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hemangiosarcoma; angiosarcoma

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

Interactions

disease interactions

Specific

cardiac angiosarcoma

General

soft tissue sarcoma (STS) vascular tissue neoplasm; angioma

References

  1. Navarrete-Dechent S, Busam KJ, Markova A. Facial Erythema in an Elderly Man. JAMA Dermatol. 2020;156(5):587-588 PMID: 32211818 https://jamanetwork.com/journals/jamadermatology/fullarticle/2763189
  2. NEJM Knowledge+ Question of the Week. Sept 12, 2023 https://knowledgeplus.nejm.org/question-of-week/1000/
  3. 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: 31448482 Review.
  4. 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: 37222197 Review.