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WHO Classification of Tumours


Haemangiosarcoma 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 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.

In the breast, angiosarcomas are subdivided into primary and secondary tumours

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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


> Primary angiosarcomas are rare in the breast (about 0.05% of all primary breast malignancies). Patients range in age from 17 to 70 years (median 38 years). The tumours are seated deeply in the breast tissue and measure 1-20 cm (average 5 cm). Diffuse breast enlargement is observed in approximately 12% of cases. Angiosarcomas metastasize mainly to lungs, skin bone und liver. Radio- and chemotherapy are ineffective. The prognosis is largely dependent on histological features:
- In well differentiated (grade I) angiosarcomas, the neoplastic anastomosing vascular channels are characterized by very wide lumina filled with erythrocytes. The endothelial cells lining the channels contain prominent hyperchromatic nuclei. Grade I tumours have an estimated 5-year survival rate of 91% and a 10-year survival rate of 81%.
- Poorly differentiated (grade III) angiosarcomas are composed to >50% of solid endothelial or spindle cell areas with necrotic foci and numerous mitoses. The lesion appears ill defined, indurated and fibrous. Grade III tumours have a 2-year survival probability of 31% and a 5- and 10-year survival probability of 14%.
- Intermediately differentiated (grade II) angiosarcomas are composed of the well differentiated pattern to at least 75%, but contain scattered solid cellular foci. Grade II tumours have a 5- and 10-year survival probability of 68%.

> Secondary angiosarcomas arise in different locations after treatment of a breast tumour:
- In skin and soft tissues of the arm following ipsilateral radical mastectomy and subsequent lymphoedema: Stewart Treves (S-T) syndrome. S-T syndrome is lethal with a median survival of 19 months

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Lymphangiosarcoma in postmastectomy lymphedema; a report of six cases in elephantiasis chirurgica.
Cancer 1: 64-81

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Chung KC, Kim HJ, Jeffers LL (2000)
Lymphangiosarcoma (Stewart-Treves syndrome) in postmastectomy patients.
J Hand Surg Am 25: 1163-8

- In skin and chest wall following radical mastectomy and local radiotherapy

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Cancellieri A, Eusebi V, Mambelli V, Ricotti G, Gardini G, Pasquinelli G (1991)
Well-differentiated angiosarcoma of the skin following radiotherapy. Report of two cases.
Pathol Res Pract 187: 301-6

- In skin and/or breast parenchyma following conservation treatment and radiotherapy. Approximately 80% of the tumours are multifocal

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Marchal C, Weber B, de Lafontan B, Resbeut M, Mignotte H, du Chatelard PP, Cutuli B, Reme-Saumon M, Broussier-Leroux A, Chaplain G, Lesaunier F, Dilhuydy JM, Lagrange JL (1999)
Nine breast angiosarcomas after conservative treatment for breast carcinoma: a survey from French comprehensive Cancer Centers.
Int J Radiat Oncol Biol Phys 44: 113-9