Prognosis of Patients with Cutaneous Angiosarcoma After Surgical Resection with Curative Intent: Is There a Difference Between the Subtypes?

• Published in: Annals of surgical oncology Vol. 30; no. 1; pp. 493 - 502
• Main Authors: Reijers, Sophie J. M., Huis in ’t Veld, Eva A., Grünhagen, Dirk J., Smith, Myles J. F., van Ginhoven, Tessa M., van Coevorden, Frits, van der Graaf, Winette T. A., Schrage, Yvonne, Strauss, Dirk C., Haas, Rick L. M., Verhoef, Cornelis J., Hayes, Andrew J., van Houdt, Winan J.
• Format: Journal Article
• Language: English
• Published: Cham Springer International Publishing 2023; Springer Nature B.V
• Subjects: Aged; Etiology; Female; Hemangiosarcoma; Humans; Lymphedema; Male; Medical prognosis; Medicine; Medicine & Public Health; Metastases; Middle Aged; Oncology; Patients; Radiation therapy; Retrospective Studies; Sarcoma; Surgery; Surgical Oncology; Tumors
• ISSN: 1068-9265; 1534-4681
• DOI: 10.1245/s10434-022-12601-1

Background The etiology of cutaneous angiosarcoma (cAS) may be idiopathic (I-cAS), or arise secondary to radiotherapy (RT-cAS), in chronic lymphedema (ST-cAS), or related to UV exposure (UV-cAS). The aim of this study was to evaluate oncological outcomes of different cAS subtypes. Patients and Methods Non-metastatic cAS patients, treated with surgery for primary disease with curative intent, were retrospectively analyzed for oncological outcome, including local recurrence (LR), distant metastases (DM), and overall survival (OS). Results A total of 234 patients were identified; 60 I-cAS, 122 RT-cAS, 9 ST-cAS, and 43 UV-cAS. The majority was female (78%), the median age was 66 years (IQR 57–76 years), the median tumor size was 4.4 cm (IQR 2.5–7.0 cm), and most common site of disease was the breast (59%). Recurrence was identified in 66% (44% LR and/or 41% DM), with a median follow up of 26.5 months (IQR 12–60 months). The 5-year OS was estimated at 50%, LRFS at 47%, and DMFS at 50%. There was no significant difference in LR, DM, or OS between the subtypes. Age < 65 years and administration of radiotherapy (RT) were significantly associated with lower LR rates (HR 0.560, 95% CI 0.3373–0.840, p = 0.005 and HR 0.421, 95% CI 0.225–0.790, p = 0.007, respectively), however no prognostic factors were identified for development of DM. Development of DM, but not LR ( p  = 0.052), was significantly associated with decreased OS (HR 6.486, 95% CI 2.939–14.318 p < 0.001). Conclusion We found no significant difference in oncological outcome between the different cAS subtypes. OS remains relatively poor, and RT is associated with lower LR rates.