Skip to main content

Advertisement

Log in

Management of Cutaneous Angiosarcoma: an Update Review

  • Skin Cancer (T Ito, Section Editor)
  • Published:
Current Treatment Options in Oncology Aims and scope Submit manuscript

Opinion statement

Cutaneous angiosarcoma is a rare and invasive malignant tumor. For localized cAS patients, wide-margin excision was recommended. Due to the latent local invasion characteristic of cAS, we suggest preoperative and postoperative radiotherapy to nearly all patients. Recently, there is growing interest in using neoadjuvant chemotherapy and/or radiotherapy as part of a combination therapy regimen, which may allow some patients to undergo potentially less disabling surgery. For metastatic cAS patients with unresectable tumors and who refuse surgery, radical radiotherapy or chemoradiotherapy may be an option. Paclitaxel was recognized as the first-line treatment. For tumors resistant to taxanes, emerging medications such as targeted agents and immunotherapy are also under investigation.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References and Recommended Reading

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. Cioffi A, Reichert S, Antonescu CR, Maki RG. Angiosarcomas and other sarcomas of endothelial origin. Hematol Oncol Clin. 2013;27(5):975–88.

    Article  Google Scholar 

  2. Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ. Angiosarcoma. Lancet Oncol. 2010;11(10):983–91.

    Article  PubMed  Google Scholar 

  3. Lee KC, Chuang S-K, Philipone EM, Peters SM. Characteristics and prognosis of primary head and neck angiosarcomas: a surveillance, epidemiology, and end results program (SEER) analysis of 1250 cases. Head Neck Pathol. 2019;13(3):378–85.

    Article  PubMed  Google Scholar 

  4. Albores-Saavedra J, Schwartz AM, Henson DE, Kostun L, Hart A, Angeles-Albores D, Chablé-Montero F. Cutaneous angiosarcoma. Analysis of 434 cases from the surveillance, epidemiology, and end results program, 1973-2007. Ann Diagn Pathol. 2011;15(2):93–7.

    Article  PubMed  Google Scholar 

  5. Fujisawa Y, Yoshino K, Fujimura T, Nakamura Y, Okiyama N, Ishitsuka Y, Watanabe R, Fujimoto M. Cutaneous angiosarcoma: the possibility of new treatment options especially for patients with large primary tumor. Front Oncol. 2018;8:46.

    Article  PubMed  PubMed Central  Google Scholar 

  6. Weidema ME, Versleijen-Jonkers YMH, Flucke UE, Desar IME, van der Graaf WTA. Targeting angiosarcomas of the soft tissues: a challenging effort in a heterogeneous and rare disease. Crit Rev Oncol Hematol. 2019;138:120–31.

    Article  CAS  PubMed  Google Scholar 

  7. Merfeld E, Gabani P, Spraker MB, Zoberi I, Kim H, Van Tine B, et al. Clinical outcomes and prognostic features of angiosarcoma: significance of prior radiation therapy. Clin Oncol. 2019;31(4):232–41.

    Article  CAS  Google Scholar 

  8. Florou V, Wilky BA. Current management of angiosarcoma: recent advances and lessons from the past. Curr Treat Options Oncol. 2021;22(7):1–10.

    Article  Google Scholar 

  9. Ishida Y, Otsuka A, Kabashima K. Cutaneous angiosarcoma: update on biology and latest treatment. Curr Opin Oncol. 2018;30(2):107–12.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  10. Foa R, Norton L, Seidman AD. Taxol (paclitaxel): a novel anti-microtubule agent with remarkable anti-neoplastic activity. Int J Clin Lab Res. 1994;24(1):6–14.

    Article  CAS  PubMed  Google Scholar 

  11. Penel N, Bui BN, Bay J-O, Cupissol D, Ray-Coquard I, Piperno-Neumann S, Kerbrat P, Fournier C, Taieb S, Jimenez M, Isambert N, Peyrade F, Chevreau C, Bompas E, Brain EGC, Blay JY. Phase II trial of weekly paclitaxel for unresectable angiosarcoma: the ANGIOTAX Study. J Clin Oncol. 2008;26(32):5269–74.

