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Treatment of Angiosarcoma of the Head and Neck: A Systematic Review
Author(s)
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

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.

 

 

RESULTS

Literature Search and Risk of Bias Assessment

There were 283 manuscripts identified, 56 articles read in full, and 40 articles included in the review (Figure). Among the 16 studies not meeting inclusion criteria, 7 did not provide enough data to isolate head and neck cAS cases,1,13-18 6 did not report outcomes related to the current review,19-24 and 3 did not provide enough data to isolate different treatment outcomes.25-27 Among the included studies, 32 reported use of WLE: WLE alone (n=21)2,7,11,28-45; WLE with RT (n=24)2,3,11,28-31,33-36,38-41,43-51; WLE with CT (n=7)2,31,35,39,41,48,52; WLE with RT and CT (n=11)2,29,31,33-35,39,40,48,52,53; WLE with RT and IT (n=3)35,54,55; and WLE with RT, CT, and IT (n=1).53 Nine studies reported MMS: MMS alone (n=5)39,56-59; MMS with RT (n=3)32,50,60,61; and MMS with RT and CT (n=1).51

CT111005247_Figure.jpg
%3Cp%3EFlow%20diagram%20depicting%20search%20strategy%20and%20study%20inclusion%20from%20a%20literature%20search%20performed%20to%20identify%20published%20studies%20indexed%20by%20MEDLINE%2C%20Cochrane%20Central%20Register%20of%20Controlled%20Trials%20(CENTRAL)%2C%20Embase%2C%20and%20PubMed%20from%20January%201%2C%201977%2C%20to%20May%208%2C%202020%2C%20reporting%20on%20cutaneous%20angiosarcoma%20and%20treatment%20modalities%20used.%3C%2Fp%3E

Risk of bias assessment identified low risk in 3 articles. High risk was identified in 5 case reports,57-61 and 1 study did not describe patient selection.43 Clayton et al56 showed intermediate risk, given the study controlled for 1 factor.

Patient Demographics

A total of 1295 patients were included. The pooled mean age of the patients was 67.5 years (range, 3–88 years), and 64.7% were male. There were 79 cases identified as T1 and 105 as T2. A total of 825 cases were treated using WLE with or without adjuvant therapy, while a total of 9 cases were treated using MMS with and without adjuvant therapies (Table). There were 461 cases treated without surgical excision: RT alone (n=261), CT alone (n=38), IT alone (n=35), RT with CT (n=81), RT with IT (n=34), and RT with CT and IT (n=12)(Table). The median follow-up period across all studies was 23.5 months (range, 1–228 months).

CT111005247_Table.jpg

Comparison Between Surgical and Nonsurgical Modalities

Wide Local Excision—Wide local excision (n=825; 63.7%) alone or in combination with other therapies was the most frequently used treatment modality. The mean (SD) OS was longest for WLE with RT, CT, and IT (n=3; 39.3 [24.1]), followed by WLE with RT (n=447; 35.9 [34.3] months), WLE with CT (n=13; 32.4 [30.2] months), WLE alone (n=324; 29.6 [34.1] months), WLE with RT and IT (n=11; 23.5 [4.9] months), and WLE with RT and CT (n=27; 20.7 [13.1] months).

Nonsurgical Modalities—Nonsurgical methods were used less frequently than surgical methods (n=461; 35.6%). The mean (SD) OS time in descending order was as follows: RT with CT and IT (n=12; 34.9 [1.2] months), RT with CT (n=81; 30.4 [37.8] months), IT alone (n=35; 25.7 [no SD reported] months), RT with IT (n=34; 20.5 [8.6] months), CT alone (n=38; 20.1 [15.9] months), and RT alone (n=261; 12.8 [8.3] months).

When comparing mean (SD) OS outcomes between surgical and nonsurgical treatment modalities, only the addition of WLE to RT significantly increased OS when compared with RT alone (WLE, 35.9 [34.3] months; RT alone, 12.8 [8.3] months; P=.001). When WLE was added to CT or both RT and CT, there was no significant difference with OS when compared with CT alone (WLE with CT, 32.4 [30.2] months; CT alone, 20.1 [15.9] months; P=.065); or both RT and CT in combination (WLE with RT and CT, 20.7 [13.1] months; RT and CT, 30.4 [37.8] months; P=.204).

Comparison Between T1 and T2 cAS

T1 Angiosarcoma—There were 79 patients identified as having T1 tumors across 16 studies.2,31,32,34,39-41,46,48-50,53,58-60,62 The mean (SD) OS was longest for WLE with RT, CT, and IT (n=2; 56.0 [6.0] months), followed by WLE with CT (n=4; 54.5 [41.0] months); WLE with RT (n=30; 39.7 [41.2] months); WLE alone (n=22; 37.2 [37.3] months); WLE with both RT and CT (n=7; 25.5 [18.7] months); RT with IT (n=2; 20.0 [11.0] months); RT with CT (n=6; 15.7 [6.8] months); and RT alone (n=1; 13 [no SD]) months)(eTable).

CT111005247_eTable.jpg

 

 

T2 Angiosarcoma—There were 105 patients with T2 tumors in 15 studies.2,31,32,34,39-41,46,48-50,52,53,57,62 The mean (SD) OS for each treatment modality in descending order was as follows: RT with CT and IT (n=1; 36 [no SD reported] months); RT with CT (n=23; 34.3 [46.3] months); WLE with RT (n=21; 26.3 [23.8] months); WLE with CT (n=8; 21.5 [16.6] months); WLE alone (n=16; 19.8 [15.6] months); WLE with RT and CT (n=14; 19.2 [10.5] months); RT alone (n=17; 10.1 [5.5] months); CT alone (n=2; 6.7 [3.7] months); and WLE with RT, CT, and IT (n=1; 6.0 [no SD] months)(eTable).

Mohs Micrographic Surgery—The use of MMS was only identified in case reports or small observational studies for a total of 9 patients. Five cASs were treated with MMS alone for a mean (SD) OS of 37 (21.5) months, with 4 reporting cAS staging: 2 were T158,59 (mean [SD] OS, 37.0 [17.0] months) and 2 were T2 tumors39,57 (mean [SD] OS, 44.5 [26.5] months). Mohs micrographic surgery with RT was used for 3 tumors (mean [SD] OS, 34.0 [26.9] months); 2 were T150,60 (mean [SD] OS, 42.0 [30.0] months) and 1 unreported staging (eTable).56 Mohs micrographic surgery with both RT and CT was used in 1 patient (unreported staging; OS, 82 months).51

Complications

Complications were rare and mainly associated with CT and RT. Four studies reported radiation dermatitis with RT.53,55,62,63 Two studies reported peripheral neuropathy and myelotoxicity with CT.35,51 Only 1 study reported poor wound healing due to surgical complications.29

COMMENT

Cutaneous angiosarcomas are rare and have limited treatment guidelines. Surgical excision does appear to be an effective adjunct to nonsurgical treatments, particularly WLE combined with RT, CT, and IT. Although MMS ultimately may be useful for cAS, the limited number and substantial heterogeneity of reported cases precludes definitive conclusions at this time.

Achieving margin control during WLE is associated with higher OS when treating angiosarcoma,36,46 which is particularly true for T1 tumors where margin control is imperative, and many cases are treated with a combination of WLE and RT. Overall survival times are lower for T2 tumors, as these tumors are larger and most likely have spread; therefore, more aggressive combination treatments were more prevalent. In these cases, complete margin control may be difficult to achieve and may not be as critical to the outcome if another form of adjuvant therapy can be administered promptly.24,64

When surgery is contraindicated, RT with or without CT was the most commonly reported treatment modality. However, these treatments were notably less effective than when used in combination with surgical resection. The use of RT alone has a recurrence rate reported up to 100% in certain studies, suggesting the need to utilize RT in combination with other modalities.23,39 It is important to note that RT often is used as monotherapy in palliative treatment, which may indirectly skew survival rates.2

Limitations of the study include a lack of randomized controlled trials. Most reports were retrospective reviews or case series, and tumor staging was sparsely reported. Finally, although MMS may provide utility in the treatment of cAS, the sample size of 9 precluded definitive conclusions from being formed about its efficacy.

CONCLUSION

Cutaneous angiosarcoma is rare and has limited data comparing different treatment modalities. The paucity of data currently limits definitive recommendations; however, both surgical and nonsurgical modalities have demonstrated potential efficacy in the treatment of cAS and may benefit from additional research. Clinicians should consider a multidisciplinary approach for patients with a diagnosis of cAS to tailor treatments on a case-by-case basis.

