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Int J Clin Exp Pathol 2011;4(6):541-551
Review Ariticle
Primary vascular tumors of bone: a spectrum of entities?
Sofie L. J. Verbeke, Judith V. M. G. Bovée
Department of Pathology, Leiden University Medical Center, Leiden, The Netherlands; Department of Pathology, Antwerp University Hospital,
Edegem, Belgium.
Received July 10, 2011; accepted July 18, 2011; Epub July 25, 2011; published August 15, 2011
Abstract: Vascular tumors of bone are a heterogeneous group. Numerous terms have been introduced as well as different classification
systems. None of the classification schemes have been accepted due to lack of consistent terminology, accepted histological criteria, and
limited correlation with clinical outcome. It is acknowledged that vascular tumors of bone originate from endothelial cells, resulting in variable
expression of endothelial markers. None of these markers are useful to discriminate between benign and malignant lesions. Although
radiologic appearance is not specific, radiologic multifocality should trigger to include a vascular neoplasm in the differential diagnosis. This
review gives an overview of current literature by describing all different histologic subtypes in correspondence with clinical, radiological and
genetic data. We propose the classification of vascular tumors of bone according to the three-tiered World Health Organization classification
scheme for soft tissue tumors dividing entities into a benign, intermediate and malignant category. Hemangioma is the most often and
commonly recognized benign lesion. Epithelioid hemangioma has been better defined over the past few years. Based on its locally aggressive
behavior and occurrence of lymph node metastases, classification within the intermediate category could be considered. Angiosarcoma is the
only accepted term for high-grade malignant vascular tumor of bone and so far, epithelioid hemangioendothelioma is the only accepted
low-grade malignant vascular tumor of bone. It is still unclear whether other low-grade malignant vascular tumors of bone (e.g.
hemangioendothelioma) truly exist. Unfortunately, molecular / genetic studies of vascular tumors of bone which might support the proposed
classification are very sparse. (IJCEP1107001).
Keywords: Vascular tumor of bone, hemangioma, epithelioid hemangioma, epithelioid hemangioendothelioma, angiosarcoma, bone tumor
Full text PDF
Address all correspondence to:
J. V. M. G. Bovée, MD, PhD
Leiden University Medical Center
Department of Pathology
L1Q, P.O. Box 9600
2300 RC Leiden, the Netherlands.
Tel: ++31 71 5266617; Fax: ++31 71 5266952
E-mail: j.v.m.g.bovee@lumc.nl
Review Ariticle
Primary vascular tumors of bone: a spectrum of entities?
Sofie L. J. Verbeke, Judith V. M. G. Bovée
Department of Pathology, Leiden University Medical Center, Leiden, The Netherlands; Department of Pathology, Antwerp University Hospital,
Edegem, Belgium.
Received July 10, 2011; accepted July 18, 2011; Epub July 25, 2011; published August 15, 2011
Abstract: Vascular tumors of bone are a heterogeneous group. Numerous terms have been introduced as well as different classification
systems. None of the classification schemes have been accepted due to lack of consistent terminology, accepted histological criteria, and
limited correlation with clinical outcome. It is acknowledged that vascular tumors of bone originate from endothelial cells, resulting in variable
expression of endothelial markers. None of these markers are useful to discriminate between benign and malignant lesions. Although
radiologic appearance is not specific, radiologic multifocality should trigger to include a vascular neoplasm in the differential diagnosis. This
review gives an overview of current literature by describing all different histologic subtypes in correspondence with clinical, radiological and
genetic data. We propose the classification of vascular tumors of bone according to the three-tiered World Health Organization classification
scheme for soft tissue tumors dividing entities into a benign, intermediate and malignant category. Hemangioma is the most often and
commonly recognized benign lesion. Epithelioid hemangioma has been better defined over the past few years. Based on its locally aggressive
behavior and occurrence of lymph node metastases, classification within the intermediate category could be considered. Angiosarcoma is the
only accepted term for high-grade malignant vascular tumor of bone and so far, epithelioid hemangioendothelioma is the only accepted
low-grade malignant vascular tumor of bone. It is still unclear whether other low-grade malignant vascular tumors of bone (e.g.
hemangioendothelioma) truly exist. Unfortunately, molecular / genetic studies of vascular tumors of bone which might support the proposed
classification are very sparse. (IJCEP1107001).
Keywords: Vascular tumor of bone, hemangioma, epithelioid hemangioma, epithelioid hemangioendothelioma, angiosarcoma, bone tumor
Full text PDF
Address all correspondence to:
J. V. M. G. Bovée, MD, PhD
Leiden University Medical Center
Department of Pathology
L1Q, P.O. Box 9600
2300 RC Leiden, the Netherlands.
Tel: ++31 71 5266617; Fax: ++31 71 5266952
E-mail: j.v.m.g.bovee@lumc.nl
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