Update and interpretation of 2021 National Comprehensive Cancer Network (NCCN) “Clinical Practice Guidelines for Bone Tumors”_Chinese Journal of Reparative and Reconstructive Surgery

Authors：

 NI Ming

Department of Orthopaedics, Pudong New Area People’s Hospital Affiliated to Shanghai University of Medicine and Health Sciences, Shanghai, 201299, P.R.China;

Corresponding author：

NI Ming, Email: gendianqing@163.com

Keywords：

Bone tumor; clinical practice; guidelines; interpretation

DOI：

10.7507/1002-1892.202103073

Video：

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The incidence of primary malignant bone tumors is low, and clinical cognition is insufficient. The establishment of diagnostic criteria is of great significance for prognosis of tumors. National Comprehensive Cancer Network (NCCN) regularly publishes “Clinical Practice Guidelines for Bone Tumors” to summarize the latest treatment progress of bone tumors. In the latest version of the guidelines released in November 2020, surgery is the main treatment for chondrosarcoma, chordoma, and giant cell tumor of bone, which can be combined with radiotherapy or targeted therapy. Ewing’s sarcoma and osteosarcoma are treated by surgery combined with chemotherapy. Immunotherapy can be used to treat high-grade undifferentiated pleomorphic sarcoma. For recurrent tumors, surgery combined with radiotherapy, chemotherapy, and/or targeted therapy can be used for control. The guidelines provide a reference for the standard treatment of bone tumors.

Citation： NI Ming. Update and interpretation of 2021 National Comprehensive Cancer Network (NCCN) “Clinical Practice Guidelines for Bone Tumors”. Chinese Journal of Reparative and Reconstructive Surgery, 2021, 35(9): 1186-1191. doi: 10.7507/1002-1892.202103073 Copy

