First Sonographic Description of Idiopathic Cutaneous Angiosarcoma of the Head and Neck

An 89-year-old woman, with a past history of systemic hypertension, aortic stenosis, and iron deficiency anemia and on treatment with hydrochlorothiazide, lisinopril, and acetylsalicylic acid, was seen for an asymptomatic lesion that had arisen a month earlier in her left frontoparietal region. She had been treated with topical corticosteroids, which were prescribed for suspected contact eczema to hair dye. Physical examination revealed an erythematous-violaceous plaque that extended down to her cheek. The surface of the lesion presented a small nodule of vascular appearance (Fig. 1, A and B). B-mode ultrasound (18 MHz probe, eSaote, MyLab25Gold) showed a predominantly hypoechoic nodule of 3.7 mm × 16.5 mm with well-defined borders, situated in the deep dermis and subcutaneous cellular tissue (Fig. 2A). The periphery of the lesion presented mixed echogenicity, in contrast to the more hypoechoic central area. In the deep region of the lesions was a hyperechoic area that may have been posterior enhancement. The epidermis appeared as a hyperechoic band. The lesion extended diffusely peripherally, and it was more difficult to identify its borders in this plane (Fig. 2, B and C). At the lateral borders of the lesion and in the non-nodular part of the tumor, areas of mixed echogenicity predominated over the hypoechoic areas. Color Doppler showed an irregular asymmetric neovascularization at the periphery of the lesion (Fig. 2, B and C). Histology performed on an ultrasound-guided punch biopsy revealed a poorly defined neoplastic proliferation in the dermis, forming of anastomosing vascular spaces growing between the collagen bundles and skin appendages; the spaces were lined by endothelial cells with nuclear hyperchromatism and nucleomegaly, with frequent pleomorphic nuclei (Fig. 1B). Stain for human herpesvirus 8 was negative. Based on the clinical, ultrasound, and histopathological findings, we made a diagnosis of idiopathic angiosarcoma of the head and neck. Idiopathic angiosarcoma of the head and neck is a rare neoplasm first described by Caro and Stubenrauch in 1945. It is formed of proliferations of cells with endothelial differentiation. The lesions initially appear as erythematous plaques that rapidly progress to violaceous nodules that bleed easily. This type of angiosarcoma grows centrifugally, and can