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Conventional Central Osteosarcoma

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Tumors and Tumor-Like Lesions of Bone

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Abstract

Conventional central osteosarcoma (CCO) is a high-grade intraosseous malignant bone tumor. The neoplastic cells produce osteoid or bone. A CCO is considered to be primary when the host bone is normal. A CCO is said to be secondary when the host bone is affected by other pathologic conditions. There is a slight male predominance. It has a bimodal age distribution: the majority of cases are diagnosed between 10 and 14 years of age, and there is a second smaller peak in older adults (>40 years old). CCOs originate in the long bone metaphysis (90%), especially in the knee region and proximal humerus (the locations with the most proliferative growth plates). The radiographic appearance of primary CCO varies greatly, depending on the amount of ossification/calcification and the amount of the lytic component. Tumors may be completely lytic or predominantly sclerotic, but they usually demonstrate a combination of these features. The presence of a purely sarcomatous stroma and the direct formation of tumoral osteoid and bone by the malignant connective tissue cells are the characteristic histological features. Histologic subtypes (variants) of primary CCO are osteoblastic (sclerotic type), chondroblastic, fibroblastic (malignant fibrous histiocytoma-like), giant cell-rich, osteoblastoma-like, epithelioid, chondroblastoma-like, telangiectatic/hemorrhagic, small cell, chondromyxoid fibroma-like, plasmacytoma-like, and multicentric (synchronous and metachronous). Treatment includes preoperative chemotherapy (neoadjuvant). The surgical approach is through surgical resection with wide surgical margins (negative margins). The biopsy tract must be removed with the tumor. Amputation is done if necessary, as well as post-op chemotherapy.

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Suggested Reading

  • Amary MF, Bacsi K, Maggiani F, Damato S, Halai D, Berisha F, et al. IDH1 and IDH2 mutations are frequent events in central chondrosarcoma and central and periosteal chondromas but not in other mesenchymal tumours. J Pathol. 2011;224(3):334–43.

    Article  CAS  PubMed  Google Scholar 

  • Arlen M, Higinbotham NL, Huvos AG, Marcove RC, Miller T, Shah IC. Radiation-induced sarcoma of bone. Cancer. 1971;28(5):1087–99.

    Article  CAS  PubMed  Google Scholar 

  • Bacchini P, Inwards C, Biscaglia R, Picci P, Bertoni F. Chondroblastoma-like osteosarcoma. Orthopedics. 1999;22(3):337–9.

    CAS  PubMed  Google Scholar 

  • Bacci G, Longhi A, Cesari M, Versari M, Bertoni F. Influence of local recurrence on survival in patients with extremity osteosarcoma treated with neoadjuvant chemotherapy: the experience of a single institution with 44 patients. Cancer. 2006;106(12):2701–6.

    Article  PubMed  Google Scholar 

  • 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(12):1479–82.

    Article  CAS  PubMed  Google Scholar 

  • Bertoni F, Unni KK, McLeod RA, Dahlin DC. Osteosarcoma resembling osteoblastoma. Cancer. 1985;55(2):416–26.

    Article  CAS  PubMed  Google Scholar 

  • Bertoni F, Bacchini P, Fabbri N, Mercuri M, Picci P, Ruggieri P, et al. Osteosarcoma. Low-grade intraosseous-type osteosarcoma, histologically resembling parosteal osteosarcoma, fibrous dysplasia, and desmoplastic fibroma. Cancer. 1993;71(2):338–45.

    Article  CAS  PubMed  Google Scholar 

  • Bertoni F, Bacchini P, Donati D, Martini A, Picci P, Campanacci M. Osteoblastoma-like osteosarcoma. The Rizzoli Institute experience. Mod Pathol. 1993;6(6):707–16.

    CAS  PubMed  Google Scholar 

  • Bertoni F, Bacchini P, Staals EL. Giant cell-rich osteosarcoma. Orthopedics. 2003;26(2):179–81.

    PubMed  Google Scholar 

  • Campanacci M, Bertoni F, Capanna R, Cervellati C. Central osteosarcoma of low grade malignancy. Ital J Orthop Traumatol. 1981;7(1):71–8.

    CAS  PubMed  Google Scholar 

  • Corradi D, Wenger DE, Bertoni F, Bacchini P, Bosio S, Goldoni M, et al. Multicentric osteosarcoma: clinicopathologic and radiographic study of 56 cases. Am J Clin Pathol. 2011;136(5):799–807.

    Article  PubMed  Google Scholar 

  • Dahlin DC, Coventry MB. Osteogenic sarcoma. A study of six hundred cases. J Bone Joint Surg Am. 1967;49(1):101–10.

