Clinical Review

Treatment of Angiosarcoma of the Head and Neck: A Systematic Review

Cutis. 2023 May;111(5):247-251,E1 | doi:10.12788/cutis.0767

Jessica E. Houpe, MD

Edward W. Seger, MD, MS

Brett C. Neill, MD

Bianca Y. Kang, MD

Thomas L.H. Hocker, MD

Murad Alam, MD, MBA, MSCI

Stanislav N. Tolkachjov, MD

Primary cutaneous angiosarcoma (cAS) of the head and neck is a rare sarcoma with a poor prognosis and limited treatment options. We conducted a systematic review of treatments used for head and neck cAS and determined the treatment modalities that offer the longest mean overall survival (OS). Forty publications totaling 1295 patients were included. Both surgical and nonsurgical modalities have shown potential efficacy in the treatment of cAS; however, limited data preclude definitive recommendations. Multidisciplinary management of cAS should be considered to tailor treatment on a case-by-case basis.

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Practice Points

Angiosarcoma is a rare tumor that is difficult to treat, with multiple treatment options being utilized.
Within this systematic review, wide local excision (WLE) combined with radiotherapy (RT), chemotherapy, and immunotherapy, as well as Mohs micrographic surgery (MMS), offered the longest mean (SD) overall survival time.
When clinicians are tasked with treating primary cutaneous angiosarcoma of the head and neck, they should consider MMS or WLE combined with RT.

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Cutaneous angiosarcoma (cAS) is a rare malignancy arising from vascular or lymphatic tissue. It classically presents during the sixth or seventh decades of life as a raised purple papule or plaque on the head and neck areas.¹ Primary cAS frequently mimics benign conditions, leading to delays in care. Such delays coupled with the aggressive nature of angiosarcomas leads to a poor prognosis. Five-year survival rates range from 11% to 50%, and more than half of patients die within 1 year of diagnosis.^2-7

Currently, there is no consensus on the most effective treatments, as the rare nature of cAS has made the development of controlled clinical trials difficult. Wide local excision (WLE) is most frequently employed; however, the tumor’s infiltrative growth makes complete resection and negative surgical margins difficult to achieve.⁸ Recently, Mohs micrographic surgery (MMS) has been postulated as a treatment option. The tissue-sparing nature and intraoperative margin control of MMS may provide tumor eradication and cosmesis benefits reported with other cutaneous malignancies.⁹

Nearly all localized cASs are treated with surgical excision with or without adjuvant treatment modalities; however, it is unclear which of these modalities provide a survival benefit. We conducted a systematic review of the literature to compare treatment modalities for localized cAS of the head and neck regions and to compare treatments based on tumor stage.

METHODS

A literature search was performed to identify published studies indexed by MEDLINE, Cochrane Central Register of Controlled Trials (CENTRAL), Embase, and PubMed from January 1, 1977, to May 8, 2020, reporting on cAS and treatment modalities used. The search was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines.⁵ Data extracted included patient demographics, tumor characteristics (including T1 [≤5 cm] and T2 [>5 cm and ≤10 cm] based on the American Joint Committee on Cancer soft tissue sarcoma staging criteria), treatments used, follow-up time, overall survival (OS) rates, and complications.^10,11

Studies were required to (1) include participants with head and neck cAS; (2) report original patient data following cAS treatment with surgical (WLE or MMS) and/or nonsurgical modalities (chemotherapy [CT], radiotherapy [RT], immunotherapy [IT]); (3) report outcome data related to OS rates following treatment; and (4) have articles published in English. Given the rare nature of cAS, there was no limitation on the number of participants needed.

The Newcastle-Ottawa scale for observational studies was used to assess the quality of studies.¹² Higher scores indicate low risk of bias, while lower scores represent high risk of bias.

Continuous data were reported with means and SDs, while categorical variables were reported as percentages. Overall survival means and SDs were compared between treatment modalities using an independent sample t test with P<.05 considered statistically significant. Due to the heterogeneity of the data, a meta-analysis was not reported.

Guidelines for assessing cancer risk may need updating

Treatment of Angiosarcoma of the Head and Neck: A Systematic Review

References

METHODS

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