    Article  CAS  PubMed  Google Scholar 

  12. Roy A, Gabani P, Davis EJ, Oppelt P, Merfeld E, Keedy VL, Zoberi I, Chrisinger JSA, Michalski JM, van Tine B, Spraker MB. Concurrent paclitaxel and radiation therapy for the treatment of cutaneous angiosarcoma. Clin Transl Radiat Oncol. 2021;27:114–20.

    Article  PubMed  PubMed Central  Google Scholar 

  13. Park C, Kim M, Kwak Y, Moon KC, Kim SH, Keam B, et al. Real-world clinical outcomes and prognostic factors for patients with advanced angiosarcoma who received systemic treatment. Cancer Res Treat. 2021.

  14. Ravi V, Wagner M, Chen TW-W, Loong HHF, Mennel RG, Yen C-C, et al. A phase II study of oraxol in the treatment of unresectable cutaneous angiosarcoma. 2020.

  15. Saito Y, Yokoyama R, Umemori Y. Cutaneous angiosarcoma: efficacy and treatment regimen of paclitaxel maintenance therapy. Dermatol Ther. 2020:e13563-e.

  16. Nagano T, Yamada Y, Ikeda T, Kanki H, Kamo T, Nishigori C. Docetaxel: a therapeutic option in the treatment of cutaneous angiosarcoma: report of 9 patients. Cancer. 2007;110(3):648–51.

    Article  CAS  PubMed  Google Scholar 

  17. Kajihara I, Kanemaru H, Miyake T, Aoi J, Masuguchi S, Fukushima S, Jinnin M, Ihn H. Combination chemotherapy with S-1 and docetaxel for cutaneous angiosarcoma resistant to paclitaxel. Drug Discov Ther. 2015;9(1):75–7.

    Article  CAS  PubMed  Google Scholar 

  18. Fujisawa Y, Nakamura Y, Kawachi Y, Otsuka F. Comparison between taxane-based chemotherapy with conventional surgery-based therapy for cutaneous angiosarcoma: a single-center experience. J Dermatolog Treat. 2014;25(5):419–23.

    Article  CAS  PubMed  Google Scholar 

  19. Pinedo HM, Kenis Y. Chemotherapy of advanced soft-tissue sarcomas in adults. Cancer Treat Rev. 1977;4(2):67–86.

    Article  CAS  PubMed  Google Scholar 

  20. Bramwell VHC, Anderson D, Charette ML. Doxorubicin-based chemotherapy for the palliative treatment of adult patients with locally advanced or metastatic soft-tissue sarcoma: a meta-analysis and clinical practice guideline. Sarcoma. 2000;4(3):103–12.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  21. Judson I, Radford JA, Harris M, Blay JY, van Hoesel Q, Le Cesne A, et al. Randomised phase II trial of pegylated liposomal doxorubicin (DOXIL®/CAELYX®) versus doxorubicin in the treatment of advanced or metastatic soft tissue sarcoma: a study by the EORTC Soft Tissue and Bone Sarcoma Group. Eur J Cancer. 2001;37(7):870–7.

    Article  CAS  PubMed  Google Scholar 

  22. Bönisch N, Langan EA, Terheyden P. Cutaneous angiosarcoma: Radiochemotherapy with liposomal pegylated doxorubicin. Hautarzt. 2019;70(9):700–6.

    Article  PubMed  Google Scholar 

  23. Wollina U, Koch A, Hansel G, Schönlebe J, Lotti T, Vojvodic A. Cutaneous angiosarcoma of head and neck–a single-centre analysis. Open Access Maced J Med Sci. 2019;7(18):2976–8.

    Article  PubMed  PubMed Central  Google Scholar 

  24. Wollina U, Hansel G, Schönlebe J, Averbeck M, Paasch U, Uhl J, Hindemann W, Simon JC. Cutaneous angiosarcoma is a rare aggressive malignant vascular tumour of the skin. J Eur Acad Dermatol Venereol. 2011;25(8):964–8.

    Article  CAS  PubMed  Google Scholar 

  25. Penel N, Italiano A, Ray-Coquard I, Chaigneau L, Delcambre C, Robin YM, Bui B, Bertucci F, Isambert N, Cupissol D, Bompas E, Bay JO, Duffaud F, Guillemet C, Blay JY, French Sarcoma Group (GSF/GETO). Metastatic angiosarcomas: doxorubicin-based regimens, weekly paclitaxel and metastasectomy significantly improve the outcome. Ann Oncol. 2012;23(2):517-523.

