How is angiosarcoma of the scalp treated?

Updated: Jan 03, 2020
  • Author: Jonathan S Zager, MD, FACS; Chief Editor: Gregory Gary Caputy, MD, PhD, FICS  more...
  • Print


The optimum treatment of cutaneous angiosarcoma has not been defined. Generally, radical surgery and postoperative radiotherapy are advocated to treat patients with these tumors. [20] In many patients, surgery often is not feasible because of the multifocal nature and local spread pattern of these tumors. Achieving a negative surgical margin is frequently difficult in patients with scalp angiosarcoma because of the extensive microscopic spread common with this disease. To assist in achieving negative margins, intraoperative frozen specimens are often obtained to help guide the extent of the resection.

Pawlik et al, however, showed that analysis of frozen specimens is not accurate for evaluating the extent of disease at the surgical margins. [16] Others have reported that the use of Mohs surgery similarly does not improve the ability to accurately define tumor-free margins. Thus, even with multiple operations and resections, the goal of histologically negative margins remains elusive. Farhood et al, in a review of patients with head and neck sarcomas of various histologic types, reported that pathologic margins obtained by wide excision were positive in more than 50% of patients. [21] In the experience of Mark et al, only 1 of 12 patients had the disease locally controlled. [22] Holden et al reported one cure in 7 patients treated with surgery alone. [15]

Given the poor results obtained with surgery alone, radiation therapy has been offered as possible adjuvant therapy. Some authors report that radiation therapy provides no benefit. For example, a study by Zhang et al of 42 individuals with primary scalp angiosarcoma, including 39 who underwent surgery, either by itself, in combination with chemotherapy or radiation therapy, or in three-modality therapy, indicated that patients do not significantly benefit from a particular form of treatment with regard to overall and recurrence-free survival rates. [23] Other reports, however, have suggested that surgery combined with radiation therapy offers the best prognosis.

In contrast, the Cassidy study, as discussed above, found 1- and 5-year survival rates to be better in individuals treated with definitive surgery. [18] Similarly, a retrospective study by Oashi et al reported that curative-intent surgery led to improved overall survival in patients with primary cutaneous angiosarcoma of the scalp and face. Evidence indicated that such treatment is beneficial even when the lesion is over 5 cm in size, with negative margins and the use of combined modalities not significantly affecting overall survival in surgical patients. [24]

Mark et al reported improved disease-free survival (DFS) with the addition of radiation therapy to surgery. [22] Similarly, Hodgkinson et al from the Mayo Clinic reported that the only 2 survivors in their series underwent surgery and received radiation therapy. [25] Pawlik et al reported that a prior history of radiation therapy strongly impacted survival upon both univariate and multivariate analyses. In fact, patients who received radiation therapy had a median survival almost 4 times longer than patients who did not receive radiation therapy.

A literature review by Hwang et al indicated that in patients with angiosarcoma of the face and scalp, longer overall survival can be achieved with a combination of radiation therapy and chemotherapy (37.0 mo) than with either type of therapy alone (22.7 and 15.1 mo, respectively). [26]

A retrospective study by Patel et al of patients with angiosarcoma of the face or scalp also indicated that the disease responds better to multimodal therapy than to individual treatments alone. The study included 55 patients, 40 of whom underwent a combination of surgery, radiation treatment, and/or chemotherapy, with the rest treated with just one of these modalities (with the exception of one patient who underwent observation without treatment). The investigators determined that the multimodality patients had a better 5-year rate of locoregional disease control than did the others (20% vs 11%, respectively), as well as higher rates of recurrence-free survival (19% vs 10%, respectively) and overall survival (46% vs 16%, respectively). [27]

Morrison et al noted, however, that angiosarcoma often recurs at the radiation-field margins, sometimes at striking distances from clinically evident disease. [7] Additionally, the disease often recurs at regional and distant sites. For these reasons, the development of new approaches, including more effective local and systemic therapy, is gravely needed.

Did this answer your question?
Additional feedback? (Optional)
Thank you for your feedback!