Abstract

Cutaneous angiosarcoma is a rare and highly aggressive neoplasm with poor prognosis. Owing to its variable presentation, it may be a challenging clinical and histological diagnosis. Herein, we describe a particular case of cutaneous angiosarcoma of the head and neck in a 71-year-old male with skin type V who presented with solid, progressive eyelid edema. A histological examination of skin biopsies first concluded cutaneous metastasis of an undifferentiated carcinomatous tumor. Immunostaining was essential to reach the correct diagnosis. The treatment of cutaneous angiosarcoma remains unsatisfactory. We sincerely hope that the prognosis of cutaneous angiosarcoma will be improved with the use of targeted therapies based on current genetic studies as it has been for melanoma.

Highlights

  • Angiosarcoma (AS) is a rare, highly aggressive malignant neoplasm derived from vascular endothelial cells that shows a predilection for the skin and superficial soft tissues

  • Having in mind the clinical progression of the purplish lesions to ulcerations and the hematoma-like lesions on the forehead, the diagnosis of cutaneous angiosarcoma was suggested, which was confirmed by diffuse and intense expression of CD31 combined with the erythroblast transformation-specific related gene (ERG) marker by the above-described tumor cells (Figs. 5 and 6)

  • This is a rare example of a new and challenging diagnosis of cutaneous angiosarcoma presenting as a solid, progressive eyelid edema

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Summary

INTRODUCTION

Angiosarcoma (AS) is a rare, highly aggressive malignant neoplasm derived from vascular endothelial cells that shows a predilection for the skin and superficial soft tissues. We describe a case of cutaneous angiosarcoma of the head and neck in a 71-year-old male with skin type V who presented with solid, progressive eyelid edema. A 71-year-old male, a diabetic and chronic tobacco user, consulted for eyelid edema associated with erosive lesions of the scalp evolving for the previous eight months. A clinical examination revealed a solid white edema of both eyelids, more marked on the left (Fig. 1), preventing the eye from opening, with some erosive lesions raising infiltrated erythematous and violaceous plaques of the scalp, overflowing on the forehead, temples, and retroauricular areas (Fig. 2). Having in mind the clinical progression of the purplish lesions to ulcerations and the hematoma-like lesions on the forehead, the diagnosis of cutaneous angiosarcoma was suggested, which was confirmed by diffuse and intense expression of CD31 combined with the ERG marker by the above-described tumor cells Palliative care was provided and the patient passed away six months later at his home

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