  26. Italiano A, Cioffi A, Penel N, Levra MG, Delcambre C, Kalbacher E, Chevreau C, Bertucci F, Isambert N, Blay JY, Bui B, Antonescu C, D'Adamo DR, Maki RG, Keohan ML. Comparison of doxorubicin and weekly paclitaxel efficacy in metastatic angiosarcomas. Cancer. 2012;118(13):3330–6.

    Article  CAS  PubMed  Google Scholar 

  27. Schöffski P, Timmermans I, Wildiers H, Dumez H, Hompes D, Christiaens M, Sciot R, Laenen A, Lee CJ, Meyskens T. Retrospective analysis of the clinical presentation, treatment and outcome of angiosarcoma in a sarcoma referral center. Oncol Res Treat. 2021;44(6):322–32.

    Article  PubMed  Google Scholar 

  28. Jordan MA, Kamath K, Manna T, Okouneva T, Miller HP, Davis C, Littlefield BA, Wilson L. The primary antimitotic mechanism of action of the synthetic halichondrin E7389 is suppression of microtubule growth. Mol Cancer Ther. 2005;4(7):1086–95.

    Article  CAS  PubMed  Google Scholar 

  29. • Fujisawa Y, Fujimura T, Matsushita S, Yamamoto Y, Uchi H, Otsuka A, et al. The efficacy of eribulin mesylate for patients with cutaneous angiosarcoma previously treated with taxane: a multicentre prospective observational study. Br J Dermatol. 2020;183(5):831–9 First multicenter, single-arm prospective observational study using eribulin mesylate with advanced cAS patients previously treated with taxane.

    Article  CAS  PubMed  Google Scholar 

  30. Smrke A, Benson C. Eribulin for recurrent cutaneous angiosarcoma. Br J Dermatol. 2020;183(5):797–8.

    Article  CAS  PubMed  Google Scholar 

  31. Shkoukani MA, Carron MA, Tulunay O, Kucuk O, Lin H-S. Angiosarcoma of the scalp with complete response to a biweekly gemcitabine and docetaxel (GEMDOC) chemotherapy regimen. Ear Nose Throat J. 2011;90(1):E26–E9.

    Article  PubMed  Google Scholar 

  32. Kajihara I, Maeda S, Yamada S, Izumi K, Masuguchi S, Fukushima S, Jinnin M, Ihn H. Biweekly gemcitabine therapy induces complete remission in cutaneous angiosarcoma resistant to multiple anticancer drugs. J Dermatol. 2015;42(12):1197–8.

    Article  PubMed  Google Scholar 

  33. Stacchiotti S, Palassini E, Sanfilippo R, Vincenzi B, Arena MG, Bochicchio AM, de Rosa P, Nuzzo A, Turano S, Morosi C, Dei Tos AP, Pilotti S, Casali PG. Gemcitabine in advanced angiosarcoma: a retrospective case series analysis from the Italian Rare Cancer Network. Ann Oncol. 2012;23(2):501–8.

    Article  CAS  PubMed  Google Scholar 

  34. Tian Z, Zhang F, Li P, Wang J, Yang J, Zhang P, Yao W, Wang X. Albumin-bound paclitaxel and gemcitabine combination therapy in soft tissue sarcoma. BMC Cancer. 2020;20(1):1–6.

    Article  Google Scholar 

  35. Le Cesne A, Ray-Coquard I, Duffaud F, Chevreau C, Penel N, Nguyen BB, et al. Trabectedin in patients with advanced soft tissue sarcoma: a retrospective national analysis of the French Sarcoma Group. Eur J Cancer. 2015;51(6):742–50.

    Article  PubMed  Google Scholar 

  36. Demetri GD, von Mehren M, Jones RL, Hensley ML, Schuetze SM, Staddon A, Milhem M, Elias A, Ganjoo K, Tawbi H, van Tine BA, Spira A, Dean A, Khokhar NZ, Park YC, Knoblauch RE, Parekh TV, Maki RG, Patel SR. Efficacy and safety of trabectedin or dacarbazine for metastatic liposarcoma or leiomyosarcoma after failure of conventional chemotherapy: results of a phase III randomized multicenter clinical trial. J Clin Oncol. 2016;34(8):786–93.