References
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Author and Disclosure Information

Dr. Houpe is from the University of Kansas School of Medicine, Kansas City. Drs. Seger, Neill, and Hocker are from the Division of Dermatology, University of Kansas Medical Center, Kansas City. Drs. Kang and Alam are from the Department of Dermatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois. Dr. Alam also is from the Departments of Otolaryngology and Surgery. Dr. Tolkachjov is from Epiphany Dermatology, Lewisville, Texas; the Department of Dermatology, University of Texas at Southwestern, Dallas; Baylor University Medical Center, Dallas; and the Texas A&M College of Medicine, Dallas.

Drs. Neill, Kang, and Hocker report no conflict of interest. Dr. Tolkachjov is on the medical advisory board for Illumisonics Inc and is a speaker and an investigator for Bioventus and Castle Biosciences. He also received a research grant from Castle Biosciences.

The eTable is available in the Appendix online at www.mdedge.com/dermatology.

Correspondence: Stanislav N. Tolkachjov, MD, Epiphany Dermatology, 1640 FM 544, Ste 100, Lewisville, TX 75056 (stan.tolkachjov@gmail.com).

Issue
Cutis - 111(5)
Publications
MDedge Dermatology
Cutis
Topics
Nonmelanoma Skin Cancer
Page Number
247-251,E1
Sections
Clinical Review
Author(s)
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
Author(s)
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
Author and Disclosure Information

Dr. Houpe is from the University of Kansas School of Medicine, Kansas City. Drs. Seger, Neill, and Hocker are from the Division of Dermatology, University of Kansas Medical Center, Kansas City. Drs. Kang and Alam are from the Department of Dermatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois. Dr. Alam also is from the Departments of Otolaryngology and Surgery. Dr. Tolkachjov is from Epiphany Dermatology, Lewisville, Texas; the Department of Dermatology, University of Texas at Southwestern, Dallas; Baylor University Medical Center, Dallas; and the Texas A&M College of Medicine, Dallas.

Drs. Neill, Kang, and Hocker report no conflict of interest. Dr. Tolkachjov is on the medical advisory board for Illumisonics Inc and is a speaker and an investigator for Bioventus and Castle Biosciences. He also received a research grant from Castle Biosciences.

The eTable is available in the Appendix online at www.mdedge.com/dermatology.

Correspondence: Stanislav N. Tolkachjov, MD, Epiphany Dermatology, 1640 FM 544, Ste 100, Lewisville, TX 75056 (stan.tolkachjov@gmail.com).

Author and Disclosure Information

Dr. Houpe is from the University of Kansas School of Medicine, Kansas City. Drs. Seger, Neill, and Hocker are from the Division of Dermatology, University of Kansas Medical Center, Kansas City. Drs. Kang and Alam are from the Department of Dermatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois. Dr. Alam also is from the Departments of Otolaryngology and Surgery. Dr. Tolkachjov is from Epiphany Dermatology, Lewisville, Texas; the Department of Dermatology, University of Texas at Southwestern, Dallas; Baylor University Medical Center, Dallas; and the Texas A&M College of Medicine, Dallas.

Drs. Neill, Kang, and Hocker report no conflict of interest. Dr. Tolkachjov is on the medical advisory board for Illumisonics Inc and is a speaker and an investigator for Bioventus and Castle Biosciences. He also received a research grant from Castle Biosciences.

The eTable is available in the Appendix online at www.mdedge.com/dermatology.

Correspondence: Stanislav N. Tolkachjov, MD, Epiphany Dermatology, 1640 FM 544, Ste 100, Lewisville, TX 75056 (stan.tolkachjov@gmail.com).

Article PDF
CT111005247.pdf
Article PDF
CT111005247.pdf

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.

 

 

RESULTS

Literature Search and Risk of Bias Assessment

There were 283 manuscripts identified, 56 articles read in full, and 40 articles included in the review (Figure). Among the 16 studies not meeting inclusion criteria, 7 did not provide enough data to isolate head and neck cAS cases,1,13-18 6 did not report outcomes related to the current review,19-24 and 3 did not provide enough data to isolate different treatment outcomes.25-27 Among the included studies, 32 reported use of WLE: WLE alone (n=21)2,7,11,28-45; WLE with RT (n=24)2,3,11,28-31,33-36,38-41,43-51; WLE with CT (n=7)2,31,35,39,41,48,52; WLE with RT and CT (n=11)2,29,31,33-35,39,40,48,52,53; WLE with RT and IT (n=3)35,54,55; and WLE with RT, CT, and IT (n=1).53 Nine studies reported MMS: MMS alone (n=5)39,56-59; MMS with RT (n=3)32,50,60,61; and MMS with RT and CT (n=1).51

CT111005247_Figure.jpg
%3Cp%3EFlow%20diagram%20depicting%20search%20strategy%20and%20study%20inclusion%20from%20a%20literature%20search%20performed%20to%20identify%20published%20studies%20indexed%20by%20MEDLINE%2C%20Cochrane%20Central%20Register%20of%20Controlled%20Trials%20(CENTRAL)%2C%20Embase%2C%20and%20PubMed%20from%20January%201%2C%201977%2C%20to%20May%208%2C%202020%2C%20reporting%20on%20cutaneous%20angiosarcoma%20and%20treatment%20modalities%20used.%3C%2Fp%3E

Risk of bias assessment identified low risk in 3 articles. High risk was identified in 5 case reports,57-61 and 1 study did not describe patient selection.43 Clayton et al56 showed intermediate risk, given the study controlled for 1 factor.

Patient Demographics

A total of 1295 patients were included. The pooled mean age of the patients was 67.5 years (range, 3–88 years), and 64.7% were male. There were 79 cases identified as T1 and 105 as T2. A total of 825 cases were treated using WLE with or without adjuvant therapy, while a total of 9 cases were treated using MMS with and without adjuvant therapies (Table). There were 461 cases treated without surgical excision: RT alone (n=261), CT alone (n=38), IT alone (n=35), RT with CT (n=81), RT with IT (n=34), and RT with CT and IT (n=12)(Table). The median follow-up period across all studies was 23.5 months (range, 1–228 months).

CT111005247_Table.jpg

Comparison Between Surgical and Nonsurgical Modalities

Wide Local Excision—Wide local excision (n=825; 63.7%) alone or in combination with other therapies was the most frequently used treatment modality. The mean (SD) OS was longest for WLE with RT, CT, and IT (n=3; 39.3 [24.1]), followed by WLE with RT (n=447; 35.9 [34.3] months), WLE with CT (n=13; 32.4 [30.2] months), WLE alone (n=324; 29.6 [34.1] months), WLE with RT and IT (n=11; 23.5 [4.9] months), and WLE with RT and CT (n=27; 20.7 [13.1] months).

Nonsurgical Modalities—Nonsurgical methods were used less frequently than surgical methods (n=461; 35.6%). The mean (SD) OS time in descending order was as follows: RT with CT and IT (n=12; 34.9 [1.2] months), RT with CT (n=81; 30.4 [37.8] months), IT alone (n=35; 25.7 [no SD reported] months), RT with IT (n=34; 20.5 [8.6] months), CT alone (n=38; 20.1 [15.9] months), and RT alone (n=261; 12.8 [8.3] months).

When comparing mean (SD) OS outcomes between surgical and nonsurgical treatment modalities, only the addition of WLE to RT significantly increased OS when compared with RT alone (WLE, 35.9 [34.3] months; RT alone, 12.8 [8.3] months; P=.001). When WLE was added to CT or both RT and CT, there was no significant difference with OS when compared with CT alone (WLE with CT, 32.4 [30.2] months; CT alone, 20.1 [15.9] months; P=.065); or both RT and CT in combination (WLE with RT and CT, 20.7 [13.1] months; RT and CT, 30.4 [37.8] months; P=.204).

Comparison Between T1 and T2 cAS

T1 Angiosarcoma—There were 79 patients identified as having T1 tumors across 16 studies.2,31,32,34,39-41,46,48-50,53,58-60,62 The mean (SD) OS was longest for WLE with RT, CT, and IT (n=2; 56.0 [6.0] months), followed by WLE with CT (n=4; 54.5 [41.0] months); WLE with RT (n=30; 39.7 [41.2] months); WLE alone (n=22; 37.2 [37.3] months); WLE with both RT and CT (n=7; 25.5 [18.7] months); RT with IT (n=2; 20.0 [11.0] months); RT with CT (n=6; 15.7 [6.8] months); and RT alone (n=1; 13 [no SD]) months)(eTable).

