Abstract
AbstractThis document is an update of the British Sarcoma Group guidelines (2016) and provides a reference standard for the clinical care of UK patients with primary malignant bone tumours (PMBT) and giant cell tumours (GCTB) of bone. The guidelines recommend treatments that are effective and should be available in the UK, and support decisions about management and service delivery. The document represents a consensus amongst British Sarcoma Group members in 2024. Key recommendations are that bone pain, or a palpable mass should always lead to further investigation and that patients with clinical or radiological findings suggestive of a primary bone tumour at any anatomic site should be referred to a specialist centre and managed by an accredited bone sarcoma multidisciplinary team. Treatment recommendations are provided for the major tumour types and for localised, metastatic and recurrent disease. Follow-up schedules are suggested.
Topics

No keywords indexed for this article. Browse by subject →

References
225
[1]
NHS Commissioning (2019) Sarcoma Services (all ages). https://www.england.nhs.uk/commissioning/publication/sarcoma-services-all-ages/. Accessed 21st October 2024
[2]
Gerrand C, Athanasou N, Brennan B, Grimer R, Judson I, Morland B, et al. UK guidelines for the management of bone sarcomas. Clin Sarcoma Res. 2016;6:1–21. 10.1186/s13569-016-0047-1
[3]
Sybil Biermann J, Hirbe A, Chow W, Bernthal NM, Boles S, Brigman B, et al (2021) NCCN Clinical Practice Guidelines In Oncology. Bone Cancer. Version 2.2022. https://www.nccn.org.
[4]
Strauss SJ, Frezza AM, Abecassis N, Bajpai J, Bauer S, Biagini R, et al. Bone sarcomas: ESMO–EURACAN–GENTURIS–ERN PaedCan Clinical Practice Guideline for diagnosis, treatment and follow-up. Ann Oncol. 2021;32:1520–36. 10.1016/j.annonc.2021.08.1995
[5]
National Institute for Health and Clinical Excellence. Sarcoma Quality Standard QS78. https://www.nice.org.uk/guidance/qs78. Accessed 21st October 2024.
[6]
National Institute for Health and Care Excellence (2015) Suspected cancer: recognition and referral. https://www.nice.org.uk/guidance/ng12. Accessed 21st October 2024
[7]
Burden and centralised treatment in Europe of rare tumours: results of RARECAREnet—a population-based study

Gemma Gatta, Riccardo Capocaccia, Laura Botta et al.

The Lancet Oncology 2017 10.1016/s1470-2045(17)30445-x
[8]
Botta L, Gatta G, Capocaccia R, Stiller C, Cañete A, Dal Maso L, et al. Long-term survival and cure fraction estimates for childhood cancer in Europe (EUROCARE-6): results from a population-based study. Lancet Oncol. 2022;23:1525–36. 10.1016/s1470-2045(22)00637-4
[9]
Whelan J, McTiernan A, Cooper N, Wong YK, Francis M, Vernon S, et al. Incidence and survival of malignant bone sarcomas in England 1979-2007. Int J Cancer. 2012;131:E508–E517. 10.1002/ijc.26426
[10]
Stiller CA, Botta L, Brewster DH, Ho VKY, Frezza AM, Whelan J, et al. Survival of adults with cancers of bone or soft tissue in Europe—Report from the EUROCARE-5 study. Cancer Epidemiol. 2018;56:146–53. 10.1016/j.canep.2018.08.010
[11]
National Cancer Registration and Analysis Service. Get Data Out - Sarcoma. https://www.cancerdata.nhs.uk/getdataout/sarcoma. Accessed 21st October 2024
[12]
Lai X, Chen S. Identification of novel biomarker candidates for immunohistochemical diagnosis to distinguish low-grade chondrosarcoma from enchondroma. Proteomics. 2015;15:2358–68. 10.1002/pmic.201400528
[13]
Verdegaal SHM, Bovee JVMG, Pansuriya TC, Grimer RJ, Ozger H, Jutte PC, et al. Incidence, Predictive Factors, and Prognosis of Chondrosarcoma in Patients with Ollier Disease and Maffucci Syndrome: An International Multicenter Study of 161 Patients. Oncologist. 2011;16:1771–9. 10.1634/theoncologist.2011-0200
[14]
Assessment of Interobserver Variability and Histologic Parameters to Improve Reliability in Classification and Grading of Central Cartilaginous Tumors

Daniël Eefting, Yvonne M. Schrage, Maartje J. A. Geirnaerdt et al.