    Article  CAS  PubMed  Google Scholar 

  37. Léauté-Labrèze C, De La Roque ED, Hubiche T, Boralevi F, Thambo J-B, Taïeb A. Propranolol for severe hemangiomas of infancy. N Engl J Med. 2008;358(24):2649–51.

    Article  PubMed  Google Scholar 

  38. Chow W, Amaya CN, Rains S, Chow M, Dickerson EB, Bryan BA. Growth attenuation of cutaneous angiosarcoma with propranolol-mediated β-blockade. JAMA Dermatol. 2015;151(11):1226–9.

    Article  PubMed  Google Scholar 

  39. Pasquier E, André N, Street J, Chougule A, Rekhi B, Ghosh J, Philip DSJ, Meurer M, MacKenzie KL, Kavallaris M, Banavali SD. Effective management of advanced angiosarcoma by the synergistic combination of propranolol and vinblastine-based metronomic chemotherapy: a bench to bedside study. EBioMedicine. 2016;6:87–95.

    Article  PubMed  PubMed Central  Google Scholar 

  40. Heinhuis KM, Ijzerman NS, Koenen AM, van der Graaf WTA, Haas RL, Beijnen JH, et al. Protocol: PropAngio study protocol: a neoadjuvant trial on the efficacy of propranolol monotherapy in cutaneous angiosarcoma—a proof of principle study. BMJ Open. 2020;10(9).

  41. Florou V, Andrea E, Neha G, Andrew R, Breelyn W, Jonathan T, et al. Safety and efficacy of immune checkpoint inhibitors in patients with angiosarcoma. 2019 CTOS Annual Meeting. 2019. Tokyo, Japan.

  42. Florou V, Rosenberg AE, Wieder E, Komanduri KV, Kolonias D, Uduman M, et al. Angiosarcoma patients treated with immune checkpoint inhibitors: a case series of seven patients from a single institution. J Immunother Cancer. 2019;7(1):1–8.

    Google Scholar 

  43. • Fujisawa Y, Yoshino K, Isei T, Namikawa K, Fujimura T, Yasuda M, et al. A multicenter, open-label, uncontrolled phase II study of ONO-4538 for cutaneous angiosarcoma (Angio Check study). 2021. Multicenter, open-label, uncontrolled phase II study which investigates Nivolumab for patients with unresectable or metastatic cAS refractory to first-line paclitaxel.

  44. Nguyen DT, Shayahi S. Pazopanib: approval for soft-tissue sarcoma. Journal of the advanced practitioner in oncology. 2013;4(1):53–7.

    PubMed  PubMed Central  Google Scholar 

  45. Jones RL, Ravi V, Brohl AS, Chawla SP, Ganjoo K, Italiano A, Attia S, Burgess M, Thornton K, Cranmer L, Liu L, Theuer C, Maki R. Results of the TAPPAS trial: an adaptive enrichment phase III trial of TRC105 and pazopanib (P) versus pazopanib alone in patients with advanced angiosarcoma (AS). Ann Oncol. 2019;30:v683.

    Article  Google Scholar 

  46. Kollár A, Jones RL, Stacchiotti S, Gelderblom H, Guida M, Grignani G, Steeghs N, Safwat A, Katz D, Duffaud F, Sleijfer S, van der Graaf WT, Touati N, Litière S, Marreaud S, Gronchi A, Kasper B. Pazopanib in advanced vascular sarcomas: an EORTC Soft Tissue and Bone Sarcoma Group (STBSG) retrospective analysis. Acta Oncol. 2017;56(1):88–92.

    Article  PubMed  Google Scholar 

  47. von Mehren M, Litwin S, Ravi V, Schuetze S, Movva S, Agulnik M, et al. Multicenter phase II trial of pazopanib (P) in patients with angiosarcoma (AS). In: Multicenter phase II trial of pazopanib (P) in patients with angiosarcoma (AS); 2019.

    Chapter  Google Scholar 

  48. Oashi K, Shibata T, Namikawa K, Takahashi A, Yokota K, Nakano E, Teramoto Y, Tsutsumida A, Maeda T, Yamazaki N, the Dermatologic Oncology Group of the Japan Clinical Oncology Group. A single-arm confirmatory trial of pazopanib in patients with paclitaxel-pretreated primary cutaneous angiosarcoma: Japan Clinical Oncology Group study (JCOG1605, JCOG-PCAS protocol). BMC Cancer. 2020;20(1):1–6.

