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Bone Sarcoma pp 131–152Cite as

Rare Bone Sarcomas

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Part of the book series: MD Anderson Cancer Care Series ((MDCCS))

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Rare tumors of bone include chordoma, adamantinoma, hemangioendothelioma, hemangiopericytoma, low-grade fibrosarcoma of bone, and malignant fibrous histiocytoma (MFH). These are distinctive and interesting diseases with unique features. They bear some relationship to a spectrum of different mesenchymal tissues. Chordomas are probably derived from notochordal cells and thus are found primarily in the sacrum and sphenoocciput. Adamantinomas, on the other hand, are found predominantly in the tibia and show a peculiar tendency toward epithelial differentiation. The other tumors are more widely located within the body; hemangioendothelioma and hemangiopericytoma are tumors related to vascular tissue, whereas low-grade fibrosarcoma and MFH are most likely derived from spindle-shaped, fibroblast-like cells.

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Suggested Readings

  • Adler B, Naheedy J, Yeager N, et al. Multifocal epithelioid hemangioendothelioma in a 16-year-old boy. Pediatr Radiol. 2005;35:1014–8.

    Article  PubMed  Google Scholar 

  • Bacci G, Ferrari S, Bertoni F, et al. Neoadjuvant chemotherapy for osseous malignant fibrous histiocytoma of the extremity: results in 18 cases and comparison with 112 contemporary osteosarcoma patients treated with the same chemotherapy regimen. J Chemother. 1997;9:293–9.

    PubMed  CAS  Google Scholar 

  • Bacci G, Picci P, Mercuri M, Bertoni F, Ferrari S. Neoadjuvant chemotherapy for high grade malignant fibrous histiocytoma of bone. Clin Orthop Relat Res. 1998;346:178–89.

    Article  PubMed  Google Scholar 

  • Bergh P, Kindblom LG, Gunterberg B, Remotti F, Ryd W, Meis-Kindblom JM. Prognostic factors in chordoma of the sacrum and mobile spine: a study of 39 patients. Cancer. 2000;88:2122–34.

    Article  PubMed  CAS  Google Scholar 

  • Bertoni F, Capanna R, Biagini R, et al. Malignant fibrous histiocytoma of soft tissue. An analysis of 78 cases located and deeply seated in the extremities. Cancer. 1985;56:356–67.

    Article  PubMed  CAS  Google Scholar 

  • Bertoni F, Capanna R, Calderoni P, et al. Primary central (medullary) fibrosarcoma of bone. Semin Diagn Pathol. 1984;1:185–98.

    PubMed  CAS  Google Scholar 

  • Bielack SS, Schroeders A, Fuchs N, et al. Malignant fibrous histiocytoma of bone: a retrospective EMSOS study of 125 cases. European Musculo-Skeletal Oncology Society. Acta Orthop Scand. 1999;70:353–60.

    Article  PubMed  CAS  Google Scholar 

  • Bohinski RJ, Rhines LD. Principles and techniques of en bloc vertebrectomy for bone tumors of the thoracolumbar spine: an overview. Neurosurg Focus. 2003;15:E7.

    Article  PubMed  Google Scholar 

  • Boriani S, Bandiera S, Biagini R, et al. Chordoma of the mobile spine: fifty years of experience. Spine. 2006;31:493–503.

    Article  PubMed  Google Scholar 

  • Boriani S, Biagini R, De Iure F, et al. En bloc resections of bone tumors of the thoracolumbar spine. A preliminary report on 29 patients. Spine. 1996;21:1927–31.

    Article  PubMed  CAS  Google Scholar 

  • Boriani S, De Iure F, Bandiera S, et al. Chondrosarcoma of the mobile spine: report on 22 cases. Spine. 2000;25:804–12.

    Article  PubMed  CAS  Google Scholar 

  • Boriani S, Weinstein JN, Biagini R. Primary bone tumors of the spine. Terminology and surgical staging. Spine. 1997;22:1036–44.

    Article  PubMed  CAS  Google Scholar 

  • Bramwell VH, Steward WP, Nooij M, et al. Neoadjuvant chemotherapy with doxorubicin and cisplatin in malignant fibrous histiocytoma of bone: a European Osteosarcoma Intergroup study. J Clin Oncol. 1999;17:3260–9.

    PubMed  CAS  Google Scholar 

  • Campanacci M, Boriani S, Giunti A. Hemangioendothelioma of bone. Cancer. 1980;46:804–14.

