• clinical features;
  • cutaneous angiosarcoma;
  • immunohistochemistry;
  • survival


Background  Cutaneous angiosarcoma (AS) is a rare malignant tumour of endothelial origin with very poor prognosis, frequent recurrences and high metastatic potential. Clinical suspicion is often raised too late, but histological findings and immunohistochemical assays have proved to be very helpful in the diagnostic process.

Patients and methods  Over the last 13 years, nine patients with AS were found in our archives. Clinical features, evolution, treatment and outcome were analysed and all biopsy specimens were reviewed by a trained dermatopathologist, with subsequent immunohistochemical assessment.

Results and conclusions  Cutaneous AS was clinically diagnosed in 4 of 9 patients, while systemic lupus erythematosus was the most common misdiagnosis. Radiotherapy was the most prescribed treatment, but many different combinations of surgery, chemotherapy and radiotherapy were observed. Mean disease-free and overall survival (15.4 and 23.7 respectively) were consistent with previous series, with local recurrence rate (2/9) lower than previously reported data. CD31 was positive in all patients. Vimentin, D2-40 and VEGFR-3 were expressed by the vast majority, Factor VIII by 3/7 and CD34 by about 1/3 of patients. Cytokeratin was negative in all patients. The patients with the most unfavourable course showed a strong expression of Ki-67, while those with the best outcome only had a slight positive Ki-67 staining. Larger studies regarding tumour cell expression of Ki-67 and other markers such as D2-40 will be helpful to evaluate a potential prognostic value of these stainings.