5:
the tumor arises from the bony surfaces
cortical erosions (≈50% of the cases)
medullary involvement (≈50% of the cases)
usually no cleavage plane between the main tumor and cortex
periosteal reaction uncommon
relatively high circumferential involvement (20-100%)
On plain radiographs high-grade surface osteosarcomas will usually display the following characteristics 1:
fluffy immature ossification
CT can demonstrate the following:
MRI can accurately depict soft tissue and medullary involvement of high-grade surface osteosarcomas as well as peritumoral and/or medullary edema.
osteoid matrix: low signal intensity
non-ossified soft tissue components: intermediate signal intensity
osteoid matrix: low signal intensity
non-ossified soft tissue components: high signal intensity
T1 C+ (Gd): marked enhancement
The radiological report should include a description of the following 1,6:
form, location and size
tumor margins and transition zone
cortical erosion, cortical breakthrough
soft tissue component
infiltration of neurovascular structures
Treatment and prognosis
Tumor management includes a combination of wide surgical excision and chemotherapy 2.
The five-year survival varies with different studies and ranges from roughly 40-80% 1-3. Localized disease and a good response to neoadjuvant good prognostic factors 1.
History and etymology
High-grade surface osteosarcoma was first described by the American orthopedic surgeon Kenneth C. Francis and his pathologist and surgeon colleagues Robert VP Hutter and Bradley L Coley in 1964 2,3,7.
Conditions that can mimic the presentation of high-grade surface osteosarcomas include 1,4:
usually aggressive periosteal reaction
less circumferential involvement
less medullary involvement
parosteal osteosarcoma: less circumferential involvement
osteosarcoma: the bulk of the tumor is located inside the bone
- 1. Klein MJ, Bonar SFM, O’Donell PG. High-grade surface osteosarcoma. In: WHO Classification of Tumours Editorial Board. Soft tissue and bone tumours. Lyon (France): International Agency for Research on Cancer; 2020. (WHO classification of tumours series, 5th ed.; vol. 3). https://publications.iarc.fr
- 2. Staals E, Bacchini P, Bertoni F. High-Grade Surface Osteosarcoma. Cancer. 2008;112(7):1592-9. doi:10.1002/cncr.23340 - Pubmed
- 3. Deng Z, Huang Z, Ding Y, Su Y, Chan C, Niu X. High-Grade Surface Osteosarcoma: Clinical Features and Oncologic Outcome. Journal of Bone Oncology. 2020;23:100288. doi:10.1016/j.jbo.2020.100288 - Pubmed
- 4. Nouri H, Ben Maitigue M, Abid L et al. Surface Osteosarcoma: Clinical Features and Therapeutic Implications. Journal of Bone Oncology. 2015;4(4):115-23. doi:10.1016/j.jbo.2015.07.002 - Pubmed
- 5. Yarmish G, Klein M, Landa J, Lefkowitz R, Hwang S. Imaging Characteristics of Primary Osteosarcoma: Nonconventional Subtypes. Radiographics. 2010;30(6):1653-72. doi:10.1148/rg.306105524 - Pubmed
- 6. M. Sampson & D. Topham. Pictorial Essay of Imaging Findings in Surface Based Bone Lesions. European Congress of Radiology - RANZCR ASM 2013. 2013. https://epos.myesr.org/poster/ranzcr/ranzcr2013/R-0080
- 7. Francis KC,Hutter RVP,Coley BL. Treatment of osteogenic sarcoma. In: GT Pack, IM Ariel, editors. Treatment of Cancer and Allied Diseases. Vol. 8, 2nd ed. New York: Harper & Row; 1964: 374– 399.