    Article  PubMed  CAS  Google Scholar 

  • Campanacci M, Giunti A, Bertoni F, et al. Adamantinoma of the long bones. The experience at the Istituto Ortopedico Rizzoli. Am J Surg Pathol. 1981;5:533–42.

    Article  PubMed  CAS  Google Scholar 

  • Capanna R, Bertoni F, Bacchini P, et al. Malignant fibrous histiocytoma of bone. The experience at the Rizzoli Institute: report of 90 cases. Cancer. 1984;54:177–87.

    Article  PubMed  CAS  Google Scholar 

  • Celik I, Bascil N, Yalcin S, et al. Ifosfamide-based chemotherapy for recurrent or metastatic hemangiopericytoma. Acta Oncol. 1997;36:348.

    Article  PubMed  CAS  Google Scholar 

  • Cole BJ, Schultz E, Smilari TF, et al. Malignant fibrous histiocytoma at the site of a total hip replacement: review of the literature and case report. Skeletal Radiol. 1997;26:559–63.

    Article  PubMed  CAS  Google Scholar 

  • Conway WF, Hayes CW. Miscellaneous lesions of bone. Radiol Clin North Am. 1993;31:339–58.

    PubMed  CAS  Google Scholar 

  • Dahlin DC, Unni KK. Bone tumors. 4th ed. Springfield: Charles C. Thomas; 1986. p. 357–65.

    Google Scholar 

  • Dahlin DC, Unni KK, Matsuno T. Malignant (fibrous) histiocytoma of bone—fact or fancy? Cancer. 1977;39:1508–16.

    Article  PubMed  CAS  Google Scholar 

  • Debus J, Schulz-Ertner D, Schad L, et al. Stereotactic fractionated radiotherapy for chordomas and chondrosarcomas of the skull base. Int J Radiat Oncol Biol Phys. 2000;47:591–6.

    Article  PubMed  CAS  Google Scholar 

  • Dorfman HD, Czerniak B. Bone tumors. St. Louis: Mosby; 1997.

    Google Scholar 

  • Dunham WK, Wilborn WH. Malignant fibrous histiocytoma of bone. Report of two cases and review of the literature. J Bone Joint Surg Am. 1979;61:939–42.

    PubMed  CAS  Google Scholar 

  • Enneking WF. A system of staging musculoskeletal neoplasms. Clin Orthop Relat Res. 1986;204:9–24.

    PubMed  Google Scholar 

  • Enneking WF, Spanier SS, Goodman MA. A system for the surgical staging of musculoskeletal sarcoma. Clin Orthop. 1980;153:106–20.

    PubMed  Google Scholar 

  • Enzinger FM, Smith BH. Hemangiopericytoma. An analysis of 106 cases. Hum Pathol. 1976;7:61–82.

    Article  PubMed  CAS  Google Scholar 

  • Faria SL, Schlupp WR, Chiminazzo H. Radiotherapy in the treatment of vertebral hemangiomas. Int J Radiat Oncol Biol Phys. 1985;1:387–90.

    Article  Google Scholar 

  • Ferigo N, Cottalorda J, Allard D, et al. Successful treatment via chemotherapy and surgical resection of a femoral hemangiopericytoma with pulmonary metastasis. J Pediatr Hematol Oncol. 2006;28:237–40.

    Article  PubMed  CAS  Google Scholar 

  • Fourney DR, Rhines LD, Hentschel SJ, et al. En bloc resection of primary sacral tumors: classification of surgical approaches and outcome. J Neurosurg Spine. 2005;3:111–22.

    Article  PubMed  Google Scholar 

  • Gebhardt MC, Lord FC, Rosenberg AE, et al. The treatment of adamantinoma of the tibia by wide resection and allograft bone transplantation. J Bone Joint Surg Am. 1987;69:1177–88.

    PubMed  CAS  Google Scholar 

  • Gosheger G, Hardes J, Ozaki T, et al. The multicentric epitheloid hemangioendothelioma of bone: a case example and review of the literature. J Cancer Res Clin Oncol. 2002;128:11–8.

    Article  PubMed  CAS  Google Scholar 

  • Haisley-Royster C, Enjolras O, Frieden IJ, et al. Kasabach–Merritt phenomenon: a retrospective study of treatment with vincristine. J Pediatr Hematol Oncol. 2002;24:459–62.

    Article  PubMed  Google Scholar 

  • Hazelbag HM, Taminiau AH, Fleuren GJ, et al. Adamantinoma of the long bones. A clinicopathological study of thirty-two patients with emphasis on histological subtype, precursor lesion, and biological behavior. J Bone Joint Surg Am. 1994;76:1482–99.