    Article  Google Scholar 

  49. • Pink D, Andreou D, Bauer S, Brodowicz T, Kasper B, Reichardt P, et al. Treatment of angiosarcoma with pazopanib and paclitaxel: results of the EVA (Evaluation of Votrient® in Angiosarcoma) Phase II Trial of the German Interdisciplinary Sarcoma Group (GISG-06). Cancers (Basel). 2021;13(6):1223 Multicenter, open-label, prospective, single-arm phase II trial with the combination of pazopanib and paclitaxel.

    Article  CAS  Google Scholar 

  50. Agulnik M, Schulte B, Robinson S, Hirbe AC, Kozak K, Chawla SP, Attia S, Rademaker A, Zhang H, Abbinanti S, Cehic R, Monga V, Milhem M, Okuno S, van Tine BA. An open-label single-arm phase II study of regorafenib for the treatment of angiosarcoma. Eur J Cancer. 2021;154:201–8.

    Article  CAS  PubMed  Google Scholar 

  51. Agulnik M, Robinson SI, Okuno SH, Siontis B, Attia S, Kocherginsky M, et al. Multicenter, open-label phase II study of daily oral regorafenib for chemotherapy-refractory, metastatic and locally advanced angiosarcoma. 2020.

    Book  Google Scholar 

  52. Donghi D, Dummer R, Cozzio A. Complete remission in a patient with multifocal metastatic cutaneous angiosarcoma with a combination of paclitaxel and sorafenib. Br J Dermatol. 2010;162(3):697–9.

    Article  CAS  PubMed  Google Scholar 

  53. Ono S, Tanioka M, Fujisawa A, Tanizaki H, Miyachi Y, Matsumura Y. Angiosarcoma of the scalp successfully treated with a single therapy of sorafenib. Arch Dermatol. 2012;148(6):683–5.

    Article  CAS  PubMed  Google Scholar 

  54. Silva E, Gatalica Z, Vranic S, Basu G, Reddy SK, Voss A. Refractory angiosarcoma of the breast with VEGFR2 upregulation successfully treated with sunitinib. Breast J. 2015;21(2):205–7.

    Article  CAS  PubMed  Google Scholar 

  55. Lu HJ, Chen PH, Yen CC, Hsiao FC, Tzeng CH, Ma H, et al. Refractory cutaneous angiosarcoma successfully treated with sunitinib. Br J Dermatol. 2013;169(1):204–6.

    Article  CAS  PubMed  Google Scholar 

  56. Ray-Coquard I, Italiano A, Bompas E, Le Cesne A, Robin Y-M, Chevreau C, et al. Sorafenib for patients with advanced angiosarcoma: a phase II Trial from the French Sarcoma Group (GSF/GETO). Oncologist. 2012;17(2):260–6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  57. Casali PG. Histology-and non-histology-driven therapy for treatment of soft tissue sarcomas. Ann Oncol. 2012;23:x167–x9.

    Article  PubMed  Google Scholar 

  58. Penel N, Ray-Coquard I, Bal-Mahieu C, Chevreau C, Le Cesne A, Italiano A, et al. Low level of baseline circulating VEGF-A is associated with better outcome in patients with vascular sarcomas receiving sorafenib: an ancillary study from a phase II trial. Target Oncol. 2014;9(3):273–7.

    Article  PubMed  Google Scholar 

  59. Tamiya H, Kamo R, Kumei A, Yanagihara S, Ishii M, Kobayashi H. Partial response of angiosarcoma of the scalp to sorafenib: association with decreased expression of vascular endothelial growth factors and their receptors. Clin Exp Dermatol: Clin Dermatol. 2012;37(7):749–52.

    Article  CAS  Google Scholar 

  60. Sleijfer S. Phase II studies in soft tissue sarcoma: time for reappraisal. Oncologist. 2012;17(2):154–6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  61. Agulnik M, Yarber JL, Okuno SH, Von Mehren M, Jovanovic BD, Brockstein BE, et al. An open-label, multicenter, phase II study of bevacizumab for the treatment of angiosarcoma and epithelioid hemangioendotheliomas. Ann Oncol. 2013;24(1):257–63.

