Angiosarcoma is a high-grade malignant neoplasia characterized by cells with endothelial phenotype. Primary angiosarcoma of bone is rare and accounts for little more than 1% of primary bone sarcomas. Affected individuals have a broad age range; most are in the seventh decade of life, and males are affected slightly more frequently than females. Clinically, it usually presents as a painful, enlarging mass, which may cause pathologic fracture. Angiosarcoma has a wide skeletal distribution, with most lesions originating in the long bones and pelvis. Approximately 30% of tumors are multifocal. The neoplastic cells express endothelial markers; its epithelioid variant also stains with epithelial markers. Angiosarcoma is a very aggressive neoplasm, and the prognosis is dismal in most cases.


Angiosarcoma Bone Malignant vascular tumor 

Suggested Reading

  1. Abdelwahab IF, Klein MJ, Hermann G, Springfield D. Angiosarcomas associated with bone infarcts. Skelet Radiol. 1998;27:546–51.Google Scholar
  2. Abraham JA, Hornicek FJ, Kaufman AM, Harmon DC, Springfield DS, Raskin KA, et al. Treatment and outcome of 82 patients with angiosarcoma. Ann Surg Oncol. 2007;14:1953–67.CrossRefGoogle Scholar
  3. Errani C, Vanel D, Gambarotti M, Alberghini M, Picci P, Faldini C. Vascular bone tumors: a proposal of a classification based on clinicopathological, radiographic and genetic features. Skelet Radiol. 2012;41:1495–507.CrossRefGoogle Scholar
  4. Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F, editors. WHO classification of tumors of soft tissue and bone. 4th ed. Lyon: IARC Press; 2013.Google Scholar
  5. Hart JL, Edgar MA, Gardner JM. Vascular tumors of bone. Semin Diagn Pathol. 2014;31:30–8.CrossRefGoogle Scholar
  6. Liu L, Kakiuchi-Kiyota S, Arnold LL, Johansson SL, Wert D, Cohen SM. Pathogenesis of human hemangiosarcomas and hemangiomas. Hum Pathol. 2013;44:2302–11.CrossRefGoogle Scholar
  7. Mittal S, Goswami C, Kanoria N, Bhattacharya A. Post-irradiation angiosarcoma of bone. J Cancer Res Ther. 2007;3:96–9.CrossRefGoogle Scholar
  8. Palmerini E, Maki RG, Staals EL, Alberghini M, Antonescu CR, Ferrari C, et al. Primary angiosarcoma of bone: a retrospective analysis of 60 patients from 2 institutions. Am J Clin Oncol. 2014;37:528–34.CrossRefGoogle Scholar
  9. van IJzendoorn DGP, JVMG B. Vascular tumors of bone: the evolvement of a classification based on molecular developments. Surg Pathol Clin. 2017;10:621–35.CrossRefGoogle Scholar
  10. Verbeke SL, Bertoni F, Bacchini P, Sciot R, Fletcher CD, Kroon HM, et al. Distinct histological features characterize primary angiosarcoma of bone. Histopathology. 2011;58:254–64.CrossRefGoogle Scholar
  11. Verbeke SL, Bertoni F, Bacchini P, Oosting J, Sciot R, Krenács T, Bovée JV. Active TGF-β signaling and decreased expression of PTEN separates angiosarcoma of bone from its soft tissue counterpart. Mod Pathol. 2013;26:1211–21.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  1. 1.Department of Pathology and Laboratory MedicineHospital for Special SurgeryNew YorkUSA
  2. 2.Department of PathologyMiller School of Medicine, University of MiamiMiamiUSA

Personalised recommendations