hemangiosarcoma; angiosarcoma
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Etiology
- head & neck type (Wilson-Jones angiosarcoma)
- typically arising on scalp
- idiopathic with a possible role of ultraviolet radiation
- post-radiotherapy type
- typically arises on breast years after breast cancer treatment
- secondary to chronic lymphedema type or Stewart-Treves syndrome
- rosacea-like angiosarcoma may represent an overlap of rosacea with cutaneous angiosarcoma
Epidemiology
- rare
Pathology
- aggressive, malignant tumor arising in blood or lymphatic vessels with uncontrolled proliferation of endothelial cells
- skin biopsy for cutaneous angiosarcoma
- dissecting vascular proliferation through the entire dermis
- ectatic vascular channels lined by atypical plump endothelial cells
- immunohistochemistry staining results negative for Human herpesvirus 8 (Kaposi sarcoma)
Clinical manifestations
- cutaneous angiosarcoma may appear like rosacea[1]
- typically affects head & neck areas of older, white, adult men
- post-radiotherapy type may arise on breast or mastectomy site years after breast cancer radiation therapy (generally 5-7 years)
- a cutaneous nodule with a bruise-like appearance in a patient with previous radiation to the area
- ecchymosis-like patches or plaques that subsequently expand to form nodules involving larger areas that further ulcerate
- diffuse involvement and satellite nodules are common
Radiology
- positron emission tomography/computed tomography for staging
Differential diagnosis
- hemangioma (< 2 cm with visible feeder vessels)
- hematoma
- radiation dermatitis
- atrophy, fibrosis, telangiectasias, & pigmentation changes
- progressive induration, edema, & thickening of the dermis
Management
- surgery with or without radiation
- mastectomy as indicated
- intravenous paclitaxel (60 mg/m2) weekly as palliative therapy
- prognosis:
- generally poor
- local & distant recurrence common
More general terms
More specific terms
References
- ↑ 1.0 1.1 Navarrete-Dechent S, Busam KJ, Markova A. Facial Erythema in an Elderly Man. JAMA Dermatol. 2020;156(5):587-588 PMID: https://www.ncbi.nlm.nih.gov/pubmed/32211818 https://jamanetwork.com/journals/jamadermatology/fullarticle/2763189
- ↑ NEJM Knowledge+ Question of the Week. Sept 12, 2023 https://knowledgeplus.nejm.org/question-of-week/1000/
- ↑ Bonito FJP, de Almeida Cerejeira D, Dahlstedt-Ferreira C et al Radiation-induced angiosarcoma of the breast: A review. Breast J. 2020 Mar;26(3):458-463. Epub 2019 Aug 25. PMID: https://www.ncbi.nlm.nih.gov/pubmed/31448482 Review.
- ↑ Dufresne A, Meeus P, Sunyach MP. Treatment of radiation-associated angiosarcoma. Curr Opin Oncol. 2023 Jul 1;35(4):296-300. Epub 2023 May 12. PMID: https://www.ncbi.nlm.nih.gov/pubmed/37222197 Review.