    Article  CAS  PubMed  Google Scholar 

  62. Ray-Coquard IL, Domont J, Tresch-Bruneel E, Bompas E, Cassier PA, Mir O, Piperno-Neumann S, Italiano A, Chevreau C, Cupissol D, Bertucci F, Bay JO, Collard O, Saada-Bouzid E, Isambert N, Delcambre C, Clisant S, le Cesne A, Blay JY, Penel N. Paclitaxel given once per week with or without bevacizumab in patients with advanced angiosarcoma: a randomized phase II trial. J Clin Oncol. 2015;33(25):2797–802.

    Article  CAS  PubMed  Google Scholar 

  63. Lebellec L, Bertucci F, Tresch-Bruneel E, Ray-Coquard I, Le Cesne A, Bompas E, et al. Prognostic and predictive factors for angiosarcoma patients receiving paclitaxel once weekly plus or minus bevacizumab: an ancillary study derived from a randomized clinical trial. BMC Cancer. 2018;18(1):1–10.

    Article  Google Scholar 

  64. Bui N, Kamat N, Ravi V, Chawla S, Lohman M, Ganjoo KN. A multicenter phase II study of Q3 week or weekly paclitaxel in combination with bevacizumab for the treatment of metastatic or unresectable angiosarcoma. Rare Tumors. 2018;10:2036361318771771.

    Article  PubMed  PubMed Central  Google Scholar 

  65. Oxenberg J, Khushalani NI, Salerno KE, Attwood K, Kane Iii JM. Neoadjuvant chemotherapy for primary cutaneous/soft tissue angiosarcoma: determining tumor behavior prior to surgical resection. J Surg Oncol. 2015;111(7):829–33.

    Article  PubMed  Google Scholar 

  66. Heinhuis KM, Ijzerman NS, van der Graaf WTA, Kerst JM, Schrage Y, Beijnen JH, et al. Neoadjuvant systemic treatment of primary angiosarcoma. Cancers (Basel). 2020;12(8):2251.

    Article  CAS  PubMed Central  Google Scholar 

  67. Constantinidou A, Sauve N, Stacchiotti S, Blay J-Y, Vincenzi B, Grignani G, Rutkowski P, Guida M, Hindi N, Klein A, Thibaud V, Sufliarsky J, Desar I, Steeghs N, Litiere S, Gelderblom H, Jones RL. Evaluation of the use and efficacy of (neo) adjuvant chemotherapy in angiosarcoma: a multicentre study. ESMO open. 2020;5(4):e000787.

    Article  PubMed  PubMed Central  Google Scholar 

  68. Wagner MJ, Cranmer LD, Loggers ET, Pollack SM. Propranolol for the treatment of vascular sarcomas. J Exp Pharmacol. 2018;10:51–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  69. Vogt T, Hafner C, Bross K, Bataille F, Jauch KW, Berand A, Landthaler M, Andreesen R, Reichle A. Antiangiogenetic therapy with pioglitazone, rofecoxib, and metronomic trofosfamide in patients with advanced malignant vascular tumors. Cancer. 2003;98(10):2251–6.

    Article  CAS  PubMed  Google Scholar 

  70. Fujisawa Y, Yoshino K, Kadono T, Miyagawa T, Nakamura Y, Fujimoto M. Chemoradiotherapy with taxane is superior to conventional surgery and radiotherapy in the management of cutaneous angiosarcoma: a multicentre, retrospective study. Br J Dermatol. 2014;171(6):1493–500.

    Article  CAS  PubMed  Google Scholar 

  71. Ihara H, Kaji T, Katsui K, Miyake T, Waki T, Katayama N, Matsuzaki H, Yamasaki O, Kuroda M, Morizane S, Kanazawa S. Single institutional experience of radiation therapy for angiosarcoma of the scalp without cervical lymph node metastases: impact of concurrent chemoradiation with maintenance chemotherapy using taxanes on patient prognosis. Mol Clin Oncol. 2019;11(5):498–504.

    CAS  PubMed  PubMed Central  Google Scholar 

  72. Ito T, Uchi H, Nakahara T, Tsuji G, Oda Y, Hagihara A, Furue M. 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(6):1387–94.

    Article  CAS  PubMed  Google Scholar 

  73. Bi S, Chen S, Wu B, Cen Y, Chen J. The effectiveness of different treatment modalities of cutaneous angiosarcoma: results from meta-analysis and observational data from SEER database. Front Oncol. 2021;11.

