Rapidly progressive and fatal case of extragenital cutaneous epithelioid angiosarcoma with visceral involvement

Abbott, Jeff; Blake, John; Secrest, Aaron M.; Florell, Scott R.

doi:10.1016/j.jdcr.2019.09.025

JAAD Case Reports

2020

DOI: 10.1016/j.jdcr.2019.09.025

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Rapidly progressive and fatal case of extragenital cutaneous epithelioid angiosarcoma with visceral involvement

Jeff Abbott

John Blake

Aaron M. Secrest

et al.

Abstract: Angiosarcomas are aggressive soft-tissue sarcomas derived from malignant endothelial cells, accounting for 1.6% of cutaneous soft-tissue sarcomas. 2 The most common locations of CAS are the head and neck (27%), breast (20%), and extremities (15%), with primary visceral origin being

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2024

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References 11 publications

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Advanced cutaneous angiosarcoma with aTRIM24::BRAFgene fusion treated with trametinib

Murphy,

Pankiw,

Gibbings

et al. 2024

BMJ Case Rep

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A postmenopausal woman presented with a painful, erythematous rash affecting her head and neck. CT imaging revealed diffuse areas of skin thickening affecting her face and neck, and necrotic lymphadenopathy. Additional imaging revealed several osteolytic bone lesions. Biopsy reported a malignant vascular neoplasm, suggestive of angiosarcoma. Molecular analysis reported aTRIM24::BRAFgene fusion. After progression on first-line paclitaxel, the MEK inhibitor trametinib was administered, resulting in an excellent clinical and radiological response. This case reports a novel gene fusion, to our knowledge, the first reported in sarcoma, and highlights the utility and importance of molecular profiling in obtaining access to a treatment that may not otherwise be considered in standard site-specific therapeutic regimens, where therapeutic options may be limited.

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Advanced cutaneous angiosarcoma with aTRIM24::BRAFgene fusion treated with trametinib

Murphy,

Pankiw,

Gibbings

et al. 2024

BMJ Case Rep

View full text Add to dashboard Cite

show abstract

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Rapidly progressive and fatal case of extragenital cutaneous epithelioid angiosarcoma with visceral involvement

Abstract: Angiosarcomas are aggressive soft-tissue sarcomas derived from malignant endothelial cells, accounting for 1.6% of cutaneous soft-tissue sarcomas. 2 The most common locations of CAS are the head and neck (27%), breast (20%), and extremities (15%), with primary visceral origin being

Cited by 1 publication

References 11 publications

Advanced cutaneous angiosarcoma with aTRIM24::BRAFgene fusion treated with trametinib

Advanced cutaneous angiosarcoma with aTRIM24::BRAFgene fusion treated with trametinib

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