Angiosarcoma Presenting as Hemifacial Edema

With this report, we describe a patient with locally advanced cutaneous angiosarcoma that presented with periorbital induration and recommend a systematic approach to the diagnostic workup of chronic, nonspecific facial edema. A 69-year-old man presented with progressive, asymmetric periorbital edema over 12 months. He had a comprehensive evaluation across multiple specialties with extensive imaging, laboratory studies, and empiric treatments with glucocorticoids and antibiotics. Punch biopsy of the involved skin revealed cutaneous angiosarcoma. Positron-emission tomography-CT identified increased metabolic activity in the left periorbital region and lymph nodes. The tumor was deemed locally advanced and inoperable. Pembrolizumab was initiated after initial treatment with taxol was not tolerated. Facial edema that progresses despite comprehensive workup and empiric treatment warrants a broad, structured diagnostic approach, including advanced imaging and dermal punch biopsy. This case exemplifies the utility of this approach and reinforces the importance of timely diagnosis in investigating elusive causes of periorbital edema.