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2.	Siegel RL, Miller KD, Jemal A. Cancer statistics, 2020. CA Cancer J Clin, 2020, 70(1): 7-30.
3.	Bone Cancer (Sarcoma of Bone): Statistics 2020. [EB/OL]. (2020-07-19). [2021-03-05]. https://www.cancer.net/cancer-types/bone-cancer/statistics.
4.	Fletcher CDM, Bridge JA, Hogendoorn P, et al. WHO Classification of Tumours of Soft Tissue and Bone. Lyon: IARC Press, 2013.
5.	Nicolle R, Ayadi M, Gomez-Brouchet A, et al. Integrated molecular characterization of chondrosarcoma reveals critical determinants of disease progression. Nat Commun, 2019, 10(1): 4622. doi: 10.1038/s41467-019-12525-7.
6.	Chow W, Frankel P, Ruel C, et al. Results of a prospective phase 2 study of pazopanib in patients with surgically unresectable or metastatic chondrosarcoma. Cancer, 2020, 126(1): 105-111.
7.	Amer KM, Munn M, Congiusta D, et al. Survival and prognosis of chondrosarcoma subtypes: SEER database analysis. J Orthop Res, 2020, 38(2): 311-319.
8.	Verdegaal SH, Bovée JV, Pansuriya TC, 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(12): 1771-1779.
9.	Schneiderman BA, Kliethermes SA, Nystrom LM. Survival in mesenchymal chondrosarcoma varies based on age and tumor location: A survival analysis of the SEER database. Clin Orthop Relat Res, 2017, 475(3): 799-805.
10.	Tap WD, Villalobos VM, Cote GM, et al. Phase Ⅰ study of the mutant IDH1 inhibitor ivosidenib: Safety and clinical activity in patients with advanced chondrosarcoma. J Clin Oncol, 2020, 38(15): 1693-1701.
11.	Chugh R, Tawbi H, Lucas DR, et al. Chordoma: the nonsarcoma primary bone tumor. Oncologist, 2007, 12(11): 1344-1350.
12.	Shih AR, Cote GM, Chebib I, et al. Clinicopathologic characteristics of poorly differentiated chordoma. Mod Pathol, 2018, 31(8): 1237-1245.
13.	McGovern SL, Mahajan A. Progress in radiotherapy for pediatric sarcomas. Curr Oncol Rep, 2012, 14(4): 320-326.
14.	Baumann BC, Lustig RA, Mazzoni S, et al. A prospective clinical trial of proton therapy for chordoma and chondrosarcoma: Feasibility assessment. J Surg Oncol, 2019, 120(2): 200-205.
15.	Ahmed AT, Abdel-Rahman O, Morsy M, et al. Management of sacrococcygeal chordoma: A systematic review and meta-analysis of observational studies. Spine (Phila Pa 1976), 2018, 43(19): E1157-E1169.
16.	Ailon T, Torabi R, Fisher CG, et al. Management of locally recurrent chordoma of the mobile spine and sacrum: A systematic review. Spine (Phila Pa 1976), 2016, 41(Suppl 20): S193-S198.
17.	Machado I, Navarro S, Llombart-Bosch A. Ewing sarcoma and the new emerging Ewing-like sarcomas: (CIC and BCOR-rearranged-sarcomas). A systematic review. Histol Histopathol, 2016, 31(11): 1169-1181.
18.	Sbaraglia M, Righi A, Gambarotti M, et al. Ewing sarcoma and Ewing-like tumors. Virchows Arch, 2020, 476(1): 109-119.
19.	Grevener K, Haveman LM, Ranft A, et al. Management and outcome of ewing sarcoma of the head and neck. Pediatr Blood Cancer, 2016, 63(4): 604-610.
20.	Bosma SE, Ayu O, Fiocco M, et al. Prognostic factors for survival in Ewing sarcoma: A systematic review. Surg Oncol, 2018, 27(4): 603-610.
21.	Grier HE, Krailo MD, Tarbell NJ, et al. Addition of ifosfamide and etoposide to standard chemotherapy for Ewing’s sarcoma and primitive neuroectodermal tumor of bone. N Engl J Med, 2003, 348(8): 694-701.
22.	Ronchi L, Buwenge M, Cortesi A, et al. Whole lung irradiation in patients with osteosarcoma and ewing sarcoma. Anticancer Res, 2018, 38(9): 4977-4985.
