Abstract
Cutaneous epithelioid angiosarcoma of the head and neck. A case report of an unusual aggressive entity with limited treatment options
Highlights
Cutaneous angiosarcoma (CA) is an uncommon malignant mesenchymal neoplasm with aggressive behavior and high mortality rate
CA usually arises in three characteristic clinical contexts, which include sun-damaged skin of the elderly, at the site of previous radiation treatment, and in lymphedematous limbs (Stewart-Treves syndrome).CA clinical presentation is characterized by large erythematous or violaceous patches, plaques or nodules, commonly affecting the face and scalp [3]
Concerning CA prognosis, tumor necrosis and epithelioid cytomorphology are associated with a worse clinical outcome [5]
Summary
Cutaneous angiosarcoma (CA) is an uncommon malignant mesenchymal neoplasm with aggressive behavior and high mortality rate. A rare histological variant of CA is the epithelioid variant angiosarcoma (EA). This neoplasm subtype arises mainly in deep soft tissues, cases with purely and primarily cutaneous involvement have been reported [4]. EA presents as erythematous or violaceous lesions. They are usually ill-defined, infiltrative hemorrhagic tumors. Treatment of choice in patients with CA remains surgical resection with or without postoperative radiation therapy [7], this is challenging because the tumor often exceeds the clinically apparent margins. In case the tumor is too extensive to consider surgical resection, there is no standard treatment.
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