A 66-year-old man presented with chronic bilateral periorbital edema with associated yellowish hue, scattered violaceous smooth macules and contracture of the forehead. He had undergone dental surgery 3 months prior to symptom onset. Laboratory workup for common causes of eyelid edema was unremarkable and MRI of the orbits was unrevealing. The patient did not respond to oral corticosteroids or antibiotics. Punch biopsies were obtained which revealed atypical lymphatic endothelial cells consistent with a diagnosis of cutaneous angiosarcoma.The patient was deemed not to be a surgical candidate and underwent 3 cycles of immunotherapy with limited response. He declined further treatment and transitioned to hospice care. Although cutaneous angiosarcoma uncommonly involves the periorbital region, it should be considered in the differential diagnosis of eyelid edema as early recognition and treatment are critical to prevent rapid intradermal spread and metastases.
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http://dx.doi.org/10.1080/01676830.2022.2056901 | DOI Listing |
Cancer Sci
January 2025
Department of Dermatology, Graduate School of Medicine, University of the Ryukyus, Okinawa, Japan.
Angiosarcoma (AS) is a rare, aggressive malignancy originating from vascular or lymphatic endothelial cells. Despite its severity, little is known about its epidemiology, and no geographical regions have previously been identified as having an exceptionally high incidence. We retrospectively analyzed medical records spanning 37 years (1987-2023) in Okinawa, Japan, identifying 135 cases of AS that were used to calculate its incidence.
View Article and Find Full Text PDFJ Craniofac Surg
December 2024
Division of Neurosurgery, Department of Surgery.
Cutaneous angiosarcoma is a rare and aggressive malignancy originating from endothelial cells lining blood vessels in the skin. The authors present a comprehensive case report of cutaneous angiosarcoma with infiltration of the scalp and skull, with an abnormal presentation highlighting the clinical features, diagnostic challenges, treatment and surgical strategies, and outcomes. The case underscores the complexity of managing this aggressive disease involving critical anatomical structures and emphasizes the need for a multidisciplinary approach to optimal patient care.
View Article and Find Full Text PDFAm J Dermatopathol
December 2024
Department of Pathology and Lab Medicine, All India Institute of Medical Sciences (AIIMS), Bhubaneswar, India; and.
Composite hemangioendothelioma comprises permutations of different histological patterns few of which have been found to have specific genetic alteration and immunohistochemical expression. It comprises retiform or epithelioid hemangioendothelioma-like areas, with a variable proportion of hemangioma or low-grade angiosarcoma-like areas. It was found to express neuroendocrine markers and was seen to have a worse prognosis in recurrence or distant metastasis.
View Article and Find Full Text PDFJ Cutan Pathol
February 2025
Department of Pathology and Dermatology, Virginia Commonwealth University Health System, Richmond, Virginia, USA.
While most forms of alopecia neoplastica are attributable to cutaneous metastases from visceral primary malignancies, rarely a diffuse primary skin cancer may present as alopecia. Herein, we present a case of angiosarcoma which clinically mimicked an inflammatory alopecia and was diagnosed by examination of alopecia-protocol horizontal histologic sections. A 72-year-old female presented to her dermatologist with a chief complaint of hair loss and pruritus.
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