9120/3 Haemangiosarcoma


Synonyms

Angiosarcoma

Definitions

Liver and intrahepatic bile ducts
ICD-O-3 topography code: C22
ICD10: C22
           

A malignant tumor arising from the endothelial cells of the blood vessels. Microscopically, it is characterized by frequently open vascular anastomosing and branching channels. The malignant cells that line the vascular channels are spindle or epithelioid and often display hyperchromatic nuclei. Angiosarcomas most frequently occur in the skin and breast. Patients with long-standing lymphedema are at increased risk of developing angiosarcoma.

Hepatic angiosarcoma
The typical occurrence is in patients aged > 60 years and there is a 3 : 1 predominance in men. However, angiosarcomas occur even in children with a very low frequency. Known etiological factors (seen in 20% of hepatic angiosarcomas) include occupational exposure to vinyl chloride monomer, arsenic, and androgenic-anabolic steroids. Iatrogenic thorium oxide (thorothrast), an α particle-radiating substance previously used as a radiological contrast medium, is historically important, but presently a very rare etiological factor. Prognosis is poor, and most patients die within 1 year.1
 
Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010



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Tumours of the central nervous system
ICD-O-3 topography code: C70, C71 and C72
ICD10: C70
           

A malignant tumor arising from the endothelial cells of the blood vessels. Microscopically, it is characterized by frequently open vascular anastomosing and branching channels. The malignant cells that line the vascular channels are spindle or epithelioid and often display hyperchromatic nuclei. Angiosarcomas most frequently occur in the skin and breast. Patients with long-standing lymphedema are at increased risk of developing angiosarcoma.



Hypopharynx, Larynx and Trachea
ICD-O-3 topography code: C13, C32, C33
ICD10: C13, C32, C33
           

A malignant tumor arising from the endothelial cells of the blood vessels. Microscopically, it is characterized by frequently open vascular anastomosing and branching channels. The malignant cells that line the vascular channels are spindle or epithelioid and often display hyperchromatic nuclei. Angiosarcomas most frequently occur in the skin and breast. Patients with long-standing lymphedema are at increased risk of developing angiosarcoma.



Colon and rectum
ICD-O-3 topography code: C18-C20
ICD10: C18-C20
           

A malignant tumor arising from the endothelial cells of the blood vessels. Microscopically, it is characterized by frequently open vascular anastomosing and branching channels. The malignant cells that line the vascular channels are spindle or epithelioid and often display hyperchromatic nuclei. Angiosarcomas most frequently occur in the skin and breast. Patients with long-standing lymphedema are at increased risk of developing angiosarcoma.



Thyroid gland
ICD-O-3 topography code: C73
ICD10: C73
           

A malignant tumor arising from the endothelial cells of the blood vessels. Microscopically, it is characterized by frequently open vascular anastomosing and branching channels. The malignant cells that line the vascular channels are spindle or epithelioid and often display hyperchromatic nuclei. Angiosarcomas most frequently occur in the skin and breast. Patients with long-standing lymphedema are at increased risk of developing angiosarcoma
2
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



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Angiosarcomas of thyroid were originally reported in Alpine countries of central Europe, and were linked to dietary iodine deficiency
3
 
Ronald A. DeLellis, Ricardo V. Lloyd, Philipp U. Heitz, Charis Eng
World Health Organization Classification of Tumours. Pathology and Genetics of Tumors of Endocrine Organs
IARC Press: Lyon 2004



. This view has been challenged by cases occurring in flatland areas or seaside regions, suggesting that other as yet unknown factors may be involved in their etiology. Most patients die within less than six months after diagnosis, regardless of the treatment protocol 4
Click to access Pubmed
Egloff B (1983)
The hemangioendothelioma of the thyroid.
Virchows Arch A Pathol Anat Histopathol 400: 119-42



5
Click to access Pubmed
Ladurner D, Tötsch M, Luze T, Bangerl I, Sandbichler P, Schmid KW (1990)
[Malignant hemangioendothelioma of the thyroid gland. Pathology, clinical aspects and prognosis].
Wien Klin Wochenschr 102: 256-9



. Entirely intrathyroid tumours generally have a longer survival than those with extrathyroidal extension.