    Article  CAS  PubMed  Google Scholar 

  • Dujardin F, Binh MB, Bouvier C, Gomez-Brouchet A, Larousserie F, de Muret A, et al. MDM2 and CDK4 immunohistochemistry is a valuable tool in the differential diagnosis of low-grade osteosarcomas and other primary fibro-osseous lesions of the bone. Mod Pathol. 2011;24(5):624–37.

    Article  CAS  PubMed  Google Scholar 

  • Enneking WF, Kagan A. “Skip” metastases in osteosarcoma. Cancer. 1975;36(6):2192–205.

    Article  CAS  PubMed  Google Scholar 

  • Farr GH, Huvos AG, Marcove RC, Higinbotham NL, Foote FW Jr. Telangiectatic osteogenic sarcoma. A review of twenty-eight cases. Cancer. 1974;34(4):1150–8.

    Article  CAS  PubMed  Google Scholar 

  • Fitzgerald RH Jr, Dahlin DC, Sim FH. Multiple metachronous osteogenic sarcoma. Report of twelve cases with two long-term survivors. J Bone Joint Surg Am. 1973;55(3):595–605.

    Article  PubMed  Google Scholar 

  • Fletcher C, Bridge JA, Hogendoorn PC, Mertens F. WHO classification of tumours of soft tissue and bone. Lyon: IARC; 2013. p. 281–91.

    Google Scholar 

  • Glasser DB, Lane JM, Huvos AG, Marcove RC, Rosen G. Survival, prognosis, and therapeutic response in osteogenic sarcoma. The Memorial Hospital experience. Cancer. 1992;69(3):698–708.

    Article  CAS  PubMed  Google Scholar 

  • Horvai A, Unni KK. Premalignant conditions of bone. J Orthop Sci. 2006;11(4):412–23.

    Article  PubMed  PubMed Central  Google Scholar 

  • Huvos AG, Higinbotham NL, Miller TR. Bone sarcomas arising in fibrous dysplasia. J Bone Joint Surg Am. 1972;54(5):1047–56.

    Article  CAS  PubMed  Google Scholar 

  • Huvos AG, Rosen G, Bretsky SS, Butler A. Telangiectatic osteogenic sarcoma: a clinicopathologic study of 124 patients. Cancer. 1982;49(8):1679–89.

    Article  CAS  PubMed  Google Scholar 

  • Kurt AM, Unni KK, McLeod RA, Pritchard DJ. Low-grade intraosseous osteosarcoma. Cancer. 1990;65(6):1418–28.

    Article  CAS  PubMed  Google Scholar 

  • Marcove RC, Sheth DS, Healey J, Huvos A, Rosen G, Meyers P. Limb-sparing surgery for extremity sarcoma. Cancer Investig. 1994;12(5):497–504.

    Article  CAS  Google Scholar 

  • Matsuno T, Unni KK, McLeod RA, Dahlin DC. Telangiectatic osteogenic sarcoma. Cancer. 1976;38(6):2538–47.

    Article  CAS  PubMed  Google Scholar 

  • Mervak TR, Unni KK, Pritchard DJ, McLeod RA. Telangiectatic osteosarcoma. Clin Orthop Relat Res. 1991;(270):135–9.

    Google Scholar 

  • Mirra JM, Bullough PG, Marcove RC, Jacobs B, Huvos AG. Malignant fibrous histiocytoma and osteosarcoma in association with bone infarcts; report of four cases, two in caisson workers. J Bone Joint Surg Am. 1974;56(5):932–40.

    Article  CAS  PubMed  Google Scholar 

  • Nakajima H, Sim FH, Bond JR, Unni KK. Small cell osteosarcoma of bone. Review of 72 cases. Cancer. 1997;79(11):2095–106.

    Article  CAS  PubMed  Google Scholar 

  • Nora FE, Unni KK, Pritchard DJ, Dahlin DC. Osteosarcoma of extragnathic craniofacial bones. Mayo Clin Proc. 1983;58(4):268–72.

    CAS  PubMed  Google Scholar 

  • Oliveira AM, Perez-Atayde AR, Inwards CY, Medeiros F, Derr V, Hsi BL, et al. USP6 and CDH11 oncogenes identify the neoplastic cell in primary aneurysmal bone cysts and are absent in so-called secondary aneurysmal bone cysts. Am J Pathol. 2004;165:1773–80.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Pierron G, Tirode F, Lucchesi C, Reynaud S, Ballet S, Cohen-Gogo S, et al. A new subtype of bone sarcoma defined by BCOR-CCNB3 gene fusion. Nat Genet. 2012;44:461–6.