    PubMed  CAS  Google Scholar 

  • Healey JH, Lane JM. Chordoma: a critical review of diagnosis and treatment. Orthop Clin North Am. 1989;20:417–26.

    PubMed  CAS  Google Scholar 

  • Heymans O, Gebhart M, Larsimont D, et al. Malignant hemangiopericytoma of the pelvis: treatment using internal hemipelvectomy. Acta Orthop Belg. 1997;63:40–2.

    PubMed  CAS  Google Scholar 

  • Hug EB, Fitzek MM, Liebsch NJ, et al. Locally challenging osteo- and chondrogenic tumors of the axial skeleton: results of combined proton and photon radiation therapy using three-dimensional treatment planning. Int J Radiat Oncol Biol Phys. 1995;31:467–76.

    Article  PubMed  CAS  Google Scholar 

  • Huvos AG, Heilweil M, Bretsky SS. The pathology of malignant fibrous histiocytoma of bone: a study of 130 patients. Am J Surg Pathol. 1985;9:853–71.

    Article  PubMed  CAS  Google Scholar 

  • Igaki H, Tokuuye K, Okumura T, et al. Clinical results of proton beam therapy for skull base chordoma. Int J Radiat Oncol Biol Phys. 2004;60:1120–6.

    Article  PubMed  Google Scholar 

  • Ignacio EA, Palmer KM, Mathur SC, et al. Epithelioid hemangioendothelioma of the lower extremity. Radiographics. 1999;19:531–7.

    PubMed  CAS  Google Scholar 

  • Juan CJ, Huang GS, Hsueh CJ, et al. Primary hemangiopericytoma of the tibia: MR and angiographic correlation. Skeletal Radiol. 2000;29:49–53.

    Article  PubMed  CAS  Google Scholar 

  • Keeney GL, Unni KK, Beabout JW, et al. Adamantinoma of long bones. A clinicopathologic study of 85 cases. Cancer. 1989;64:730–7.

    Article  PubMed  CAS  Google Scholar 

  • Kleer CG, Unni KK, McLeod RA. Epithelioid hemangioendothelioma of bone. Am J Surg Pathol. 1996;20:1301–11.

    Article  PubMed  CAS  Google Scholar 

  • Marmor E, Rhines LD, Weinberg JS, et al. Total en bloc lumbar spondylectomy. Case report. J Neurosurg. 2001;95 suppl 2:264–9.

    PubMed  CAS  Google Scholar 

  • Mirra JM. Hemangiopericytoma. In: Mirra JM, Picci P, Gold RH, editors. Bone tumors: diagnosis and treatment. Philadelphia: Lea & Febiger; 1989. p. 1436–53.

    Google Scholar 

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

    PubMed  CAS  Google Scholar 

  • Moon NF, Mori H. Adamantinoma of the appendicular skeleton—updated. Clin Orthop Relat Res. 1986;204:215–37.

    PubMed  Google Scholar 

  • Mori H, Shima R, Nakanishi H, et al. Adamantinoma of the tibia—a case report and a statistical review of reported cases. Acta Pathol Jpn. 1981;31:701–9.

    PubMed  CAS  Google Scholar 

  • Nishida J, Sim FH, Wenger DE, et al. Malignant fibrous histiocytoma of bone. A clinicopathologic study of 81 patients. Cancer. 1997;79:482–93.

    Article  PubMed  CAS  Google Scholar 

  • Noel G, Feuvret L, Calugaru V, et al. Chordomas of the base of the skull and upper cervical spine. One hundred patients irradiated by a 3D conformal technique combining photon and proton beams. Acta Oncol. 2005;44:700–8.

    Article  PubMed  Google Scholar 

  • Park L, Delaney TF, Liebsch NJ, et al. Sacral chordomas: Impact of high-dose proton/photon-beam radiation therapy combined with or without surgery for primary versus recurrent tumor. Int J Radiat Oncol Biol Phys. 2006;65:1514–21.

    Article  PubMed  Google Scholar 

  • Picci P, Bacci G, Ferrari S, et al. Neoadjuvant chemotherapy in malignant fibrous histiocytoma of bone and in osteosarcoma located in the extremities: analogies and differences between the two tumors. Ann Oncol. 1997;8:1107–15.

    Article  PubMed  CAS  Google Scholar 

  • Qureshi AA, Shott S, Mallin BA, et al. Current trends in the management of adamantinoma of long bones. An international study. J Bone Joint Surg Am. 2000;82:1122–31.