American Journal of Surgical Pathology 2009 10.1097/pas.0b013e31817eec2b
[15]
Gelderblom H, Hogendoorn PCWW, Dijkstra SD, van Rijswijk CS, Krol AD, Taminiau AHMM, et al. The clinical approach towards chondrosarcoma. Oncologist. 2008;13:320–9. 10.1634/theoncologist.2007-0237
[16]
Riedel RF, Larrier N, Dodd L, Kirsch D, Martinez S, Brigman BE. The Clinical Management of Chondrosarcoma. Curr Treat Options Oncol. 2009;10:94–106. 10.1007/s11864-009-0088-2
[17]
Osteosarcoma: A Surveillance, Epidemiology, and End Results program‐based analysis from 1975 to 2017

Sarah Cole, D. Matthew Gianferante, Bin Zhu et al.

Cancer 2022 10.1002/cncr.34163
[18]
Gianferante DM, Mirabello L, Savage SA. Germline and somatic genetics of osteosarcoma — connecting aetiology, biology and therapy. Nat Rev Endocrinol. 2017;13:480–91. 10.1038/nrendo.2017.16
[19]
WHO Editorial Board. WHO classification of bone tumours. In WHO Classification of Tumours Editorial Board. Soft tissue and bone tumours. Bovee J, Flanagan AM, Lazar AJ, Nielsen GP and Yoshida A (eds) pp 338. International Agency for Research on Cancer (2020)
[20]
Palmerini E, Gambarotti M, Italiano A, Nathenson MJ, Ratan R, Dileo P, et al. A global collaboRAtive study of CIC-rearranged, BCOR::CCNB3-rearranged and other ultra-rare unclassified undifferentiated small round cell sarcomas (GRACefUl). Eur J Cancer. 2023;183:11–23. 10.1016/j.ejca.2023.01.003
[21]
Brahmi M, Gaspar N, Gantzer J, Toulmonde M, Boudou‐Rouquette P, Bompas E, et al. Patterns of care and outcome of CIC‐rearranged sarcoma patients: A nationwide study of the French sarcoma group. Cancer Med. 2023;12:7801–7. 10.1002/cam4.5539
[22]
Kobayashi H, Zhang L, Hirai T, Tsuda Y, Ikegami M, Tanaka S. Clinical characteristics of undifferentiated pleomorphic sarcoma of bone and the impact of adjuvant chemotherapy on the affected patients: a population-based cohort study. Jpn J Clin Oncol. 2022;52:589–98. 10.1093/jjco/hyac017
[23]
Tirabosco R, Mangham DC, Rosenberg AE, Vujovic S, Bousdras K, Pizzolitto S, et al. Brachyury expression in extra-axial skeletal and soft tissue chordomas: a marker that distinguishes chordoma from mixed tumor/myoepithelioma/parachordoma in soft tissue. Am J Surg Pathol. 2008;32:572–80. 10.1097/pas.0b013e31815b693a
[24]
Stacchiotti S, Sommer J, Ares C, Blay JY, Bolle S, Boriani S, et al. Building a global consensus approach to chordoma: a position paper from the medical and patient community. Lancet Oncol. 2015;16:71–83. 10.1016/s1470-2045(14)71190-8
[25]
Aytekin MN, Öztürk R, Amer K. Epidemiological Study of Adamantinoma from US Surveillance, Epidemiology, and End Results Program: III Retrospective Analysis. J Oncol. 2020;2020:2809647–8. 10.1155/2020/2809647
[26]
Roque P, Mankin HJ, Rosenberg A. Adamantinoma: an unusual bone tumour. Chir Organ Mov. 2008;92:149–54. 10.1007/s12306-008-0060-5
[27]