23.	Whelan J, Le Deley MC, Dirksen U, et al. High-dose chemotherapy and blood autologous stem-cell rescue compared with standard chemotherapy in localized high-risk ewing sarcoma: results of Euro-E. W. I. N. G. 99 and Ewing-2008. J Clin Oncol, 2018, 36(31): JCO2018782516. doi: 10.1200/JCO.2018.78.2516.
24.	Italiano A, Mir O, Mathoulin-Pelissier S, et al. Cabozantinib in patients with advanced Ewing sarcoma or osteosarcoma (CABONE): a multicentre, single-arm, phase 2 trial. Lancet Oncol, 2020, 21(3): 446-455.
25.	van der Heijden L, Dijkstra PDS, Blay JY, et al. Giant cell tumour of bone in the denosumab era. Eur J Cancer, 2017, 77: 75-83.
26.	Emori M, Kaya M, Sasaki M, et al. Pre-operative selective arterial embolization as a neoadjuvant therapy for proximal humerus giant cell tumor of bone: radiological and histological evaluation. Jpn J Clin Oncol, 2012, 42(9): 851-855.
27.	Scoccianti G, Totti F, Scorianz M, et al. Preoperative denosumab with curettage and cryotherapy in giant cell tumor of bone: Is there an increased risk of local recurrence? Clin Orthop Relat Res, 2018, 476(9): 1783-1790.
28.	Errani C, Tsukamoto S, Leone G, et al. Denosumab may increase the risk of local recurrence in patients with giant-cell tumor of bone treated with curettage. J Bone Joint Surg (Am), 2018, 100(6): 496-504.
29.	Klein MJ, Siegal GP. Osteosarcoma: anatomic and histologic variants. Am J Clin Pathol, 2006, 125(4): 555-581.
30.	Bertrand TE, Cruz A, Binitie O, et al. Do surgical margins affect local recurrence and survival in extremity, nonmetastatic, high-grade osteosarcoma? Clin Orthop Relat Res, 2016, 474(3): 677-683.
31.	Collins M, Wilhelm M, Conyers R, et al. Benefits and adverse events in younger versus older patients receiving neoadjuvant chemotherapy for osteosarcoma: findings from a meta-analysis. J Clin Oncol, 2013, 31(18): 2303-2312.
32.	Wenzel C, Bartsch R, Hussian D, et al. Zoledronate in a patient with pamidronate refractory hypercalcemia syndrome. Support Care Cancer, 2004, 12(9): 678-681.
33.	Sun J, Xu H, Qi M, et al. Identification of key genes in osteosarcoma by meta-analysis of gene expression microarray. Mol Med Rep, 2019, 20(4): 3075-3084.
34.	Calvert GT, Randall RL, Jones KB, et al. At-risk populations for osteosarcoma: the syndromes and beyond. Sarcoma, 2012, 2012: 152382. doi: 10.1155/2012/152382.
35.	Mavrogenis AF, Abati CN, Romagnoli C, et al. Similar survival but better function for patients after limb salvage versus amputation for distal tibia osteosarcoma. Clin Orthop Relat Res, 2012, 470(6): 1735-1748.
36.	Smeland S, Bielack SS, Whelan J, et al. Survival and prognosis with osteosarcoma: outcomes in more than 2000 patients in the EURAMOS-1 (European and American Osteosarcoma Study) cohort. Eur J Cancer, 2019, 109: 36-50.
37.	Duffaud F, Mir O, Boudou-Rouquette P, et al. Efficacy and safety of regorafenib in adult patients with metastatic osteosarcoma: a non-comparative, randomised, double-blind, placebo-controlled, phase 2 study. Lancet Oncol, 2019, 20(1): 120-133.
38.	Davis LE, Bolejack V, Ryan CW, et al. Randomized double-blind phase Ⅱ study of regorafenib in patients with metastatic osteosarcoma. J Clin Oncol, 2019, 37(16): 1424-1431.
39.	Bacci G, Picci P, Mercuri M, et al. Neoadjuvant chemotherapy for high grade malignant fibrous histiocytoma of bone. Clin Orthop Relat Res, 1998, (346): 178-189.
40.	Marabelle A, Fakih M, Lopez J, et al. Association of tumour mutational burden with outcomes in patients with advanced solid tumours treated with pembrolizumab: prospective biomarker analysis of the multicohort, open-label, phase 2 KEYNOTE-158 study. Lancet Oncol, 2020, 21(10): 1353-1365.

