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Bone: Angiosarcoma

Written2008-02Sofie LJ Verbeke, Judith VMG Bovée
Department of Pathology, Leiden University Medical Center, Leiden, The Netherlands
Updated2016-11David G.P. van Ijzendoorn and Judith V.M.G. Bovée
Department of Pathology, Leiden University Medical Center, Leiden, The Netherlands.

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Abstract Review on Angiosarcoma of bones, with data on clinics, and the genes involved.


ICD-Topo C400-C403,C408-C414,C418-C419 BONES & JOINTS
ICD-Morpho 9120/3 Hemangiosarcoma
Atlas_Id 5359
Phylum Bones::Angiosarcoma
WHO/OMS Classification Bones
Other namesPrimary malignant vascular tumours of bone
Note Variable proliferation of tumour cells which show an endothelial phenotype
Microscopy - PMVTB: epithelioid morphology - HE (10x)


Note Angiosarcoma is a high grade primary malignant vascular tumour of bone as recognized in the 2013 World Health Organization (WHO) classification. It can sometimes be difficult to histologically distinguish angiosarcoma from Epithelioid haemangioendothelioma as they can show overlapping morphology , but recently multiple fusion genes and mutations have been identified that can help in the differential diagnosis. The prognosis for angiosarcoma is generally very poor.

Clinics and Pathology

Disease Primary malignant vascular tumours of bone
Etiology Although there is in a very small percentage an association between angiosarcoma and radiation, the etiology of the majority of angiosarcomas remains unknown.
Epidemiology Angiosarcoma is extremely rare and represent less than 1% of primary malignant bone tumours.
Clinics Angiosarcomas of bone arise in adults and have a wide, nearly equal age distribution from the second till eighth decade. In contrast to epithelioid haemangioendotheliomas have a tendency to occur in young adults (second - third decade). It seems that males are slightly more affected than females.
Angiosarcomas of bone have a wide skeletal distribution; however, they have a tendency to occur in the short and long tubular bones of the extremities of which the femur, tibia and humerus are most often affected. In contrast to the soft tissue counterpart, one third of the tumours are multifocal, involving two or more distant bones.
In general, malignant vascular tumours of bone present as a painful mass. Depending on the size and localization of the tumour, neurological deficit or other symptoms can occur.
Pathology Similar to the soft tissue counterpart ( Angiosarcoma of the soft tissue), malignant vascular tumours of bone have variable histological features, varying from well differentiated to poor differentiated lesions. To a certain extent, the formation of vascular channels is one of the most common referred hallmarks of this tumour. These lesions show a variable amount of mitotic features, atypical mitotic figures, necrosis and cytonuclear atypia. Cells can have a spindle cell or an epithelioid morphology.
Treatment Prefered therapy, whenever possible, is surgical intervention (wide en bloc resection or amputation). Moreover radiotherapy is also applied, although there is no general rule for treatment. Treatment depends upon multiple factors, such as age, size, location of the tumour, and the extent of disease. Chemotherapy is used, although its usefulness is not well documented.
Prognosis Angiosarcoma of bone has 33% 5 year overall survival. When primary angiosarcoma of bone exhibits more than 3 mitosis per 10 HPF with prominent nucleolus and fewer than five eosinophilic granulocytes per 10 HPF they will likely have a worse prognosis.


Note Recently mutations and amplifications have been described for angiosarcoma, most of these aberrations occur in the tyrosine-kinase pathways specific for vascular receptors. Currently PTPRB, PLCG1, CIC, KDR, and FLT4 mutations and MYC amplifications. have been described for angiosarcoma. There is no evidence that there is a molecular genetic difference between angiosarcoma of bone and angiosarcoma occurring in soft tissue.


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This paper should be referenced as such :
van Ijzendoorn DGP, Bovée JVMG
Bone: Angiosarcom
Atlas Genet Cytogenet Oncol Haematol. in press
On line version :
History of this paper:
Verbeke, SLJ ; Bove, JVMG. Bone: Angiosarcoma. Atlas Genet Cytogenet Oncol Haematol. 2009;13(2):147-148.

External links

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