Conflict of interest None declared.
Cutaneous angiosarcoma: own experience over 13 years. Clinical features, disease course and immunohistochemical profile
Article first published online: 2 SEP 2010
© 2010 The Authors. Journal of the European Academy of Dermatology and Venereology © 2010 European Academy of Dermatology and Venereology
Journal of the European Academy of Dermatology and Venereology
Volume 24, Issue 10, pages 1230–1234, October 2010
How to Cite
Donghi, D., Kerl, K., Dummer, R., Schoenewolf, N. and Cozzio, A. (2010), Cutaneous angiosarcoma: own experience over 13 years. Clinical features, disease course and immunohistochemical profile. Journal of the European Academy of Dermatology and Venereology, 24: 1230–1234. doi: 10.1111/j.1468-3083.2010.03624.x
Written informed consent was obtained from contacted patients who were still alive at submission date.
- Issue published online: 2 SEP 2010
- Article first published online: 2 SEP 2010
- Received: 17 November 2009; Accepted: 21 January 2010
- clinical features;
- cutaneous angiosarcoma;
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.