1. Bethesda. SEER cancer statistics fact sheets: bone and joint cancer. [EB/OL]. (2019-01-23). [2021-03-05]. http://seer.cancer.gov/statfacts/html/bones.html.
2. Siegel RL, Miller KD, Jemal A. Cancer statistics, 2020. CA Cancer J Clin, 2020, 70(1): 7-30.
3. Bone Cancer (Sarcoma of Bone): Statistics 2020. [EB/OL]. (2020-07-19). [2021-03-05]. https://www.cancer.net/cancer-types/bone-cancer/statistics.
4. Fletcher CDM, Bridge JA, Hogendoorn P, et al. WHO Classification of Tumours of Soft Tissue and Bone. Lyon: IARC Press, 2013.
5. Nicolle R, Ayadi M, Gomez-Brouchet A, et al. Integrated molecular characterization of chondrosarcoma reveals critical determinants of disease progression. Nat Commun, 2019, 10(1): 4622. doi: 10.1038/s41467-019-12525-7.
6. Chow W, Frankel P, Ruel C, et al. Results of a prospective phase 2 study of pazopanib in patients with surgically unresectable or metastatic chondrosarcoma. Cancer, 2020, 126(1): 105-111.
7. Amer KM, Munn M, Congiusta D, et al. Survival and prognosis of chondrosarcoma subtypes: SEER database analysis. J Orthop Res, 2020, 38(2): 311-319.
8. Verdegaal SH, Bovée JV, Pansuriya TC, 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(12): 1771-1779.
9. Schneiderman BA, Kliethermes SA, Nystrom LM. Survival in mesenchymal chondrosarcoma varies based on age and tumor location: A survival analysis of the SEER database. Clin Orthop Relat Res, 2017, 475(3): 799-805.
10. Tap WD, Villalobos VM, Cote GM, et al. Phase Ⅰ study of the mutant IDH1 inhibitor ivosidenib: Safety and clinical activity in patients with advanced chondrosarcoma. J Clin Oncol, 2020, 38(15): 1693-1701.
11. Chugh R, Tawbi H, Lucas DR, et al. Chordoma: the nonsarcoma primary bone tumor. Oncologist, 2007, 12(11): 1344-1350.
12. Shih AR, Cote GM, Chebib I, et al. Clinicopathologic characteristics of poorly differentiated chordoma. Mod Pathol, 2018, 31(8): 1237-1245.
13. McGovern SL, Mahajan A. Progress in radiotherapy for pediatric sarcomas. Curr Oncol Rep, 2012, 14(4): 320-326.
14. Baumann BC, Lustig RA, Mazzoni S, et al. A prospective clinical trial of proton therapy for chordoma and chondrosarcoma: Feasibility assessment. J Surg Oncol, 2019, 120(2): 200-205.
15. Ahmed AT, Abdel-Rahman O, Morsy M, et al. Management of sacrococcygeal chordoma: A systematic review and meta-analysis of observational studies. Spine (Phila Pa 1976), 2018, 43(19): E1157-E1169.
16. Ailon T, Torabi R, Fisher CG, et al. Management of locally recurrent chordoma of the mobile spine and sacrum: A systematic review. Spine (Phila Pa 1976), 2016, 41(Suppl 20): S193-S198.
17. Machado I, Navarro S, Llombart-Bosch A. Ewing sarcoma and the new emerging Ewing-like sarcomas: (CIC and BCOR-rearranged-sarcomas). A systematic review. Histol Histopathol, 2016, 31(11): 1169-1181.
18. Sbaraglia M, Righi A, Gambarotti M, et al. Ewing sarcoma and Ewing-like tumors. Virchows Arch, 2020, 476(1): 109-119.
19. Grevener K, Haveman LM, Ranft A, et al. Management and outcome of ewing sarcoma of the head and neck. Pediatr Blood Cancer, 2016, 63(4): 604-610.
20. Bosma SE, Ayu O, Fiocco M, et al. Prognostic factors for survival in Ewing sarcoma: A systematic review. Surg Oncol, 2018, 27(4): 603-610.
21. Grier HE, Krailo MD, Tarbell NJ, et al. Addition of ifosfamide and etoposide to standard chemotherapy for Ewing’s sarcoma and primitive neuroectodermal tumor of bone. N Engl J Med, 2003, 348(8): 694-701.
22. Ronchi L, Buwenge M, Cortesi A, et al. Whole lung irradiation in patients with osteosarcoma and ewing sarcoma. Anticancer Res, 2018, 38(9): 4977-4985.
23. Whelan J, Le Deley MC, Dirksen U, et al. High-dose chemotherapy and blood autologous stem-cell rescue compared with standard chemotherapy in localized high-risk ewing sarcoma: results of Euro-E. W. I. N. G. 99 and Ewing-2008. J Clin Oncol, 2018, 36(31): JCO2018782516. doi: 10.1200/JCO.2018.78.2516.
