Primary cutaneous and soft tissue
angiosarcoma is a rare but highly aggressive
malignancy. To date, surgical resection is the mainstay of treatment, but poor prognosis is expected. To investigate whether there are factors associated with poor prognosis after surgical resection and to develop a treatment guideline for current
therapy, we retrospectively collected data on 28 patients who underwent surgery as initial treatment and reviewed patient demographics,
tumor characteristics, disease courses, and prognoses from September 1996 to May 2013. Of these 28 patients, 17 (60.7%) were men and the mean age at first diagnosis was 66.57 ± 18.57 years. Anatomically, 17 (60.7%)
tumors were in the scalp and 11 (39.3%) were in other sites of the body. Of the 28 patients, 23 (82.1%) had achieved
negative surgical margins, 24 (85.7%) received adjuvant
radiation therapy, and 17 (60.7%) received
adjuvant chemotherapy. Twenty-one patients (75%) died during a mean follow-up time of 35.86 ± 28.91 months, and all deaths were caused by
angiosarcoma. The 5-year overall survival rate was 17.86%. Sixteen (57.1%) patients had locoregional
tumor recurrence, and 20 (71.4%) had distant
metastases, with a median of 9.17 (range, 1.9-98.07) months to recurrence or
metastasis. Possible predictors of poor prognosis (P < 0.05) in terms of disease-free survival after surgical resection were male sex,
cardiovascular disease, smoking, and scalp
angiosarcomas, those in terms of overall survival were older than 70 years, male sex,
cardiovascular disease, smoking, scalp
angiosarcomas, distant
metastases, and not receiving
adjuvant chemotherapy. In conclusion, although
multimodal treatments are used, the overall prognosis after surgical resection is still poor, especially for patients with the above predictive factors. An early diagnosis and complete resection of the primary
tumor with or without
adjuvant radiotherapy and
chemotherapy are suggested for a potential better outcome. For those who have a diffuse lesion pattern with the involvement of vital structures, recurrence, or
metastasis, palliative resection could be an alternative treatment choice.