  74. Shin JY, Roh SG, Lee NH, Yang KM. Predisposing factors for poor prognosis of angiosarcoma of the scalp and face: systematic review and meta-analysis. Head Neck. 2017;39(2):380–6.

    Article  PubMed  Google Scholar 

  75. Trofymenko O, Curiel-Lewandrowski C. Surgical treatment associated with improved survival in patients with cutaneous angiosarcoma. Journal of the European Academy of Dermatology and Venereology: JEADV. 2017;32(1):e29–31.

    Article  PubMed  Google Scholar 

  76. Florou V, Wilky BA. Current and future directions for angiosarcoma therapy. Curr Treat Options Oncol. 2018;19(3):1–13.

    Article  Google Scholar 

  77. Sinnamon AJ, Neuwirth MG, McMillan MT, Ecker BL, Bartlett EK, Zhang PJ, et al. A prognostic model for resectable soft tissue and cutaneous angiosarcoma. J Surg Oncol. 2016;114(5):557–63.

    Article  PubMed  Google Scholar 

  78. • Katano A, Yamashita H, Nakagawa K. Radical radiotherapy for localized cutaneous angiosarcoma of the scalp. Mol Clin Oncol. 2021;15(3):1–5 First retrospective cohort study investigating efficacy of RT for radical radiotherapy without surgery.

    Article  Google Scholar 

  79. Hata M, Wada H, Ogino I, Omura M, Koike I, Tayama Y, Odagiri K, Kasuya T, Inoue T. Radiation therapy for angiosarcoma of the scalp. Strahlenther Onkol. 2014;190(10):899–904.

    Article  PubMed  Google Scholar 

  80. Ogawa K, Takahashi K, Asato Y, Yamamoto Y, Taira K, Matori S, Iraha S, Yagi N, Yogi A, Haranaga S, Fujita J, Uezato H, Murayama S. Treatment and prognosis of angiosarcoma of the scalp and face: a retrospective analysis of 48 patients. The British journal of radiology. 2012;85(1019):e1127–e33.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  81. Guadagnolo BA, Zagars GK, Araujo D, Ravi V, Shellenberger TD, Sturgis EM. Outcomes after definitive treatment for cutaneous angiosarcoma of the face and scalp. Head Neck. 2011;33(5):661–7.

    Article  PubMed  Google Scholar 

  82. Roy A, Gabani P, Davis EJ, Oppelt P, Merfeld E, Keedy VL, et al. Concurrent paclitaxel and radiation therapy for the treatment of cutaneous angiosarcoma. Clin Transl Radiat Oncol. 2021;27:114–20.

    Article  PubMed  PubMed Central  Google Scholar 

  83. Watanabe M, Nakai K, Iwaki Y, Ozawa T, Kamo R, Tsuruta D. Successful long-term treatment with pazopanib after prior interleukin-2 therapy in patients with metastatic cutaneous angiosarcoma of the scalp. Dermatol Ther. 2020;33(6):e14007.

    Article  CAS  PubMed  Google Scholar 

Download references

Availability of Data and Material

Not applicable.

Code Availability

Not applicable.

Funding

This study was supported by Sichuan Science and Technology Program 2020YFS0267.

Author information

Authors and Affiliations

Authors

Contributions

All authors contributed to the study conception. Literature search and data analysis were performed by Siwei Bi, Ai Zhong, Xiya Yin, and Jingyi Li. The first draft of the manuscript was written by Siwei Bi, and all authors commented on previous versions of the manuscript. Ying Cen and Junjie Chen critically revised the work. All authors read and approved the final manuscript.

Corresponding authors

Correspondence to Ying Cen or Junjie Chen.

Ethics declarations

Ethics Approval

Not applicable.

Consent to Participate

Not applicable.

Consent for publication

Not applicable.

Human and Animal Rights

This article does not contain any studies with human or animal subjects performed by any of the authors.

Conflict of Interest

The authors declare no competing interests.

Additional information

Publisher’s note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

This article is part of the Topical Collection on Skin Cancer

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Bi, S., Zhong, A., Yin, X. et al. Management of Cutaneous Angiosarcoma: an Update Review. Curr. Treat. Options in Oncol. 23, 137–154 (2022). https://doi.org/10.1007/s11864-021-00933-1

Download citation

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11864-021-00933-1

Keywords

Navigation