CT111005247_eTable.jpg

 

 

T2 Angiosarcoma—There were 105 patients with T2 tumors in 15 studies.2,31,32,34,39-41,46,48-50,52,53,57,62 The mean (SD) OS for each treatment modality in descending order was as follows: RT with CT and IT (n=1; 36 [no SD reported] months); RT with CT (n=23; 34.3 [46.3] months); WLE with RT (n=21; 26.3 [23.8] months); WLE with CT (n=8; 21.5 [16.6] months); WLE alone (n=16; 19.8 [15.6] months); WLE with RT and CT (n=14; 19.2 [10.5] months); RT alone (n=17; 10.1 [5.5] months); CT alone (n=2; 6.7 [3.7] months); and WLE with RT, CT, and IT (n=1; 6.0 [no SD] months)(eTable).

Mohs Micrographic Surgery—The use of MMS was only identified in case reports or small observational studies for a total of 9 patients. Five cASs were treated with MMS alone for a mean (SD) OS of 37 (21.5) months, with 4 reporting cAS staging: 2 were T158,59 (mean [SD] OS, 37.0 [17.0] months) and 2 were T2 tumors39,57 (mean [SD] OS, 44.5 [26.5] months). Mohs micrographic surgery with RT was used for 3 tumors (mean [SD] OS, 34.0 [26.9] months); 2 were T150,60 (mean [SD] OS, 42.0 [30.0] months) and 1 unreported staging (eTable).56 Mohs micrographic surgery with both RT and CT was used in 1 patient (unreported staging; OS, 82 months).51

Complications

Complications were rare and mainly associated with CT and RT. Four studies reported radiation dermatitis with RT.53,55,62,63 Two studies reported peripheral neuropathy and myelotoxicity with CT.35,51 Only 1 study reported poor wound healing due to surgical complications.29

COMMENT

Cutaneous angiosarcomas are rare and have limited treatment guidelines. Surgical excision does appear to be an effective adjunct to nonsurgical treatments, particularly WLE combined with RT, CT, and IT. Although MMS ultimately may be useful for cAS, the limited number and substantial heterogeneity of reported cases precludes definitive conclusions at this time.

Achieving margin control during WLE is associated with higher OS when treating angiosarcoma,36,46 which is particularly true for T1 tumors where margin control is imperative, and many cases are treated with a combination of WLE and RT. Overall survival times are lower for T2 tumors, as these tumors are larger and most likely have spread; therefore, more aggressive combination treatments were more prevalent. In these cases, complete margin control may be difficult to achieve and may not be as critical to the outcome if another form of adjuvant therapy can be administered promptly.24,64

When surgery is contraindicated, RT with or without CT was the most commonly reported treatment modality. However, these treatments were notably less effective than when used in combination with surgical resection. The use of RT alone has a recurrence rate reported up to 100% in certain studies, suggesting the need to utilize RT in combination with other modalities.23,39 It is important to note that RT often is used as monotherapy in palliative treatment, which may indirectly skew survival rates.2

Limitations of the study include a lack of randomized controlled trials. Most reports were retrospective reviews or case series, and tumor staging was sparsely reported. Finally, although MMS may provide utility in the treatment of cAS, the sample size of 9 precluded definitive conclusions from being formed about its efficacy.

CONCLUSION

Cutaneous angiosarcoma is rare and has limited data comparing different treatment modalities. The paucity of data currently limits definitive recommendations; however, both surgical and nonsurgical modalities have demonstrated potential efficacy in the treatment of cAS and may benefit from additional research. Clinicians should consider a multidisciplinary approach for patients with a diagnosis of cAS to tailor treatments on a case-by-case basis.

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.

 

 

RESULTS

Literature Search and Risk of Bias Assessment

There were 283 manuscripts identified, 56 articles read in full, and 40 articles included in the review (Figure). Among the 16 studies not meeting inclusion criteria, 7 did not provide enough data to isolate head and neck cAS cases,1,13-18 6 did not report outcomes related to the current review,19-24 and 3 did not provide enough data to isolate different treatment outcomes.25-27 Among the included studies, 32 reported use of WLE: WLE alone (n=21)2,7,11,28-45; WLE with RT (n=24)2,3,11,28-31,33-36,38-41,43-51; WLE with CT (n=7)2,31,35,39,41,48,52; WLE with RT and CT (n=11)2,29,31,33-35,39,40,48,52,53; WLE with RT and IT (n=3)35,54,55; and WLE with RT, CT, and IT (n=1).53 Nine studies reported MMS: MMS alone (n=5)39,56-59; MMS with RT (n=3)32,50,60,61; and MMS with RT and CT (n=1).51

CT111005247_Figure.jpg
%3Cp%3EFlow%20diagram%20depicting%20search%20strategy%20and%20study%20inclusion%20from%20a%20literature%20search%20performed%20to%20identify%20published%20studies%20indexed%20by%20MEDLINE%2C%20Cochrane%20Central%20Register%20of%20Controlled%20Trials%20(CENTRAL)%2C%20Embase%2C%20and%20PubMed%20from%20January%201%2C%201977%2C%20to%20May%208%2C%202020%2C%20reporting%20on%20cutaneous%20angiosarcoma%20and%20treatment%20modalities%20used.%3C%2Fp%3E

Risk of bias assessment identified low risk in 3 articles. High risk was identified in 5 case reports,57-61 and 1 study did not describe patient selection.43 Clayton et al56 showed intermediate risk, given the study controlled for 1 factor.

Patient Demographics

A total of 1295 patients were included. The pooled mean age of the patients was 67.5 years (range, 3–88 years), and 64.7% were male. There were 79 cases identified as T1 and 105 as T2. A total of 825 cases were treated using WLE with or without adjuvant therapy, while a total of 9 cases were treated using MMS with and without adjuvant therapies (Table). There were 461 cases treated without surgical excision: RT alone (n=261), CT alone (n=38), IT alone (n=35), RT with CT (n=81), RT with IT (n=34), and RT with CT and IT (n=12)(Table). The median follow-up period across all studies was 23.5 months (range, 1–228 months).

CT111005247_Table.jpg

Comparison Between Surgical and Nonsurgical Modalities

Wide Local Excision—Wide local excision (n=825; 63.7%) alone or in combination with other therapies was the most frequently used treatment modality. The mean (SD) OS was longest for WLE with RT, CT, and IT (n=3; 39.3 [24.1]), followed by WLE with RT (n=447; 35.9 [34.3] months), WLE with CT (n=13; 32.4 [30.2] months), WLE alone (n=324; 29.6 [34.1] months), WLE with RT and IT (n=11; 23.5 [4.9] months), and WLE with RT and CT (n=27; 20.7 [13.1] months).

Nonsurgical Modalities—Nonsurgical methods were used less frequently than surgical methods (n=461; 35.6%). The mean (SD) OS time in descending order was as follows: RT with CT and IT (n=12; 34.9 [1.2] months), RT with CT (n=81; 30.4 [37.8] months), IT alone (n=35; 25.7 [no SD reported] months), RT with IT (n=34; 20.5 [8.6] months), CT alone (n=38; 20.1 [15.9] months), and RT alone (n=261; 12.8 [8.3] months).

When comparing mean (SD) OS outcomes between surgical and nonsurgical treatment modalities, only the addition of WLE to RT significantly increased OS when compared with RT alone (WLE, 35.9 [34.3] months; RT alone, 12.8 [8.3] months; P=.001). When WLE was added to CT or both RT and CT, there was no significant difference with OS when compared with CT alone (WLE with CT, 32.4 [30.2] months; CT alone, 20.1 [15.9] months; P=.065); or both RT and CT in combination (WLE with RT and CT, 20.7 [13.1] months; RT and CT, 30.4 [37.8] months; P=.204).

Comparison Between T1 and T2 cAS

T1 Angiosarcoma—There were 79 patients identified as having T1 tumors across 16 studies.2,31,32,34,39-41,46,48-50,53,58-60,62 The mean (SD) OS was longest for WLE with RT, CT, and IT (n=2; 56.0 [6.0] months), followed by WLE with CT (n=4; 54.5 [41.0] months); WLE with RT (n=30; 39.7 [41.2] months); WLE alone (n=22; 37.2 [37.3] months); WLE with both RT and CT (n=7; 25.5 [18.7] months); RT with IT (n=2; 20.0 [11.0] months); RT with CT (n=6; 15.7 [6.8] months); and RT alone (n=1; 13 [no SD]) months)(eTable).

CT111005247_eTable.jpg

 

 

T2 Angiosarcoma—There were 105 patients with T2 tumors in 15 studies.2,31,32,34,39-41,46,48-50,52,53,57,62 The mean (SD) OS for each treatment modality in descending order was as follows: RT with CT and IT (n=1; 36 [no SD reported] months); RT with CT (n=23; 34.3 [46.3] months); WLE with RT (n=21; 26.3 [23.8] months); WLE with CT (n=8; 21.5 [16.6] months); WLE alone (n=16; 19.8 [15.6] months); WLE with RT and CT (n=14; 19.2 [10.5] months); RT alone (n=17; 10.1 [5.5] months); CT alone (n=2; 6.7 [3.7] months); and WLE with RT, CT, and IT (n=1; 6.0 [no SD] months)(eTable).

