Rapidly Progressive Periorbital Oedema: A Case of Cutaneous Angiosarcoma

Abstract

Angiosarcoma is a rare form of malignant endothelial cell tumour characterised by rapidly infiltrating anaplastic cells of vascular or lymphatic origin. We report an uncommon case of cutaneous angiosarcoma (cAS) manifesting as rapidly progressive unilateral periorbital oedema. Due to the acute onset of disease, the patient was initially treated with antibiotics for presumed periorbital cellulitis. The lack of response to conservative management raised the suspicion of a more serious condition, which eventually revealed the diagnosis of angiosarcoma through skin biopsy. As suggested by several previous case reports, the subtle manifestation of cAS made it a great mimicker of benign skin conditions. This case report serves as a reminder to the aggressive nature of angiosarcoma which can lead to marked facial swelling within several weeks. As the tumour was not resectable by the time of diagnosis, the patient was offered palliative radiotherapy.

Keywords: Angiosarcoma; Eyelid; Oedema; Periorbital.

Fig. 1

Right unilateral periorbital oedema.

Fig. 2

Angiosarcoma (on the right) adjacent to SCC (on the left) with intervening normal sebaceous glands. The tumour consists of irregular anastomosing channels lined by markedly pleomorphic cells. The vessels dissect through the dermis, and the tumour is poorly circumscribed. This is a relatively well-differentiated area, so you can see the obvious vascular nature of the tumour.

Fig. 3

ERG showing the tumour dissecting through the dermis into the subcutaneous fat. Note the above SCC does not stain for this marker, allowing clear distinction.