24. Italiano A, Mir O, Mathoulin-Pelissier S, et al. Cabozantinib in patients with advanced Ewing sarcoma or osteosarcoma (CABONE): a multicentre, single-arm, phase 2 trial. Lancet Oncol, 2020, 21(3): 446-455.
25. van der Heijden L, Dijkstra PDS, Blay JY, et al. Giant cell tumour of bone in the denosumab era. Eur J Cancer, 2017, 77: 75-83.
26. Emori M, Kaya M, Sasaki M, et al. Pre-operative selective arterial embolization as a neoadjuvant therapy for proximal humerus giant cell tumor of bone: radiological and histological evaluation. Jpn J Clin Oncol, 2012, 42(9): 851-855.
27. Scoccianti G, Totti F, Scorianz M, et al. Preoperative denosumab with curettage and cryotherapy in giant cell tumor of bone: Is there an increased risk of local recurrence? Clin Orthop Relat Res, 2018, 476(9): 1783-1790.
28. Errani C, Tsukamoto S, Leone G, et al. Denosumab may increase the risk of local recurrence in patients with giant-cell tumor of bone treated with curettage. J Bone Joint Surg (Am), 2018, 100(6): 496-504.
29. Klein MJ, Siegal GP. Osteosarcoma: anatomic and histologic variants. Am J Clin Pathol, 2006, 125(4): 555-581.
30. Bertrand TE, Cruz A, Binitie O, et al. Do surgical margins affect local recurrence and survival in extremity, nonmetastatic, high-grade osteosarcoma? Clin Orthop Relat Res, 2016, 474(3): 677-683.
31. Collins M, Wilhelm M, Conyers R, et al. Benefits and adverse events in younger versus older patients receiving neoadjuvant chemotherapy for osteosarcoma: findings from a meta-analysis. J Clin Oncol, 2013, 31(18): 2303-2312.
32. Wenzel C, Bartsch R, Hussian D, et al. Zoledronate in a patient with pamidronate refractory hypercalcemia syndrome. Support Care Cancer, 2004, 12(9): 678-681.
33. Sun J, Xu H, Qi M, et al. Identification of key genes in osteosarcoma by meta-analysis of gene expression microarray. Mol Med Rep, 2019, 20(4): 3075-3084.
34. Calvert GT, Randall RL, Jones KB, et al. At-risk populations for osteosarcoma: the syndromes and beyond. Sarcoma, 2012, 2012: 152382. doi: 10.1155/2012/152382.
35. Mavrogenis AF, Abati CN, Romagnoli C, et al. Similar survival but better function for patients after limb salvage versus amputation for distal tibia osteosarcoma. Clin Orthop Relat Res, 2012, 470(6): 1735-1748.
36. Smeland S, Bielack SS, Whelan J, et al. Survival and prognosis with osteosarcoma: outcomes in more than 2000 patients in the EURAMOS-1 (European and American Osteosarcoma Study) cohort. Eur J Cancer, 2019, 109: 36-50.
37. Duffaud F, Mir O, Boudou-Rouquette P, et al. Efficacy and safety of regorafenib in adult patients with metastatic osteosarcoma: a non-comparative, randomised, double-blind, placebo-controlled, phase 2 study. Lancet Oncol, 2019, 20(1): 120-133.
38. Davis LE, Bolejack V, Ryan CW, et al. Randomized double-blind phase Ⅱ study of regorafenib in patients with metastatic osteosarcoma. J Clin Oncol, 2019, 37(16): 1424-1431.
39. Bacci G, Picci P, Mercuri M, et al. Neoadjuvant chemotherapy for high grade malignant fibrous histiocytoma of bone. Clin Orthop Relat Res, 1998, (346): 178-189.
40. Marabelle A, Fakih M, Lopez J, et al. Association of tumour mutational burden with outcomes in patients with advanced solid tumours treated with pembrolizumab: prospective biomarker analysis of the multicohort, open-label, phase 2 KEYNOTE-158 study. Lancet Oncol, 2020, 21(10): 1353-1365.

Chinese Journal of Reparative and Reconstructive Surgery

Update and interpretation of 2021 National Comprehensive Cancer Network (NCCN) “Clinical Practice Guidelines for Bone Tumors”

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