Mohs Micrographic Surgery—The use of MMS was only identified in case reports or small observational studies for a total of 9 patients. Five cASs were treated with MMS alone for a mean (SD) OS of 37 (21.5) months, with 4 reporting cAS staging: 2 were T158,59 (mean [SD] OS, 37.0 [17.0] months) and 2 were T2 tumors39,57 (mean [SD] OS, 44.5 [26.5] months). Mohs micrographic surgery with RT was used for 3 tumors (mean [SD] OS, 34.0 [26.9] months); 2 were T150,60 (mean [SD] OS, 42.0 [30.0] months) and 1 unreported staging (eTable).56 Mohs micrographic surgery with both RT and CT was used in 1 patient (unreported staging; OS, 82 months).51

Complications

Complications were rare and mainly associated with CT and RT. Four studies reported radiation dermatitis with RT.53,55,62,63 Two studies reported peripheral neuropathy and myelotoxicity with CT.35,51 Only 1 study reported poor wound healing due to surgical complications.29

COMMENT

Cutaneous angiosarcomas are rare and have limited treatment guidelines. Surgical excision does appear to be an effective adjunct to nonsurgical treatments, particularly WLE combined with RT, CT, and IT. Although MMS ultimately may be useful for cAS, the limited number and substantial heterogeneity of reported cases precludes definitive conclusions at this time.

Achieving margin control during WLE is associated with higher OS when treating angiosarcoma,36,46 which is particularly true for T1 tumors where margin control is imperative, and many cases are treated with a combination of WLE and RT. Overall survival times are lower for T2 tumors, as these tumors are larger and most likely have spread; therefore, more aggressive combination treatments were more prevalent. In these cases, complete margin control may be difficult to achieve and may not be as critical to the outcome if another form of adjuvant therapy can be administered promptly.24,64

When surgery is contraindicated, RT with or without CT was the most commonly reported treatment modality. However, these treatments were notably less effective than when used in combination with surgical resection. The use of RT alone has a recurrence rate reported up to 100% in certain studies, suggesting the need to utilize RT in combination with other modalities.23,39 It is important to note that RT often is used as monotherapy in palliative treatment, which may indirectly skew survival rates.2

Limitations of the study include a lack of randomized controlled trials. Most reports were retrospective reviews or case series, and tumor staging was sparsely reported. Finally, although MMS may provide utility in the treatment of cAS, the sample size of 9 precluded definitive conclusions from being formed about its efficacy.

CONCLUSION

Cutaneous angiosarcoma is rare and has limited data comparing different treatment modalities. The paucity of data currently limits definitive recommendations; however, both surgical and nonsurgical modalities have demonstrated potential efficacy in the treatment of cAS and may benefit from additional research. Clinicians should consider a multidisciplinary approach for patients with a diagnosis of cAS to tailor treatments on a case-by-case basis.

