Angiosarcoma

Definition:

Angiosarcomas are rare, generally aggressive, malignant mesenchymal tumours of vascular origin and constitute less than 1% of all sarcomas. Their most frequent site of origin is the head and neck, especially the scalp, of elderly men. Angiosarcomas are likely derived from vascular endothelial cells, possibly resulting from aberrant angiogenesis. Studies have shown relatively increased expression of vascular endothelial growth factor (VEGF) and its receptors (VEGFr1-3) in human angiosarcoma. The most common gene mutation in angiosarcomas is KRAS as well as other RAS mutations. Activation of the RAS pathway is often tumorigenic. Although immortalised murine endothelial cells form benign haemangiomas in vivo, the addition of activated HRAS produces rapidly growing, poorly differentiated angiosarcomas.

References:

[1]. Cristina Antonescu, et al. Malignant vascular tumors--an update. Mod Pathol. 2014 Jan;27 Suppl 1:S30-8. [Content Brief]

[2]. Harold C Sullivan, et al. The utility of ERG, CD31 and CD34 in the cytological diagnosis of angiosarcoma: an analysis of 25 cases. J Clin Pathol. 2015 Jan;68(1):44-50. [Content Brief]

[3]. Ioanna Letsa, et al. Angiosarcoma of the face and scalp: effective systemic treatment in the older patient. J Geriatr Oncol. 2014 Jul;5(3):276-80. [Content Brief]

[4]. Justin T Huntington, et al. Angiosarcoma: A rare malignancy with protean clinical presentations. J Surg Oncol. 2015 Jun;111(8):941-50. [Content Brief]

[5]. M Tanioka, et al. Angiosarcoma of the scalp: absence of vascular endothelial cadherin in primary and metastatic lesions. Br J Dermatol. 2001 Feb;144(2):380-3. [Content Brief]

[6]. Robin J Young, et al. Angiosarcoma. Lancet Oncol. 2010 Oct;11(10):983-91. [Content Brief]

[7]. Samir H Patel, et al. Angiosarcoma of the scalp and face: the Mayo Clinic experience. JAMA Otolaryngol Head Neck Surg. 2015 Apr;141(4):335-40. [Content Brief]

[8]. Takayuki Ohguri, et al. Angiosarcoma of the scalp treated with curative radiotherapy plus recombinant interleukin-2 immunotherapy. Int J Radiat Oncol Biol Phys. 2005 Apr 1;61(5):1446-53. [Content Brief]

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