Atlas of Genetics and Cytogenetics in Oncology and Haematology

Home   Genes   Leukemias   Solid Tumours   Cancer-Prone   Deep Insight   Case Reports   Journals  Portal   Teaching   
X Y 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 NA

Bone: Vascular Tumours

Identity

Note Benign vascular lesions of bone are relatively common and occur most frequent as an asymptomatic incidental finding in the skull or spine. Primary malignant vascular tumors of bone are rare. They represent less than 1% of primary malignant bone tumors reported by the Netherlands Committee on Bone Tumors and 0,5% of those registered at the Mayo Clinic. Clinically they are extremely aggressive and have a very poor prognosis. Survival rates are unknown, but in those cases reported patients die very soon after diagnosis due to wide spread metastasis and lack of tailored therapy.

Classification

Note Over the years, the terminology and classification of vascular tumors of bone has been highly controversial and in literature a great variety of names has been proposed. Today, angiosarcoma is the most acceptable term for high-grade malignant vascular tumors of bone, as recognized in the 2002 World Health Organization classification.
However, the classification of low-grade vascular to intermediate tumors of bone, in particular of haemangioendotheliomas, is extremely difficult due to the lack of uniform terminology and accepted histological criteria. Many authors have proposed different classification systems, but due to small numbers of cases, their large diversity and the lack of good correlation with clinical outcome none of them have been generally accepted so far.
The large variety of histological features of vascular tumors of bone suggests that it should be regarded as a spectrum with on one side the overtly benign lesions and on the other side the frankly malignant lesions. In between there are the low and intermediate grade lesions in which numerous histomorphological diversity can be seen and for which classification is most difficult.
Note Schematic representation of histological spectrum of vascular tumors of bone: accepted histological criteria to classify vascular tumors of bone are lacking.
    Today, the most accepted classification of vascular tumours of bone is the 2002 WHO classification:
  • Haemangioma and related lesions
  • Angiosarcoma
    		•

    Clinics and Pathology

    Disease Haemangioma and related lesions
    Note Multiple lesions are defined as (haem)angiomatosis.
    Phenotype / cell stem origin Endothelial cell
    Epidemiology Haemangiomas are relatively common.
    Clinics In general asymptomatic.
    Cytogenetics No cytogenetic investigations reported.
    Prognosis Haemangiomas have a good prognosis and low recurrence rate.

    Disease Angiosarcoma
    Epidemiology Extremely rare
    Clinics In general, presents as a painful mass. Depending on the size and localization of the tumour, neurological deficit or other symptoms can occur.
    Cytogenetics No cytogenetic investigations of angiosarcoma in bone are reported.
    Prognosis It is accepted that histologically well differentiated tumours have a better prognosis than poorly differentiated tumours.

    Bibliography

    Bone tumors. Diagnosis, treatment, and prognosis.
    Huvos AG
    W.B. Saunders Company, 1991.
     
    Radiologic atlas of bone tumors.
    Mulder JD, Schutte HE, Kroon HM, Taconis WK.
    Elsevier, 1993.
     
    Bone tumors.
    Dorfman HD, Czerniak B.
    Mosby, 1998.
     
    Benign vascular lesions of bone: radiologic and pathologic features.
    Wenger DE, Wold LE.
    Skeletal Radiol 2000; 29(2): 63-74. (Review)
    PMID 10741493
     
    Malignant vascular lesions of bone: radiologic and pathologic features.
    Wenger DE, Wold LE.
    Skeletal Radiol 2000; 29(11): 619-631. (Review)
    PMID 11201031
     
    Epithelioid vascular tumors of bone: a review and proposal of a classification scheme.
    O'Connell JX, Nielsen GP, Rosenberg AE.
    Adv Anat Pathol 2001; 8(2): 74-82. (Review)
    PMID 11236956
     
    World Health Organization Classification of Tumours: Pathology and genetics of tumours of soft tissue and bone.
    Fletcher CDM, Krishnan Unni K, Mertens F.
    IARCpress Lyon 2002.
     
    Epithelioid and spindle cell haemangioma of bone.
    Maclean FM, Schatz J, McCarthy SW, Scolyer RA, Stalley P, Bonar SF.
    Sketletal Radiol 2007; 36 Suppl 1: S50-57.
    PMID 16688448
     
    REVIEW articlesautomatic search in PubMed
    Last year publicationsautomatic search in PubMed

    Contributor(s)

    Written02-2008Sofie LJ Verbeke, Judith VMG Bovée
    Department of Pathology, Leiden University Medical Center, Leiden, The Netherlands

    Citation

    This paper should be referenced as such :
    Verbeke SLJ, Bovée JVMG . Bone: Vascular Tumours. Atlas Genet Cytogenet Oncol Haematol. February 2008 .
    URL : http://AtlasGeneticsOncology.org/Tumors/VascularBoneID5357.html

    © Atlas of Genetics and Cytogenetics in Oncology and Haematology
    indexed on : Thu Apr 17 14:14:53 2008

    Home   Genes   Leukemias   Solid Tumours   Cancer-Prone   Deep Insight   Case Reports   Journals  Portal   Teaching   

    For comments and suggestions or contributions, please contact us

    j.l.huret@chu-poitiers.fr.