References
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  44. Perez MC, Padhya TA, Messina JL, et al. Cutaneous angiosarcoma: a single-institution experience. Ann Surg Oncol. 2013;20:3391-3397.
  45. Veness M, Cooper S. Treatment of cutaneous angiosarcomas of the head and neck. Australas Radiol. 1995;39:277-281.
  46. Barttelbort SW, Stahl R, Ariyan S. Cutaneous angiosarcoma of the face and scalp. Plast Reconstr Surg. 1989;84:55-59.
  47. Bernstein JM, Irish JC, Brown DH, et al. Survival outcomes for cutaneous angiosarcoma of the scalp versus face. Head Neck. 2017;39:1205-1211.
  48. Köhler HF, Neves RI, Brechtbühl ER, et al. Cutaneous angiosarcoma of the head and neck: report of 23 cases from a single institution. Otolaryngol Head Neck Surg. 2008;139:519-524.
  49. Morales PH, Lindberg RD, Barkley HT Jr. Soft tissue angiosarcomas. Int J Radiat Oncol Biol Phys. 1981;7:1655-1659.
  50. Wollina U, Hansel G, Schönlebe J, et al. Cutaneous angiosarcoma is a rare aggressive malignant vascular tumour of the skin. J Eur Acad Dermatol Venereol. 2011;25:964-968.
  51. Wollina U, Koch A, Hansel G, et al. A 10-year analysis of cutaneous mesenchymal tumors (sarcomas and related entities) in a skin cancer center. Int J Dermatol. 2013;52:1189-1197.
  52. Bien E, Stachowicz-Stencel T, Balcerska A, et al. Angiosarcoma in children - still uncontrollable oncological problem. The report of the Polish Paediatric Rare Tumours Study. Eur J Cancer Care (Engl). 2009;18:411-420.
  53. Suzuki G, Yamazaki H, Takenaka H, et al. Definitive radiation therapy for angiosarcoma of the face and scalp. In Vivo. 2016;30:921-926.
  54. Miki Y, Tada T, Kamo R, et al. Single institutional experience of the treatment of angiosarcoma of the face and scalp. Br J Radiol. 2013;86:20130439.
  55. Ohguri T, Imada H, Nomoto S, et al. Angiosarcoma of the scalp treated with curative radiotherapy plus recombinant interleukin-2 immunotherapy. Int J Radiat Oncol Biol Phys. 2005;61:1446-1453.
  56. Clayton BD, Leshin B, Hitchcock MG, et al. Utility of rush paraffin-embedded tangential sections in the management of cutaneous neoplasms. Dermatol Surg. 2000;26:671-678.
  57. Goldberg DJ, Kim YA. Angiosarcoma of the scalp treated with Mohs micrographic surgery. J Dermatol Surg Oncol. 1993;19:156-158.
  58. Mikhail GR, Kelly AP Jr. Malignant angioendothelioma of the face. J Dermatol Surg Oncol. 1977;3:181-183.
  59. Muscarella VA. Angiosarcoma treated by Mohs micrographic surgery. J Dermatol Surg Oncol. 1993;19:1132-1133.
  60. Bullen R, Larson PO, Landeck AE, et al. Angiosarcoma of the head and neck managed by a combination of multiple biopsies to determine tumor margin and radiation therapy. report of three cases and review of the literature. Dermatol Surg. 1998;24:1105-1110.
  61. Wiwatwongwana D, White VA, Dolman PJ. Two cases of periocular cutaneous angiosarcoma. Ophthalmic Plast Reconstr Surg. 2010;26:365-366.
  62. Morrison WH, Byers RM, Garden AS, et al. Cutaneous angiosarcoma of the head and neck. A therapeutic dilemma. Cancer. 1995;76:319-327.
  63. Hata M, Wada H, Ogino I, et al. Radiation therapy for angiosarcoma of the scalp: treatment outcomes of total scalp irradiation with X-rays and electrons. Strahlenther Onkol. 2014;190:899-904.
  64. Hwang K, Kim MY, Lee SH. Recommendations for therapeutic decisions of angiosarcoma of the scalp and face. J Craniofac Surg. 2015;26:E253-E256.
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  23. Patel SH, Hayden RE, Hinni ML, et al. Angiosarcoma of the scalp and face: the Mayo Clinic experience. JAMA Otolaryngol Head Neck Surg. 2015;141:335-340.
  24. Guadagnolo BA, Zagars GK, Araujo D, et al. Outcomes after definitive treatment for cutaneous angiosarcoma of the face and scalp. Head Neck. 2011;33:661-667.
  25. Zhang Y, Yan Y, Zhu M, et al. Clinical outcomes in primary scalp angiosarcoma. Oncol Lett. 2019;18:5091-5096.
  26. Kamo R, Ishii M. Histological differentiation, histogenesis and prognosis of cutaneous angiosarcoma. Osaka City Med J. 2011;57:31-44.
  27. Ito T, Uchi H, Nakahara T, et al. Cutaneous angiosarcoma of the head and face: a single-center analysis of treatment outcomes in 43 patients in Japan. J Cancer Res Clin Oncol. 2016;142:1387-1394.
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  29. Buschmann A, Lehnhardt M, Toman N, et al. Surgical treatment of angiosarcoma of the scalp: less is more. Ann Plast Surg. 2008;61:399-403.
  30. Cassidy RJ, Switchenko JM, Yushak ML, et al. The importance of surgery in scalp angiosarcomas. Surg Oncol. 2018;27:A3-A8.
  31. Choi JH, Ahn KC, Chang H, et al. Surgical treatment and prognosis of angiosarcoma of the scalp: a retrospective analysis of 14 patients in a single institution. Biomed Res Int. 2015;2015:321896.
  32. Chow TL, Kwan WW, Kwan CK. Treatment of cutaneous angiosarcoma of the scalp and face in Chinese patients: local experience at a regional hospital in Hong Kong. Hong Kong Med J. 2018;24:25-31.
  33. Donghi D, Kerl K, Dummer R, et al. Cutaneous angiosarcoma: own experience over 13 years. clinical features, disease course and immunohistochemical profile. J Eur Acad Dermatol Venereol. 2010;24:1230-1234.
  34. Ferrari A, Casanova M, Bisogno G, et al. Malignant vascular tumors in children and adolescents: a report from the Italian and German Soft Tissue Sarcoma Cooperative Group. Med Pediatr Oncol. 2002;39:109-114.
  35. Fujisawa Y, Nakamura Y, Kawachi Y, et al. Comparison between taxane-based chemotherapy with conventional surgery-based therapy for cutaneous angiosarcoma: a single-center experience. J Dermatolog Treat. 2014;25:419-423.
  36. Hodgkinson DJ, Soule EH, Woods JE. Cutaneous angiosarcoma of the head and neck. Cancer. 1979;44:1106-1113.
  37. Lim SY, Pyon JK, Mun GH, et al. Surgical treatment of angiosarcoma of the scalp with superficial parotidectomy. Ann Plast Surg. 2010;64:180-182.
  38. Maddox JC, Evans HL. Angiosarcoma of skin and soft tissue: a study of forty-four cases. Cancer. 1981;48:1907-1921.
  39. Mark RJ, Tran LM, Sercarz J, et al. Angiosarcoma of the head and neck. The UCLA experience 1955 through 1990. Arch Otolaryngol Head Neck Surg. 1993;119:973-978.
  40. Morgan MB, Swann M, Somach S, et al. Cutaneous angiosarcoma: a case series with prognostic correlation. J Am Acad Dermatol. 2004;50:867-874.
  41. Mullins B, Hackman T. Angiosarcoma of the head and neck. Int Arch Otorhinolaryngol. 2015;19:191-195.
  42. Ogawa K, Takahashi K, Asato Y, et al. Treatment and prognosis of angiosarcoma of the scalp and face: a retrospective analysis of 48 patients. Br J Radiol. 2012;85:E1127-E1133.
  43. Panje WR, Moran WJ, Bostwick DG, et al. Angiosarcoma of the head and neck: review of 11 cases. Laryngoscope. 1986;96:1381-1384.
  44. Perez MC, Padhya TA, Messina JL, et al. Cutaneous angiosarcoma: a single-institution experience. Ann Surg Oncol. 2013;20:3391-3397.
  45. Veness M, Cooper S. Treatment of cutaneous angiosarcomas of the head and neck. Australas Radiol. 1995;39:277-281.
  46. Barttelbort SW, Stahl R, Ariyan S. Cutaneous angiosarcoma of the face and scalp. Plast Reconstr Surg. 1989;84:55-59.
  47. Bernstein JM, Irish JC, Brown DH, et al. Survival outcomes for cutaneous angiosarcoma of the scalp versus face. Head Neck. 2017;39:1205-1211.
  48. Köhler HF, Neves RI, Brechtbühl ER, et al. Cutaneous angiosarcoma of the head and neck: report of 23 cases from a single institution. Otolaryngol Head Neck Surg. 2008;139:519-524.
  49. Morales PH, Lindberg RD, Barkley HT Jr. Soft tissue angiosarcomas. Int J Radiat Oncol Biol Phys. 1981;7:1655-1659.
  50. Wollina U, Hansel G, Schönlebe J, et al. Cutaneous angiosarcoma is a rare aggressive malignant vascular tumour of the skin. J Eur Acad Dermatol Venereol. 2011;25:964-968.
  51. Wollina U, Koch A, Hansel G, et al. A 10-year analysis of cutaneous mesenchymal tumors (sarcomas and related entities) in a skin cancer center. Int J Dermatol. 2013;52:1189-1197.
  52. Bien E, Stachowicz-Stencel T, Balcerska A, et al. Angiosarcoma in children - still uncontrollable oncological problem. The report of the Polish Paediatric Rare Tumours Study. Eur J Cancer Care (Engl). 2009;18:411-420.
  53. Suzuki G, Yamazaki H, Takenaka H, et al. Definitive radiation therapy for angiosarcoma of the face and scalp. In Vivo. 2016;30:921-926.
  54. Miki Y, Tada T, Kamo R, et al. Single institutional experience of the treatment of angiosarcoma of the face and scalp. Br J Radiol. 2013;86:20130439.
  55. Ohguri T, Imada H, Nomoto S, et al. Angiosarcoma of the scalp treated with curative radiotherapy plus recombinant interleukin-2 immunotherapy. Int J Radiat Oncol Biol Phys. 2005;61:1446-1453.
  56. Clayton BD, Leshin B, Hitchcock MG, et al. Utility of rush paraffin-embedded tangential sections in the management of cutaneous neoplasms. Dermatol Surg. 2000;26:671-678.
  57. Goldberg DJ, Kim YA. Angiosarcoma of the scalp treated with Mohs micrographic surgery. J Dermatol Surg Oncol. 1993;19:156-158.
