Abstract

Primary Cutaneous Angiosarcoma (PCA) is a rare but aggressive malignant neoplasm with propensity for metastasis and persistence. The etiology of PCAs is unknown, however, a frequent association with sun exposure, radiation and lymphedema have been reported. Prognosis is poor with a mean 5-year survival rate of less than 30%. Positive tumor resection margins, involvement of the scalp, and old age are associated with poor survival prognosis, while surgical treatment with negative margins is associated with improved survival. Histologically, angiosarcomas, show wide morphological variation, even within different areas of the same tumor. Common histological features include different sizes and shapes of vascular spaces with areas of anastomosis. Neoplastic cells are usually pleomorphic and may have cytoplasmic vacuoles. Mitotic figures are frequently identified. We report 3 unusual presentations of PCAs. First case presented in an 84-year-old female with right cheek lesion. Histological examination revealed irregular dilated blood vessels lined by prominent endothelial cells, some of which presented inside the lumina of the vessels. Second case presented in an 86-year-old female with two lesions on the nose and neck. Sebaceous differentiation was identified on the neck lesion. Third case is a 22-year-old male which initially presented with a” cyst” on the scalp for more than a year. At the time of diagnosis, widespread metastasis was identified. This study is aimed to report three unusual presentations of PCAs.

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