Breast
ICD-O-3 topography code: C50
ICD10: C50
           

A malignant tumour showing endothelial differentiation.





Nasal cavity and Sinuses
ICD-O-3 topography code: C30-C31
ICD10: C30-C31
           

A malignant tumor arising from the endothelial cells of the blood vessels. Microscopically, it is characterized by frequently open vascular anastomosing and branching channels. The malignant cells that line the vascular channels are spindle or epithelioid and often display hyperchromatic nuclei. Angiosarcomas most frequently occur in the skin and breast. Patients with long-standing lymphedema are at increased risk of developing angiosarcoma.



Connective, subcutaneous and other soft tissues
ICD-O-3 topography code: C49
ICD10: C49
           

A malignant tumor arising from the endothelial cells of the blood vessels. Microscopically, it is characterized by frequently open vascular anastomosing and branching channels. The malignant cells that line the vascular channels are spindle or epithelioid and often display hyperchromatic nuclei. Haemangiosarcomas most frequently occur in the skin and breast. Patients with long-standing lymphedema are at increased risk of developing angiosarcoma.



Kidney
ICD-O-3 topography code: C64
ICD10: C64
           

Hemangiosarcoma is a malignant tumour arising from the endothelial cells of the blood vessels. Microscopically, it is characterized by vascular anastomosing and branching channels. The malignant cells lining the vascular channels are spindle shaped or epithelioid and often display hyperchromatic nuclei. Angiosarcomas most frequently occur in the skin and breast. Patients with long-standing lymphedema are at increased risk of developing angiosarcoma
11
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



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Primary renal angiosarcomas are exceedingly rare. They affect adults (mean age 58 years), with a strong male predominance (ratio 19:4). The prognosis is poor, due to rapid development of haematogenous metastasis. The mean survival is approximately 8 months
12
 
Eble JE, Sauter G, Epstein JI, Sesterhenn IA (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Urinary System and Male Genital Organs.
3rd Edition
IARC Press: Lyon 2004



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Cervix uteri
ICD-O-3 topography code: C53
ICD10: C53
           

Hemangiosarcoma is a malignant tumour arising from the endothelial cells of the blood vessels. Microscopically, it is characterized by frequently open vascular anastomosing and branching channels. The malignant cells that line the vascular channels are spindle shaped or epithelioid and often display hyperchromatic nuclei. Angiosarcomas most frequently occur in the skin and breast. Patients with long-standing lymphedema are at increased risk of developing angiosarcoma.

Cervical hemangiosarcoma is extremely rare. It arises in adults. Patients typically present with vaginal bleeding or discharge. The prognosis is poor
13
 
Tavassoli FA, Devilee P (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Breast and Female Genital Organs.
3rd Edition
IARC Press: Lyon 2003



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Small intestine
ICD-O-3 topography code: C17
ICD10: C17
           

A malignant tumor arising from the endothelial cells of the blood vessels. Microscopically, it is characterized by frequently open vascular anastomosing and branching channels. The malignant cells that line the vascular channels are spindle or epithelioid and often display hyperchromatic nuclei. Angiosarcomas most frequently occur in the skin and breast. Patients with long-standing lymphedema are at increased risk of developing angiosarcoma.

Intestinal angiosarcoma
In rare instances, angiosarcoma can involve the small intestine or other parts of the gastrointestinal tract, particularly in older adults with uterine or urinary bladder cancer previously treated by irradiation.

Grossly, angiosarcoma forms a haemorrhagic, transmural, often ulcerated mass that often extends into mesentery. The lesions may be multifocal and/or metastases, e.g. from soft tissues of the head and neck.

Histologically, the irregular anastomosing vascular channels are lined by atypical spindled to epithelioid endothelial cells with variable mitotic activity. Although most are high-grade, isolated examples are well-differentiated and low-grade.