  58. Mikhail GR, Kelly AP Jr. Malignant angioendothelioma of the face. J Dermatol Surg Oncol. 1977;3:181-183.
  59. Muscarella VA. Angiosarcoma treated by Mohs micrographic surgery. J Dermatol Surg Oncol. 1993;19:1132-1133.
  60. Bullen R, Larson PO, Landeck AE, et al. Angiosarcoma of the head and neck managed by a combination of multiple biopsies to determine tumor margin and radiation therapy. report of three cases and review of the literature. Dermatol Surg. 1998;24:1105-1110.
  61. Wiwatwongwana D, White VA, Dolman PJ. Two cases of periocular cutaneous angiosarcoma. Ophthalmic Plast Reconstr Surg. 2010;26:365-366.
  62. Morrison WH, Byers RM, Garden AS, et al. Cutaneous angiosarcoma of the head and neck. A therapeutic dilemma. Cancer. 1995;76:319-327.
  63. Hata M, Wada H, Ogino I, et al. Radiation therapy for angiosarcoma of the scalp: treatment outcomes of total scalp irradiation with X-rays and electrons. Strahlenther Onkol. 2014;190:899-904.
  64. Hwang K, Kim MY, Lee SH. Recommendations for therapeutic decisions of angiosarcoma of the scalp and face. J Craniofac Surg. 2015;26:E253-E256.
Issue
Cutis - 111(5)
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Cutis - 111(5)
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247-251,E1
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247-251,E1
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MDedge Dermatology
Cutis
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Cutis
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Nonmelanoma Skin Cancer
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Treatment of Angiosarcoma of the Head and Neck: A Systematic Review
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Treatment of Angiosarcoma of the Head and Neck: A Systematic Review
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Seger, MD, MS</bylineFull> <bylineTitleText/> <USOrGlobal/> <wireDocType/> <newsDocType/> <journalDocType/> <linkLabel/> <pageRange>247-251,E1</pageRange> <citation/> <quizID/> <indexIssueDate/> <itemClass qcode="ninat:text"/> <provider qcode="provider:"> <name/> <rightsInfo> <copyrightHolder> <name/> </copyrightHolder> <copyrightNotice/> </rightsInfo> </provider> <abstract/> <metaDescription>Cutaneous angiosarcoma (cAS) is a rare malignancy arising from vascular or lymphatic tissue. It classically presents during the sixth or seventh decades of life</metaDescription> <articlePDF>294724</articlePDF> <teaserImage/> <title>Treatment of Angiosarcoma of the Head and Neck: A Systematic Review</title> <deck/> <disclaimer/> <AuthorList/> <articleURL/> <doi/> <pubMedID/> <publishXMLStatus/> <publishXMLVersion>1</publishXMLVersion> <useEISSN>0</useEISSN> <urgency/> <pubPubdateYear>2023</pubPubdateYear> <pubPubdateMonth>May</pubPubdateMonth> <pubPubdateDay/> <pubVolume>111</pubVolume> <pubNumber>5</pubNumber> <wireChannels/> <primaryCMSID/> <CMSIDs> <CMSID>2161</CMSID> </CMSIDs> <keywords> <keyword>nonmelanoma skin cancer</keyword> <keyword> angiosarcoma</keyword> </keywords> <seeAlsos/> <publications_g> <publicationData> <publicationCode>CT</publicationCode> <pubIssueName>May 2023</pubIssueName> <pubArticleType>Original Articles | 2161</pubArticleType> <pubTopics/> <pubCategories/> <pubSections/> <journalTitle>Cutis</journalTitle> <journalFullTitle>Cutis</journalFullTitle> <copyrightStatement>Copyright 2015 Frontline Medical Communications Inc., Parsippany, NJ, USA. All rights reserved.</copyrightStatement> </publicationData> </publications_g> <publications> <term canonical="true">12</term> </publications> <sections> <term canonical="true">49</term> </sections> <topics> <term canonical="true">245</term> </topics> <links> <link> <itemClass qcode="ninat:composite"/> <altRep contenttype="application/pdf">images/18002433.pdf</altRep> <description role="drol:caption"/> <description role="drol:credit"/> </link> </links> </header> <itemSet> <newsItem> <itemMeta> <itemRole>Main</itemRole> <itemClass>text</itemClass> <title>Treatment of Angiosarcoma of the Head and Neck: A Systematic Review</title> <deck/> </itemMeta> <itemContent> <p class="abstract">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.</p> <p> <em><em>Cutis. </em>2023;111:247-251, E1.</em> </p> <p>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.<sup>1</sup> 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.<sup>2-7</sup> </p> <p>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.<sup>8</sup> 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.<sup>9<br/><br/></sup>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.</p> <h3>METHODS</h3> <p>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.<sup>5</sup> Data extracted included patient demographics, tumor characteristics (including T1 [≤5 cm] and T2 [<span class="body">&gt;</span>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.<sup>10,11</sup></p> <p>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.<br/><br/>The Newcastle-Ottawa scale for observational studies was used to assess the quality of studies.<sup>12</sup> Higher scores indicate low risk of bias, while lower scores represent high risk of bias. <br/><br/>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 <i>t</i> test with <i>P</i><span class="body">&lt;</span>.05 considered statistically significant. Due to the heterogeneity of the data, a meta-analysis was not reported. </p> <h3>RESULTSLiterature Search and Risk of Bias Assessment</h3> <p>There were 283 manuscripts identified, 56 articles read in full, and 40 articles included in the review (Figure). Among the 16 studies not meeting inclusion criteria, 7 did not provide enough data to isolate head and neck cAS cases,<sup>1,13-18</sup> 6 did not report outcomes related to the current review,<sup>19-24</sup> and 3 did not provide enough data to isolate different treatment outcomes.<sup>25-27</sup> Among the included studies, 32 reported use of WLE: WLE alone (n<span class="body">=</span>21)<sup>2,7,11,28-45</sup>; WLE with RT (n<span class="body">=</span>24)<sup>2,3,11,28-31,33-36,38-41,43-51</sup>; WLE with CT (n<span class="body">=</span>7)<sup>2,31,35,39,41,48,52</sup>; WLE with RT and CT (n<span class="body">=</span>11)<sup>2,29,31,33-35,39,40,48,52,53</sup>; WLE with RT and IT (n<span class="body">=</span>3)<sup>35,54,55</sup>; and WLE with RT, CT, and IT (n<span class="body">=</span>1).<sup>53</sup> Nine studies reported MMS: MMS alone (n<span class="body">=</span>5)<sup>39,56-59</sup>; MMS with RT (n<span class="body">=</span>3)<sup>32,50,60,61</sup>; and MMS with RT and CT (n<span class="body">=</span>1).<sup>51</sup></p> <p>Risk of bias assessment identified low risk in 3 articles. High risk was identified in 5 case reports,<sup>57-61</sup> and 1 study did not describe patient selection.<sup>43</sup> Clayton et al<sup>56</sup> showed intermediate risk, given the study controlled for 1 factor.</p> <h3>Patient Demographics</h3> <p>A total of 1295 patients were included. The pooled mean age of the patients was 67.5 years (range, 3–88 years), and 64.7% were male. There were 79 cases identified as T1 and 105 as T2. A total of 825 cases were treated using WLE with or without adjuvant therapy, while a total of 9 cases were treated using MMS with and without adjuvant therapies (Table). There were 461 cases treated without surgical excision: RT alone (n<span class="body">=</span>261), CT alone (n<span class="body">=</span>38), IT alone (n<span class="body">=</span>35), RT with CT (n<span class="body">=</span>81), RT with IT (n<span class="body">=</span>34), and RT with CT and IT (n<span class="body">=</span>12)(Table). The median follow-up period across all studies was 23.5 months (range, 1–228 months). </p> <h3>Comparison Between Surgical and Nonsurgical Modalities</h3> <p><i>Wide Local Excision—</i>Wide local excision (n<span class="body">=</span>825; 63.7%) alone or in combination with other therapies was the most frequently used treatment modality. The mean (SD) OS was longest for WLE with RT, CT, and IT (n<span class="body">=</span>3; 39.3 [24.1]), followed by WLE with RT (n<span class="body">=</span>447; 35.9 [34.3] months), WLE with CT (n<span class="body">=</span>13; 32.4 [30.2] months), WLE alone (n<span class="body">=</span>324; 29.6 [34.1] months), WLE with RT and IT (n<span class="body">=</span>11; 23.5 [4.9] months), and WLE with RT and CT (n<span class="body">=</span>27; 20.7 [13.1] months).