Bone: Angiosarcoma

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Bone: Angiosarcoma

Identity

Other names Primary malignant vascular tumours of bone
Note Variable proliferation of tumour cells which show an endothelial phenotype

Microscopy - PMVTB: epithelioid morphology - HE (10x)

Classification

Note Angiosarcoma is the most accepted term for high-grade malignant vascular tumors of bone, as recognized in the 2002 World Health Organization (WHO) classification. However, the classification of low to intermediate grade vascular tumors of bone, in particular of haemangioendotheliomas, is extremely difficult due to the lack of uniform terminology and accepted histological criteria. Many classification systems have been proposed, but none of them have been generally accepted so far.

Today, there is no generally accepted classification scheme. However, the 2002 WHO classification subdivide angiosarcoma in

well differentiated angiosarcomas,
poorly differentiated angiosaromas and
epithelioid haemangioendotheliomas.

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 Primary malignant vascular tumours of bone (PMVTB) are 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.
Epithelioid haemangioendothelioma is considered a separate entity with distinct histomorphological features and clinically a relatively favorable prognosis. These tumours consist of strands or cords of epithelioid cells with abundant eosinophilic cytoplasm and the presence of intracytoplasmatic vacuoles. Small vascular channels can be seen and the myxoid or hyalinised stromal component is most characteristic for this type of lesion.

Treatment 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. Therapeutic options are surgical intervention (wide en bloc resection or amputation) and radiotherapy. Chemotherapy is used, although its usefulness is not well documented.

Prognosis Survival rates of malignant vascular tumours of bone are unknown. It is accepted that histologically well differentiated tumours have a better prognosis than poorly differentiated tumours. It is suggested that multifocal lesions also have a better prognosis, most likely due to the fact that these lesions are more often well differentiated.

Cytogenetics

Note No underlying genetic disorders or aberrations are reported in malignant vascular tumours of bone so far.
In literature two independent cases of epithelioid haemangioendothelioma of bone were analyzed and revealed in one case a reciprocal translocation of chromosome 10 and 14 and in the other case complex rearrangements involving deletions and gains of 11q and 12q.
No karyotypes are reported for malignant vascular tumours of bone so far.

External links

Other database http://cgap.nci.nih.gov/Chromosomes/AbnCytSearchForm

Bibliography

Bone Tumors. Clinical, radiologic and pathologic correlations.

Mirra JM

Lea..

Malignant vascular lesions of bone: radiologic and pathologic features.

Wenger DE, Wold LE

Skeletal radiology. 2000 ; 29 (11) : 619-631.

PMID 11201031

Total resection of an intracerebral hemangioendothelioma in an infant. Case report and review of the literature.

Hodaie M, Becker L, Teshima I, Rutka JT

Pediatric neurosurgery. 2001 ; 34 (2) : 104-112.

PMID 11287811

Epithelioid vascular tumors of bone: a review and proposal of a classification scheme.

O'Connell JX, Nielsen GP, Rosenberg AE

Advances in anatomic pathology. 2001 ; 8 (2) : 74-82.

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.

Vascular tumors of bone: A study of 17 cases other than ordinary hemangioma, with an evaluation of the relationship of hemangioendothelioma of bone to epithelioid hemangioma, epithelioid hemangioendothelioma, and high-grade angiosarcoma.

Evans HL, Raymond AK, Ayala AG

Human pathology. 2003 ; 34 (7) : 680-689.

PMID 12874764

Chromosome analysis and molecular cytogenetic investigations of an epithelioid hemangioendothelioma.

Tsarouha H, Kyriazoglou AI, Ribeiro FR, Teixeira MR, Agnantis N, Pandis N

Cancer genetics and cytogenetics. 2006 ; 169 (2) : 164-168.

PMID 16938576

Post-irradiation angiosarcoma of bone.

Mittal S, Goswami C, Kanoria N, Bhattacharya A

Journal of cancer research and therapeutics. 2007 ; 3 (2) : 96-99.

PMID 17998731

REVIEW articles automatic search in PubMed

Last year publications automatic search in PubMed

Contributor(s)

Written 02-2008 Sofie 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: Angiosarcoma. Atlas Genet Cytogenet Oncol Haematol. February 2008 .
URL : http://AtlasGeneticsOncology.org/Genes/BoneAngiosarcID5359.html

© Atlas of Genetics and Cytogenetics in Oncology and Haematology
indexed on : Wed Sep 24 21:08:42 2008

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For comments and suggestions or contributions, please contact us

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

Other names	Primary malignant vascular tumours of bone
Note	Variable proliferation of tumour cells which show an endothelial phenotype


	Microscopy - PMVTB: epithelioid morphology - HE (10x)

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	Primary malignant vascular tumours of bone (PMVTB) are 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. Epithelioid haemangioendothelioma is considered a separate entity with distinct histomorphological features and clinically a relatively favorable prognosis. These tumours consist of strands or cords of epithelioid cells with abundant eosinophilic cytoplasm and the presence of intracytoplasmatic vacuoles. Small vascular channels can be seen and the myxoid or hyalinised stromal component is most characteristic for this type of lesion.
Treatment	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. Therapeutic options are surgical intervention (wide en bloc resection or amputation) and radiotherapy. Chemotherapy is used, although its usefulness is not well documented.
Prognosis	Survival rates of malignant vascular tumours of bone are unknown. It is accepted that histologically well differentiated tumours have a better prognosis than poorly differentiated tumours. It is suggested that multifocal lesions also have a better prognosis, most likely due to the fact that these lesions are more often well differentiated.

REVIEW articles	automatic search in PubMed
Last year publications	automatic search in PubMed

Written	02-2008	Sofie LJ Verbeke, Judith VMG Bovée
		Department of Pathology, Leiden University Medical Center, Leiden, The Netherlands