Immunolabelling is positive in nearly all cases for CD31 (membrane pattern), but more variable for CD34 and FVIII-related antigen. Keratins can be present, especially in epithelioid variants
14
Click to access Pubmed
Allison KH, Yoder BJ, Bronner MP, Goldblum JR, Rubin BP (2004)
Angiosarcoma involving the gastrointestinal tract: a series of primary and metastatic cases.
Am J Surg Pathol 28: 298-307



15
Click to access Pubmed
Taxy JB, Battifora H (1988)
Angiosarcoma of the gastrointestinal tract. A report of three cases.
Cancer 62: 210-6



16
 
Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010



.



Urinary system: Renal pelvis, ureter, bladder, urethra
ICD-O-3 topography code: C65-C68
ICD10: C65-C68
           

A malignant tumor arising from the endothelial cells of the blood vessels. Microscopically, it is characterized by frequently open vascular anastomosing and branching channels. The malignant cells that line the vascular channels are spindle or epithelioid and often display hyperchromatic nuclei. Angiosarcomas most frequently occur in the skin and breast. Patients with long-standing lymphedema are at increased risk of developing angiosarcoma.



Adrenal gland
ICD-O-3 topography code: C74
ICD10: C74
           

Angiosarcoma is a malignant tumor arising from the endothelial cells of the blood vessels. Microscopically, it is characterized by frequently open vascular anastomosing and branching channels. The malignant cells that line the vascular channels are spindle or epithelioid and often display hyperchromatic nuclei. Angiosarcomas most frequently occur in the skin and breast. Patients with long-standing lymphedema are at increased risk of developing angiosarcoma.
Angiosarcomas in the adrenal gland are usually of the epithelioid type
17
Click to access Pubmed
Henry JF, Sebag F, Iacobone M, Hubbard J, Maweja S (2002)
[Lessons learned from 274 laparoscopic adrenalectomies].
Ann Chir 127: 512-9



18
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Wenig BM, Abbondanzo SL, Heffess CS (1994)
Epithelioid angiosarcoma of the adrenal glands. A clinicopathologic study of nine cases with a discussion of the implications of finding "epithelial-specific" markers.
Am J Surg Pathol 18: 62-73



and have a variable prognosis 19
Click to access Pubmed
Wenig BM, Abbondanzo SL, Heffess CS (1994)
Epithelioid angiosarcoma of the adrenal glands. A clinicopathologic study of nine cases with a discussion of the implications of finding "epithelial-specific" markers.
Am J Surg Pathol 18: 62-73



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Bone
ICD-O-3 topography code: C40-C41
ICD10: C40-C41
           

A malignant tumor arising from the endothelial cells of the blood vessels. Microscopically, it is characterized by frequently open vascular anastomosing and branching channels. The malignant cells that line the vascular channels are spindle or epithelioid and often display hyperchromatic nuclei. Angiosarcomas most frequently occur in the skin and breast. Patients with long-standing lymphedema are at increased risk of developing angiosarcoma.



Prostate gland
ICD-O-3 topography code: C61
ICD10: C61
           

A malignant tumor arising from the endothelial cells of the blood vessels. Microscopically, it is characterized by frequently open vascular anastomosing and branching channels. The malignant cells that line the vascular channels are spindle or epithelioid and often display hyperchromatic nuclei. Angiosarcomas most frequently occur in the skin and breast. Patients with long-standing lymphedema are at increased risk of developing angiosarcoma.



Bronchus and lung
ICD-O-3 topography code: C34
ICD10: C34
           

A malignant tumor arising from the endothelial cells of the blood vessels. Microscopically, it is characterized by frequently open vascular anastomosing and branching channels. The malignant cells that line the vascular channels are spindle or epithelioid and often display hyperchromatic nuclei. Angiosarcomas most frequently occur in the skin and breast. Patients with long-standing lymphedema are at increased risk of developing angiosarcoma.



Skin
ICD-O-3 topography code: C44
ICD10: C44
           

A malignant tumor arising from the endothelial cells of the blood vessels. Microscopically, it is characterized by frequently open vascular anastomosing and branching channels. The malignant cells that line the vascular channels are spindle or epithelioid and often display hyperchromatic nuclei. Angiosarcomas most frequently occur in the skin and breast. Patients with long-standing lymphedema are at increased risk of developing angiosarcoma.