Angiosarcoma is a rare tumor accounting for less than one percent of all sarcomas. It occurs typically in the skin and soft tissues of the head and neck.
It is a very malignant neoplastic proliferation arising from vascular endothelial cells. Systemic metastasis at the time of diagnosis is reported to be as high as 80%.
The typical primary site for metastatic cerebral angiosarcoma is the heart, accounting for 57% of cases. Other primary sites have been described, such as Dacron aortic vascular grafts, aorta, pleura, skin, liver, penis, bone, placenta and the orbit. Cerebral metastases are multiple in 57.9% of the cases. There is a male predominance and the mean age is 42.2 years.
The described risk factors for the development of angiosarcomas are radiation therapy, foreign object, chronic lymphedema associated with radical mastectomy, A-V fistulas and certain environmental carcinogens like vinyl chloride, arsenic and Thorotrast.
In a series of 53 post-radiation sarcomas, Laskin and al. reported that the most frequent radiation-induced sarcoma was malignant fibrous histiocytoma (68%) and that an angiosarcoma represented only one case (2%), on the thoracic wall. Another series of 2500 patients treated for ENT cancer reported only 5 sarcomas in the irradiated zone. None were angiosarcoma.
Vascular prostheses, especially Dacron prostheses, also have the potential to induce malignant mesenchymatous changes (2,4,11,16). We, however, did not find any cases that occurred after insertion of a vascular endoprosthesis.
Angiosarcoma classically presents with cutaneous involvement of the head and neck region in elderly patients or in the limbs of patients with lymphedema.
Angiosarcoma most frequently occurs in the skin of the head and neck region of elderly persons, lymphedematous limbs, or in deep soft tissue
A smaller, but increasingly recognized subset of angiosarcoma has been described in the deep soft tissues and in areas of prior irradiation, trauma or in association with foreign bodies such as ateriovenous shunts.
Deep-seated angiosarcoma
Deep-seated angiosarcoma frequently has an epithelioid cytomorphology. The epithelioid phenotype of angiosarcoma was first described in cases of cutaneous angiosarcoma by Rosai et al in 1976 and later documented in deep soft tissue by Weiss et al and Fletcher et al.
Intra-abdominal angiosarcoma has been described previously, usually arising in the liver, spleen and rarely adrenal glands, ovaries or as diffuse angiosarcomatosis.
Variants
littoral cell angiosarcoma
pediatric angiosarcomas
Localization
ovarian angiosarcoma (9591733)
angiosarcoma of the scalp and face
Cytogenetics
complex cytogenetic aberrations without consistent recurring chromosome aberration
some recurrent aberrations among angiosarcomas arising in the same location
Predisposition
xeroderma pigmentosum (11044816)
Case report
References
Autocrine and paracrine roles of VEGF/VEGFR-2 and VEGF-C/VEGFR-3 signaling in angiosarcomas of the scalp and face. Tokuyama W, Mikami T, Masuzawa M, Okayasu I. Hum Pathol. 2009 Nov 12. PMID: 19913279
Billings SD, McKenney JK, Folpe AL, Hardacre MC, Weiss SW. Cutaneous angiosarcoma following breast-conserving surgery and radiation: an analysis of 27 cases. Am J Surg Pathol. 2004 Jun;28(6):781-8. PMID: 15166670
Allison KH, Yoder BJ, Bronner MP, Goldblum JR, Rubin BP. Angiosarcoma involving the gastrointestinal tract: a series of primary and metastatic cases. Am J Surg Pathol. 2004 Mar;28(3):298-307. PMID: 15104292
Nucci MR, Krausz T, Lifschitz-Mercer B, Chan JK, Fletcher CD. Angiosarcoma of the ovary: clinicopathologic and immunohistochemical analysis of four cases with a broad morphologic spectrum. Am J Surg Pathol. 1998 May;22(5):620-30. PMID: 9591733
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