</p> <p><i>Nonsurgical Modalities—</i>Nonsurgical methods were used less frequently than surgical methods (n<span class="body">=</span>461; 35.6%). The mean (SD) OS time in descending order was as follows: RT with CT and IT (n<span class="body">=</span>12; 34.9 [1.2] months), RT with CT (n<span class="body">=</span>81; 30.4 [37.8] months), IT alone (n<span class="body">=</span>35; 25.7 [no SD reported] months), RT with IT (n<span class="body">=</span>34; 20.5 [8.6] months), CT alone (n<span class="body">=</span>38; 20.1 [15.9] months), and RT alone (n<span class="body">=</span>261; 12.8 [8.3] months). <br/><br/>When comparing mean (SD) OS outcomes between surgical and nonsurgical treatment modalities, only the addition of WLE to RT significantly increased OS when compared with RT alone (WLE, 35.9 [34.3] months; RT alone, 12.8 [8.3] months; <i>P</i><span class="body">=</span>.001). When WLE was added to CT or both RT and CT, there was no significant difference with OS when compared with CT alone (WLE with CT, 32.4 [30.2] months; CT alone, 20.1 [15.9] months; <i>P</i><span class="body">=</span>.065); or both RT and CT in combination (WLE with RT and CT, 20.7 [13.1] months; RT and CT, 30.4 [37.8] months; <i>P</i><span class="body">=</span>.204).</p> <h3>Comparison Between T1 and T2 cAS</h3> <p><i>T1 Angiosarcoma—</i>There were 79 patients identified as having T1 tumors across 16 studies.<sup>2,31,32,34,39-41,46,48-50,53,58-60,62</sup> The mean (SD) OS was longest for WLE with RT, CT, and IT (n<span class="body">=</span>2; 56.0 [6.0] months), followed by WLE with CT (n<span class="body">=</span>4; 54.5 [41.0] months); WLE with RT (n<span class="body">=</span>30; 39.7 [41.2] months); WLE alone (n<span class="body">=</span>22; 37.2 [37.3] months); WLE with both RT and CT (n<span class="body">=</span>7; 25.5 [18.7] months); RT with IT (n<span class="body">=</span>2; 20.0 [11.0] months); RT with CT (n=6; 15.7 [6.8] months); and RT alone (n<span class="body">=</span>1; 13 [no SD]) months)(eTable).<i> </i></p> <p><i>T2 Angiosarcoma—</i>There were 105 patients with T2 tumors in 15 studies.<sup>2,31,32,34,39-41,46,48-50,52,53,57,62</sup> The mean (SD) OS for each treatment modality in descending order was as follows: RT with CT and IT (n<span class="body">=</span>1; 36 [no SD reported] months); RT with CT (n<span class="body">=</span>23; 34.3 [46.3] months); WLE with RT (n<span class="body">=</span>21; 26.3 [23.8] months); WLE with CT (n<span class="body">=</span>8; 21.5 [16.6] months); WLE alone (n<span class="body">=</span>16; 19.8 [15.6] months); WLE with RT and CT (n<span class="body">=</span>14; 19.2 [10.5] months); RT alone (n<span class="body">=</span>17; 10.1 [5.5] months); CT alone (n<span class="body">=</span>2; 6.7 [3.7] months); and WLE with RT, CT, and IT (n<span class="body">=</span>1; 6.0 [no SD] months)(eTable). <br/><br/><i>Mohs Micrographic Surgery—</i>The use of MMS was only identified in case reports or small observational studies for a total of 9 patients. Five cASs were treated with MMS alone for a mean (SD) OS of 37 (21.5) months, with 4 reporting cAS staging: 2 were T1<sup>58,59</sup> (mean [SD] OS, 37.0 [17.0] months) and 2 were T2 tumors<sup>39,57</sup> (mean [SD] OS, 44.5 [26.5] months). Mohs micrographic surgery with RT was used for 3 tumors (mean [SD] OS, 34.0 [26.9] months); 2 were T1<sup>50,60</sup> (mean [SD] OS, 42.0 [30.0] months) and 1 unreported staging (eTable).<sup>56</sup> Mohs micrographic surgery with both RT and CT was used in 1 patient (unreported staging; OS, 82 months).<sup>51</sup></p> <h3>Complications</h3> <p>Complications were rare and mainly associated with CT and RT. Four studies reported radiation dermatitis with RT.<sup>53,55,62,63</sup> Two studies reported peripheral neuropathy and myelotoxicity with CT.<sup>35,51</sup> Only 1 study reported poor wound healing due to surgical complications.<sup>29</sup> </p> <h3>COMMENT</h3> <p>Cutaneous angiosarcomas are rare and have limited treatment guidelines. Surgical excision does appear to be an effective adjunct to nonsurgical treatments, particularly WLE combined with RT, CT, and IT. Although MMS ultimately may be useful for cAS, the limited number and substantial heterogeneity of reported cases precludes definitive conclusions at this time. </p> <p>Achieving margin control during WLE is associated with higher OS when treating angiosarcoma,<sup>36,46</sup> which is particularly true for T1 tumors where margin control is imperative, and many cases are treated with a combination of WLE and RT. Overall survival times are lower for T2 tumors, as these tumors are larger and most likely have spread; therefore, more aggressive combination treatments were more prevalent. In these cases, complete margin control may be difficult to achieve and may not be as critical to the outcome if another form of adjuvant therapy can be administered promptly.<sup>24,64</sup> <br/><br/>When surgery is contraindicated, RT with or without CT was the most commonly reported treatment modality. However, these treatments were notably less effective than when used in combination with surgical resection. The use of RT alone has a recurrence rate reported up to 100% in certain studies, suggesting the need to utilize RT in combination with other modalities.<sup>23,39</sup> It is important to note that RT often is used as monotherapy in palliative treatment, which may indirectly skew survival rates.<sup>2</sup> <br/><br/>Limitations of the study include a lack of randomized controlled trials. Most reports were retrospective reviews or case series, and tumor staging was sparsely reported. Finally, although MMS may provide utility in the treatment of cAS, the sample size of 9 precluded definitive conclusions from being formed about its efficacy.</p> <h3>CONCLUSION</h3> <p>Cutaneous angiosarcoma is rare and has limited data comparing different treatment modalities. The paucity of data currently limits definitive recommendations; however, both surgical and nonsurgical modalities have demonstrated potential efficacy in the treatment of cAS and may benefit from additional research. Clinicians should consider a multidisciplinary approach for patients with a diagnosis of cAS to tailor treatments on a case-by-case basis. </p> <h2>References</h2> <p class="reference"> 1. Rodríguez-Jiménez P, Jimenez YD, Reolid A, et al. State of the art of Mohs surgery for rare cutaneous tumors in the Spanish Registry of Mohs Surgery (REGESMOHS). <i>Int J Dermatol. </i>2020;59:321-325.</p> <p class="reference"> 2. Alqumber NA, Choi JW, Kang MK. The management and prognosis of facial and scalp angiosarcoma: a retrospective analysis of 15 patients. <i>Ann Plast Surg. </i>2019;83:55-62.<br/><br/> 3. Pawlik TM, Paulino AF, McGinn CJ, et al. Cutaneous angiosarcoma of the scalp: a multidisciplinary approach. <i>Cancer. </i>2003;98:1716-1726.<br/><br/> 4. Deyrup AT, McKenney JK, Tighiouart M, et al. Sporadic cutaneous angiosarcomas: a proposal for risk stratification based on 69 cases. <i>Am J Surg Pathol. </i>2008;32:72-77.<br/><br/> 5. Meis-Kindblom JM, Kindblom LG. Angiosarcoma of soft tissue: a study of 80 cases. <i>Am J Surg Pathol. </i>1998;22:683-697.<br/><br/> 6. Harbour P, Song DH. The skin and subcutaneous tissue. In: Brunicardi FC, Andersen DK, Billiar TR, et al, eds. <i>Schwartz’s Principles of Surgery</i>.<i> </i>11th ed<i>.</i> McGraw-Hill Education; 2019. Accessed April 24, 2023. https://accesssurgery.mhmedical.com/content.aspx?bookid=2576&amp;sectionid=216206374<br/><br/> 7. Oashi K, Namikawa K, Tsutsumida A, et al. Surgery with curative intent is associated with prolonged survival in patients with cutaneous angiosarcoma of the scalp and face—a retrospective study of 38 untreated cases in the Japanese population. <i>Eur J Surg Oncol. </i>2018;44:823-829.<br/><br/> 8. Young RJ, Brown NJ, Reed MW, et al. Angiosarcoma. <i>Lancet Oncol. </i>2010;11:983-991.<br/><br/> 9. Tolkachjov SN, Brodland DG, Coldiron BM, et al. Understanding Mohs micrographic surgery: a review and practical guide for the nondermatologist. <i>Mayo Clin Proc. </i>2017;92:1261-1271.<br/><br/>10. Amin M, Edge SB, Greene FL, et al, eds. <i>AJCC Cancer Staging Manual. </i>8th ed. Springer; 2017.<br/><br/>11. Holden CA, Spittle MF, Jones EW. Angiosarcoma of the face and scalp, prognosis and treatment. <i>Cancer. </i>1987;59:1046-1057.<br/><br/>12. Cook DA, Reed DA. Appraising the quality of medical education research methods: the Medical Education Research Study Quality Instrument and the Newcastle-Ottawa Scale-Education. <i>Acad Med. </i>2015;90:1067-1076.<br/><br/>13. Lee BL, Chen CF, Chen PC, et al. Investigation of prognostic features in primary cutaneous and soft tissue angiosarcoma after surgical resection: a retrospective study. <i>Ann Plast Surg. </i>2017;78(3 suppl 2):S41-S46.</p> <p class="reference">14. Shen CJ, Parzuchowski AS, Kummerlowe MN, et al. Combined modality therapy improves overall survival for angiosarcoma. <i>Acta Oncol. </i>2017;56:1235-1238.<br/><br/>15. Breakey RW, Crowley TP, Anderson IB, et al. The surgical management of head and neck sarcoma: the Newcastle experience. <i>J Plast Reconstr Aesthet Surg. </i>2017;70:78-84.<br/><br/>16. Singla S, Papavasiliou P, Powers B, et al. Challenges in the treatment of angiosarcoma: a single institution experience. <i>Am J Surg. </i>2014;208:254-259.