Cutaneous epithelioid angiosarcoma of the head and neck. A case report of an unusual aggressive entity with limited treatment options

摘要

Cutaneous angiosarcoma (CA) is an uncommon malignant mesenchymal neoplasm with aggressive behavior and ahigh mortality rate. A rare histological variant of CA is the epithelioid variant angiosarcoma (EA), which is associatedwith a worse clinical outcome. We report a 74-year-old female with an 8-month history of an ulcerated, poorly defined,painless tumoral mass on her scalp, face, and neck. Her past medical history included rheumatoid arthritis. A panelof immunohistochemical markers positive for vascular markers CD31 and CD34, confirmed the diagnosis. She hadan overall 9-month survival since the diagnosis was made. Comorbid diseases and tumoral size make most of CApatients ineligible surgical candidates. Limited treatment options are available and due to its accelerated progression,achieving local control and prevention of metastasis in EA is challenging. We emphasize the need for early diagnosisand a multidisciplinary approach in order to improve survival in these patients.