    PubMed  Google Scholar 

  • Rich TA, Schiller A, Suit HD, et al. Clinical and pathologic review of 48 cases of chordoma. Cancer. 1985;56:182–7.

    Article  PubMed  CAS  Google Scholar 

  • Rosenthal DI, Treat ME, Mankin HJ, et al. Treatment of epithelioid hemangioendothelioma of bone using a novel combined approach. Skeletal Radiol. 2001;30:219–22.

    Article  PubMed  CAS  Google Scholar 

  • Sahin-Akyar G, Fitoz S, Akpolat I, et al. Primary hemangiopericytoma of bone located in the tibia. Skeletal Radiol. 1997;26:47–50.

    Article  PubMed  CAS  Google Scholar 

  • Saito T, Oda Y, Tanaka K, et al. Low-grade fibrosarcoma of the proximal humerus. Pathol Int. 2003;53:115–20.

    Article  PubMed  Google Scholar 

  • Schuh A, Zeiler G, Holzwarth U, et al. Malignant fibrous histiocytoma at the site of a total hip arthroplasty. Clin Orthop Relat Res. 2004;425:218–22.

    Article  PubMed  Google Scholar 

  • Sundaresan N, Rosen G, Boriani S. Primary malignant tumors of the spine. Orthop Clin North Am. 2009;40:21–36, v.

    Article  PubMed  Google Scholar 

  • Taconis WK, Mulder JD. Fibrosarcoma and malignant fibrous histiocytoma of long bones: radiographic features and grading. Skeletal Radiol. 1984;11:237–45.

    Article  PubMed  CAS  Google Scholar 

  • Tang JS, Gold RH, Mirra JM, et al. Hemangiopericytoma of bone. Cancer. 1988;62:848–59.

    Article  PubMed  CAS  Google Scholar 

  • Tillman RM, Choong PF, Beabout JW, et al. Epithelioid hemangioendothelioma of bone. Orthopedics. 1997;20:177–80.

    PubMed  CAS  Google Scholar 

  • Tomita K, Kawahara N, Baba H, et al. Total en bloc spondylectomy. A new surgical technique for primary malignant vertebral tumors. Spine. 1997;22:324–33.

    Article  PubMed  CAS  Google Scholar 

  • Troop JK, Mallory TH, Fisher DA, et al. Malignant fibrous histiocytoma after total hip arthroplasty. A case report. Clin Orthop Relat Res. 1990;253:297–300.

    PubMed  Google Scholar 

  • Tsuneyoshi M, Dorfman HD, Bauer TW. Epithelioid hemangioendothelioma of bone. A clinicopathologic, ultrastructural, and immunohistochemical study. Am J Surg Pathol. 1986;10:754–64.

    Article  PubMed  CAS  Google Scholar 

  • Unni KK. Dahlin’s bone tumors: general aspects and data on 11,087 cases. 5th ed. New York: Lippincott-Raven; 1996.

    Google Scholar 

  • Weber DC, Rutz HP, Pedroni ES, et al. Results of spot-scanning proton radiation therapy for chordoma and chondrosarcoma of the skull base: the Paul Scherrer Institut experience. Int J Radiat Oncol Biol Phys. 2005;63:401–9.

    Article  PubMed  Google Scholar 

  • Weiss SW, Enzinger FM. Epithelioid hemangioendothelioma: a vascular tumor often mistaken for a carcinoma. Cancer. 1982;50:970–81.

    Article  PubMed  CAS  Google Scholar 

  • Welles L, Dorfman H, Valentine ES, et al. Low grade malignant hemangioendothelioma of bone: a disease potentially curable with radiotherapy. Med Pediatr Oncol. 1994;23:144–8.

    Article  PubMed  CAS  Google Scholar 

  • Wold LE, Unni KK, Beabout JW, et al. Hemangioendothelial sarcoma of bone. Am J Surg Pathol. 1982;6:59–70.

    Article  PubMed  CAS  Google Scholar 

  • York JE, Kaczaraj A, Abi-Said D, et al. Sacral chordoma: 40-year experience at a major cancer center. Neurosurgery. 1999;44:74–9 [discussion 79–80].

    Article  PubMed  CAS  Google Scholar 

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Correspondence to Valerae O. Lewis .

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Lewis, V.O., Guadagnolo, A., Rhines, L.D., Patel, S. (2013). Rare Bone Sarcomas. In: Lin, P., Patel, S. (eds) Bone Sarcoma. MD Anderson Cancer Care Series. Springer, Boston, MA. https://doi.org/10.1007/978-1-4614-5194-5_8

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