<br/><br/>17. Sasaki R, Soejima T, Kishi K, et al. Angiosarcoma treated with radiotherapy: impact of tumor type and size on outcome. <i>Int J Radiat Oncol Biol Phys. </i>2002;52:1032-1040.<br/><br/>18. Naka N, Ohsawa M, Tomita Y, et al. Angiosarcoma in Japan. A review of 99 cases. <i>Cancer. </i>1995;75:989-996.<br/><br/>19. DeMartelaere SL, Roberts D, Burgess MA, et al. Neoadjuvant chemotherapy-specific and overall treatment outcomes in patients with cutaneous angiosarcoma of the face with periorbital involvement. <i>Head Neck. </i>2008;30:639-646.<br/><br/>20. Ward JR, Feigenberg SJ, Mendenhall NP, et al. Radiation therapy for angiosarcoma. <i>Head Neck. </i>2003;25:873-878.<br/><br/>21. Letsa I, Benson C, Al-Muderis O, et al. Angiosarcoma of the face and scalp: effective systemic treatment in the older patient. <i>J Geriatr Oncol. </i>2014;5:276-280.<br/><br/>22. Buehler D, Rice SR, Moody JS, et al. Angiosarcoma outcomes and prognostic factors: a 25-year single institution experience. <i>Am J Clin Oncol. </i>2014;37:473-479.<br/><br/>23. Patel SH, Hayden RE, Hinni ML, et al. Angiosarcoma of the scalp and face: the Mayo Clinic experience. <i>JAMA Otolaryngol Head Neck Surg. </i>2015;141:335-340.<br/><br/>24. Guadagnolo BA, Zagars GK, Araujo D, et al. Outcomes after definitive treatment for cutaneous angiosarcoma of the face and scalp. <i>Head Neck. </i>2011;33:661-667.<br/><br/>25. Zhang Y, Yan Y, Zhu M, et al. Clinical outcomes in primary scalp angiosarcoma. <i>Oncol Lett. </i>2019;18:5091-5096.<br/><br/>26. Kamo R, Ishii M. Histological differentiation, histogenesis and prognosis of cutaneous angiosarcoma. <i>Osaka City Med J. </i>2011;57:31-44.<br/><br/>27. Ito T, Uchi H, Nakahara T, et al. Cutaneous angiosarcoma of the head and face: a single-center analysis of treatment outcomes in 43 patients in Japan. <i>J Cancer Res Clin Oncol. </i>2016;142:1387-1394.<br/><br/>28. Aust MR, Olsen KD, Lewis JE, et al. Angiosarcomas of the head and neck: clinical and pathologic characteristics. <i>Ann Otol Rhinol Laryngol. </i>1997;106:943-951.<br/><br/>29. Buschmann A, Lehnhardt M, Toman N, et al. Surgical treatment of angiosarcoma of the scalp: less is more. <i>Ann Plast Surg. </i>2008;61:399-403.<br/><br/>30. Cassidy RJ, Switchenko JM, Yushak ML, et al. The importance of surgery in scalp angiosarcomas. <i>Surg Oncol. </i>2018;27:A3-A8.<br/><br/>31. Choi JH, Ahn KC, Chang H, et al. Surgical treatment and prognosis of angiosarcoma of the scalp: a retrospective analysis of 14 patients in a single institution. <i>Biomed Res Int. </i>2015;2015:321896.<br/><br/>32. Chow TL, Kwan WW, Kwan CK. Treatment of cutaneous angiosarcoma of the scalp and face in Chinese patients: local experience at a regional hospital in Hong Kong. <i>Hong Kong Med J. </i>2018;24:25-31.<br/><br/>33. Donghi D, Kerl K, Dummer R, et al. Cutaneous angiosarcoma: own experience over 13 years. clinical features, disease course and immunohistochemical profile. <i>J Eur Acad Dermatol Venereol. </i>2010;24:1230-1234.<br/><br/>34. Ferrari A, Casanova M, Bisogno G, et al. Malignant vascular tumors in children and adolescents: a report from the Italian and German Soft Tissue Sarcoma Cooperative Group. <i>Med Pediatr Oncol. </i>2002;39:109-114.<br/><br/>35. Fujisawa Y, Nakamura Y, Kawachi Y, et al. Comparison between taxane-based chemotherapy with conventional surgery-based therapy for cutaneous angiosarcoma: a single-center experience. <i>J Dermatolog Treat. </i>2014;25:419-423.<br/><br/>36. Hodgkinson DJ, Soule EH, Woods JE. Cutaneous angiosarcoma of the head and neck. <i>Cancer. </i>1979;44:1106-1113.<br/><br/>37. Lim SY, Pyon JK, Mun GH, et al. Surgical treatment of angiosarcoma of the scalp with superficial parotidectomy. <i>Ann Plast Surg. </i>2010;64:180-182.<br/><br/>38. Maddox JC, Evans HL. Angiosarcoma of skin and soft tissue: a study of forty-four cases. <i>Cancer. </i>1981;48:1907-1921.<br/><br/>39. Mark RJ, Tran LM, Sercarz J, et al. Angiosarcoma of the head and neck. The UCLA experience 1955 through 1990. <i>Arch Otolaryngol Head Neck Surg. </i>1993;119:973-978.<br/><br/>40. Morgan MB, Swann M, Somach S, et al. Cutaneous angiosarcoma: a case series with prognostic correlation. <i>J Am Acad Dermatol. </i>2004;50:867-874.<br/><br/>41. Mullins B, Hackman T. Angiosarcoma of the head and neck. <i>Int Arch Otorhinolaryngol. </i>2015;19:191-195.<br/><br/>42. Ogawa K, Takahashi K, Asato Y, et al. Treatment and prognosis of angiosarcoma of the scalp and face: a retrospective analysis of 48 patients. <i>Br J Radiol. </i>2012;85:E1127-E1133.<br/><br/>43. Panje WR, Moran WJ, Bostwick DG, et al. Angiosarcoma of the head and neck: review of 11 cases. <i>Laryngoscope. </i>1986;96:1381-1384.<br/><br/>44. Perez MC, Padhya TA, Messina JL, et al. Cutaneous angiosarcoma: a single-institution experience. <i>Ann Surg Oncol. </i>2013;20:3391-3397.<br/><br/>45. Veness M, Cooper S. Treatment of cutaneous angiosarcomas of the head and neck. <i>Australas Radiol. </i>1995;39:277-281.</p> <p class="reference">46. Barttelbort SW, Stahl R, Ariyan S. Cutaneous angiosarcoma of the face and scalp. <i>Plast Reconstr Surg. </i>1989;84:55-59.<br/><br/>47. Bernstein JM, Irish JC, Brown DH, et al. Survival outcomes for cutaneous angiosarcoma of the scalp versus face. <i>Head Neck. </i>2017;39:1205-1211.<br/><br/>48. Köhler HF, Neves RI, Brechtbühl ER, et al. Cutaneous angiosarcoma of the head and neck: report of 23 cases from a single institution. <i>Otolaryngol Head Neck Surg. </i>2008;139:519-524.<br/><br/>49. Morales PH, Lindberg RD, Barkley HT Jr. Soft tissue angiosarcomas. <i>Int J Radiat Oncol Biol Phys. </i>1981;7:1655-1659.<br/><br/>50. Wollina U, Hansel G, Schönlebe J, et al. Cutaneous angiosarcoma is a rare aggressive malignant vascular tumour of the skin. <i>J Eur Acad Dermatol Venereol. </i>2011;25:964-968.<br/><br/>51. Wollina U, Koch A, Hansel G, et al. A 10-year analysis of cutaneous mesenchymal tumors (sarcomas and related entities) in a skin cancer center. <i>Int J Dermatol. </i>2013;52:1189-1197.<br/><br/>52. Bien E, Stachowicz-Stencel T, Balcerska A, et al. Angiosarcoma in children - still uncontrollable oncological problem. The report of the Polish Paediatric Rare Tumours Study. <i>Eur J Cancer Care (Engl). </i>2009;18:411-420.<br/><br/>53. Suzuki G, Yamazaki H, Takenaka H, et al. Definitive radiation therapy for angiosarcoma of the face and scalp. <i>In Vivo. </i>2016;30:921-926.<br/><br/>54. Miki Y, Tada T, Kamo R, et al. Single institutional experience of the treatment of angiosarcoma of the face and scalp. <i>Br J Radiol. </i>2013;86:20130439.<br/><br/>55. Ohguri T, Imada H, Nomoto S, et al. 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Recommendations for therapeutic decisions of angiosarcoma of the scalp and face. <i>J Craniofac Surg. </i>2015;26:E253-E256.</p> </itemContent> </newsItem> <newsItem> <itemMeta> <itemRole>in</itemRole> <itemClass>text</itemClass> <title/> <deck/> </itemMeta> <itemContent> <p class="insidehead">Practice <strong>Points</strong></p> <ul class="insidebody"> <li>Angiosarcoma is a rare tumor that is difficult to treat, with multiple treatment options being utilized.</li> <li>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.</li> <li>When clinicians are tasked with treating primary cutaneous angiosarcoma of the head and neck, they should consider MMS or WLE combined with RT.</li> </ul> </itemContent> </newsItem> <newsItem> <itemMeta> <itemRole>bio</itemRole> <itemClass>text</itemClass> <title/> <deck/> </itemMeta> <itemContent> <p class="disclosure">Dr. Houpe is from the University of Kansas School of Medicine, Kansas City. Drs. Seger, Neill, and Hocker are from the Division of Dermatology, University of Kansas Medical Center, Kansas City. Drs. Kang and Alam are from the Department of Dermatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois. Dr. Alam also is from the Departments of Otolaryngology and Surgery. Dr. Tolkachjov is from Epiphany Dermatology, Lewisville, Texas; the Department of Dermatology, University of Texas at Southwestern, Dallas; Baylor University Medical Center, Dallas; and the Texas A&amp;M College of Medicine, Dallas.</p> <p class="disclosure">Drs. Neill, Kang, and Hocker report no conflict of interest. Dr. Tolkachjov is on the medical advisory board for Illumisonics Inc and is a speaker and an investigator for Bioventus and Castle Biosciences. He also received a research grant from Castle Biosciences.<br/><br/>The eTable is available in the Appendix online at www.mdedge.com/dermatology.<br/><br/>Correspondence: Stanislav N. Tolkachjov, MD, Epiphany Dermatology, 1640 FM 544, Ste 100, Lewisville, TX 75056 (stan.tolkachjov@gmail.com). <br/><br/>doi:10.12788/cutis.0767</p> </itemContent> </newsItem> </itemSet></root>
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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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