What is the prognosis of cutaneous angiosarcoma of the scalp?

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

Overall, the prognosis for cutaneous angiosarcoma is poor. Angiosarcoma has a tendency for metastasis via lymphatic or hematogenous routes, and late local recurrence and metastasis after years of apparent remission and successful local control are well documented. A number of large clinical series have assessed prognostic factors. [12, 13]

Tumor size may affect prognosis. Weiss and Goldblum report tumors less than 5 cm in diameter are associated with a significantly better prognosis than larger lesions. [14] Holden et al analyzed patients with tumors smaller than 5 cm, 5-10 cm, and larger than 10 cm and demonstrated a statistically significant correlation between tumor size and survival rate. [15] Multiple studies have compared tumors larger and smaller than 5 cm, all concluding that smaller tumors have a better overall prognosis for overall survival (OS) and/or disease-free survival (DFS). Perez et al, Maddox and Evans, and Pawlik et al report smaller (< 5 cm) tumors are associated with significantly longer OS and DFS. [8, 16, 1] In the most recent of these studies, Perez et al showed median 5-year OS of 48.4% for smaller than 5 cm versus 11.5% for larger than 5 cm. Thus, one of the most important factors in determining prognosis with cutaneous angiosarcoma appears to be initial lesion size.

Other factors have a less substantial effect on prognosis. Maddox and Evans found that patients with moderate or marked lymphocytic inflammatory responses in the tumor survived longer (P = .002). [8] Pawlik et al noted that patients with multifocal disease had decreased DFS compared with patients who had only one lesion. [16] In contrast, tumor grade did not appear to correlate with survival. Holden et al also could not correlate histopathologic grade with survival outcome. [15] Despite this, some researchers contend that low-grade tumors have a more indolent course and a better prognosis than high-grade lesions.

A retrospective study by Alqumber et al of 15 patients indicated that in facial and scalp angiosarcoma, treatment prognosis is not influenced by the reconstructive method. The investigators determined that reconstruction with a flap rather than a skin graft led to delayed median time to recurrence detection (8.75 vs 7.32 mo, respectively) and earlier median time to metastasis (3.75 vs 6.53 mo, respectively), but that both methods were associated with the same median overall survival period (16.7 mo). [17]

A study by Cassidy et al indicated that in patients with nonmetastatic scalp angiosarcoma, the overall survival rate is worse in those aged 65 years or older, in patients with a tumor size of 5 cm or greater, and in individuals who are not treated with definitive surgery. The investigators estimated that study patients who underwent definitive surgery had 1- and 5-year survival rates of 78.2% and 34.1%, respectively, compared with 68.0% and 18.0%, respectively, for patients treated not with definitive surgery but with definitive radiation or a combination of radiation and chemotherapy. [18]

A study by Bernstein et al indicated that survival rates associated with angiosarcoma of the scalp are worse than for angiosarcoma of the face. Scalp and facial angiosarcoma had 5-year locoregional control rates of 9% and 53%, respectively, as well as recurrence-free survival rates of 5% and 27%, respectively, and overall survival rates of 9% and 26%, respectively. In addition, scalp angiosarcomas tended to present at a larger size, possibly because they were less likely to be noticed until they were more advanced. [19]

Death from disease occurs by metastasis, local extension, or tumor invasion. Recurrences and metastases are usually noted within 2 years of diagnosis. Holden et al reported that only 12% of patients survived 5 years or longer, with half dying within 15 months of presentation. [15] In a single institutional review, Perez et al reported 5-year DFS and OS to be 32% and 35%, respectively. [1]


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