Liede A, Hernandez RK, Tang E, Li C, Bennett B, Wong SS, et al. Epidemiology of benign giant cell tumor of bone in the Chinese population. J Bone Oncol. 2018;12:96–100. 10.1016/j.jbo.2018.07.003
[28]
Chan C, Adler Z, Reith J, Gibbs C. Risk Factors for Pulmonary Metastases from Giant Cell Tumor of Bone. J Bone Jt Surg Am. 2015;97:420–8. 10.2106/jbjs.n.00678
[29]
Wang J, Liu X, Yang Y, Yang R, Tang X, Yan T, et al. Pulmonary metastasis of giant cell tumour: a retrospective study of three hundred and ten cases. Int Orthop (SICOT). 2021;45:769–78. 10.1007/s00264-020-04907-0
[30]
Palmerini E, Picci P, Reichardt P, Downey G. Malignancy in Giant Cell Tumor of Bone: A Review of the Literature. Technol Cancer Res Treat 2019;18:1533033819840000. https://doi.org/10.1177/1533033819840000 10.1177/1533033819840000
[31]
Behjati S, Tarpey PS, Presneau N, Scheipl S, Pillay N, Van Loo P, et al. Distinct H3F3A and H3F3B driver mutations define chondroblastoma and giant cell tumor of bone. Nat Genet 2013;45:1479–82. 10.1038/ng.2814
[32]
Sbaraglia M, Righi A, Gambarotti M, Vanel D, Picci P, Dei Tos AP. Soft Tissue Tumors Rarely Presenting Primary in Bone; Diagnostic Pitfalls. Surg Pathol Clin. 2017;10:705–30. 10.1016/j.path.2017.04.013
[33]
Pakos EE, Grimer RJ, Peake D, Spooner D, Carter SR, Tillman RM, et al. The ‘other’ bone sarcomas: prognostic factors and outcomes of spindle cell sarcomas of bone. J Bone Jt Surg Br. 2011;93:1271–8. 10.1302/0301-620x.93b9.27166
[34]
Koo MM, Lyratzopoulos G, Herbert A, Abel GA, Taylor RM, Barber JA, et al. Association of Self-reported Presenting Symptoms With Timeliness of Help-Seeking Among Adolescents and Young Adults With Cancer in the BRIGHTLIGHT Study. JAMA Netw Open. 3. https://doi.org/10.1001/JAMANETWORKOPEN.2020.15437 (2020) 10.1001/jamanetworkopen.2020.15437
[35]
National Institute for Health and Care Excellence (NICE). Guidance on cancer services - improving outcomes for people with sarcoma - the manual. https://www.nice.org.uk/guidance/csg9. (2006). Accessed 21 October 2024.
[36]
Mankin HJ, Mankin CJ, Simon M. The Hazards of the Biopsy, Revisited. For the Members of the Musculoskeletal Tumor Society. J Bone Jt Surg Am. 1996;78:656–63. 10.2106/00004623-199605000-00004
[37]
NHS Scotland. Scottish Referral Guidelines for Suspected Cancer - Sarcomas and bone cancers (2019). https://www.cancerreferral.scot.nhs.uk/sarcomas-and-bone-cancers/ Accessed 21st October 2024
[38]
Fairbairn J, Green R, Saifuddin A Recommendations for cross-sectional imaging in cancer management, Second edition. Musculoskeletal tumours. Faculty of Clinical Radiology (2014).
[39]
Annovazzi A, Ferraresi V, Anelli V, Covello R, Vari S, Zoccali C, et al. [18F]FDG PET/CT quantitative parameters for the prediction of histological response to induction chemotherapy and clinical outcome in patients with localised bone and soft-tissue Ewing sarcoma. Eur Radio. 2021;31:7012–21. 10.1007/s00330-021-07841-w
[40]