    Article  CAS  PubMed  Google Scholar 

  • Porretta CA, Dahlin DC, Janes JM. Sarcoma in Paget’s disease of bone. J Bone Joint Surg Am. 1957;39-A(6):1314–29.

    Article  CAS  PubMed  Google Scholar 

  • Rosen G, Tan C, Sanmaneechai A, Beattie EJ Jr, Marcove R, Murphy ML. The rationale for multiple drug chemotherapy in the treatment of osteogenic sarcoma. Cancer. 1975;35(3 suppl):936–45.

    Article  CAS  PubMed  Google Scholar 

  • Rosen G, Marcove RC, Caparros B, Nirenberg A, Kosloff C, Huvos AG. Primary osteogenic sarcoma: the rationale for preoperative chemotherapy and delayed surgery. Cancer. 1979;43(6):2163–77.

    Article  CAS  PubMed  Google Scholar 

  • Rosen G, Caparros B, Huvos AG, Kosloff C, Nirenberg A, Cacavio A, et al. Preoperative chemotherapy for osteogenic sarcoma: selection of postoperative adjuvant chemotherapy based on the response of the primary tumor to preoperative chemotherapy. Cancer. 1982;49(6):1221–30.

    Article  CAS  PubMed  Google Scholar 

  • Sim FH, Cupps RE, Dahlin DC, Ivins JC. Postradiation sarcoma of bone. J Bone Joint Surg Am. 1972;54(7):1479–89.

    Article  CAS  PubMed  Google Scholar 

  • Sim FH, Unni KK, Beabout JW, Dahlin DC. Osteosarcoma with small cells simulating Ewing’s tumor. J Bone Joint Surg Am. 1979;61(2):207–15.

    Article  CAS  PubMed  Google Scholar 

  • Tabareau-Delalande F, Collin C, Gomez-Brouchet A, Decouvelaere AV, Bouvier C, Larousserie F, et al. Diagnostic value of investigating GNAS mutations in fibro-osseous lesions: a retrospective study of 91 cases of fibrous dysplasia and 40 other fibro-osseous lesions. Mod Pathol. 2013;26(7):911–21.

    Article  CAS  PubMed  Google Scholar 

  • Tarkkanen M, Böhling T, Gamberi G, Ragazzini P, Benassi MS, Kivioja A, et al. Comparative genomic hybridization of low-grade central osteosarcoma. Mod Pathol. 1998;11(5):421–6.

    CAS  PubMed  Google Scholar 

  • Unni KK, Dahlin DC, McLeod RA, Pritchard DJ. Intraosseous well-differentiated osteosarcoma. Cancer. 1977;40(3):1337–47.

    Article  CAS  PubMed  Google Scholar 

  • Unni KK, Inwards CY. Dahlin’s bone tumors: general aspects and data on 11,087 cases. 6th ed. Philadelphia: Lippincott Williams & Williams; 2010. p. 122–57.

    Google Scholar 

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Author information

Authors and Affiliations

  1. University of Bologna, Villa Erbosa Hospital, Bologna, Italy

    Franco Bertoni

  2. Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA

    Jodi M. Carter

  3. Villa Erbosa Hospital, Bologna, Italy

    Patrizia Bacchini

Authors
  1. Franco Bertoni
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  2. Jodi M. Carter
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  3. Patrizia Bacchini
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Corresponding author

Correspondence to Jodi M. Carter .

Editor information

Editors and Affiliations

  1. Department of Pathology, University of Buenos Aires, Buenos Aires, Argentina

    Eduardo Santini-Araujo

  2. Laboratory of Orthopaedic Pathology, Buenos Aires, Argentina

    Ricardo K. Kalil

  3. Pathology, BBTEAM Pathology, Bologna, Italy

    Franco Bertoni

  4. Department of Pathology, Kyung Hee University Hospital, Seoul, Korea (Republic of)

    Yong-Koo Park

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Bertoni, F., Carter, J.M., Bacchini, P. (2020). Conventional Central Osteosarcoma. In: Santini-Araujo, E., Kalil, R.K., Bertoni, F., Park, YK. (eds) Tumors and Tumor-Like Lesions of Bone. Springer, Cham. https://doi.org/10.1007/978-3-030-28315-5_13

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  • DOI: https://doi.org/10.1007/978-3-030-28315-5_13

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-28314-8

  • Online ISBN: 978-3-030-28315-5

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