Quartuccio N, Fox J, Kuk D, Wexler LH, Baldari S, Cistaro A, et al. Pediatric bone sarcoma: diagnostic performance of ¹⁸F-FDG PET/CT versus conventional imaging for initial staging and follow-up. AJR Am J Roentgenol. 2015;204:153–60. 10.2214/ajr.14.12932
[41]
Aryal A, Kumar VS, Shamim SA, Gamanagatti S, Khan SA. What Is the Comparative Ability of 18F-FDG PET/CT, 99mTc-MDP Skeletal Scintigraphy, and Whole-body MRI as a Staging Investigation to Detect Skeletal Metastases in Patients with Osteosarcoma and Ewing Sarcoma? Clin Orthop Relat Res. 2021;479:1768–79. 10.1097/corr.0000000000001681
[42]
Guinot A, Tabone-Eglinger S, Isnardi V, Bahri H, Surdez D, Delattre O, et al. Staging of newly diagnosed Ewing sarcoma: Results of bone marrow aspiration and biopsy versus (18)FDG-PET/CT imaging for bone marrow involvement. Eur J Cancer. 2023;179:56–64. 10.1016/j.ejca.2022.11.002
[43]
Oliveira I, Singla N, Chavda A, Saifuddin A. The value of chest and skeletal staging studies in conventional chondrosarcoma. Skelet Radio. 2021;50:125–35. 10.1007/s00256-020-03539-0
[44]
Hongtao L, Hui Z, Bingshun W, Xiaojin W, Zhiyu W, Shuier Z, et al. 18F-FDG positron emission tomography for the assessment of histological response to neoadjuvant chemotherapy in osteosarcomas: A meta-analysis. Surg Oncol. 2012;21:e165–e170. 10.1016/j.suronc.2012.07.002
[45]
Meyer JS, Nadel HR, Marina N, Womer RB, Brown KLB, Eary JF, et al. Imaging guidelines for children with Ewing sarcoma and osteosarcoma: A report from the Children’s Oncology Group Bone Tumor Committee. Paediatr Blood Cancer. 2008;51:163–70. 10.1002/pbc.21596
[46]
Enneking WF, Spanier SS, Goodman MA. A System for the Surgical Staging of Musculoskeletal Sarcoma. Clin Orth Relat Res. 1980;153:106–20. 10.1097/00003086-198011000-00013
[47]
Amin MB, Edge SB, Greene FL AJCC cancer staging manual. AJCC, American Joint Committee on Cancer: Berlin (2017)
[48]
Bramer JAMM, van Linge JH, Grimer RJ, Scholten RJPM. Prognostic factors in localized extremity osteosarcoma: a systematic review. Eur J Surg Oncol. 2009;35:1030–6. 10.1016/j.ejso.2009.01.011
[49]
Leavey PJ, Mascarenhas L, Marina N, Chen Z, Krailo M, Miser J, et al. Prognostic factors for patients with Ewing sarcoma (EWS) at first recurrence following multi-modality therapy: A report from the Children’s Oncology Group. Paed Blood Cancer. 2008;51:334–8. 10.1002/pbc.21618
[50]
Ingley KM, Wan S, Vöö S, Windsor R, Michelagnoli M, Saifuddin A, et al. Is It Time to Call Time on Bone Marrow Biopsy for Staging Ewing Sarcoma (ES)? Cancers. 2021;13:3261. 10.3390/cancers13133261

Showing 50 of 225 references

Metrics
37
Citations
225
References
Details
Published
Nov 16, 2024
Vol/Issue
132(1)
Pages
32-48
License
View
Cite This Article
Craig Gerrand, Fernanda Amary, Hanny A. Anwar, et al. (2024). UK guidelines for the management of bone sarcomas. British Journal of Cancer, 132(1), 32-48. https://doi.org/10.1038/s41416-024-02868-4