Abstract
Background Angiosarcomas are rare and heterogeneous tumors with poor prognosis. The clinical subtypes are classified depending on the primary site and etiology. Methods We conducted a retrospective, monocentric study of 136 patients with localized AS between May 1985 and November 2018. Overall survival (OS), local recurrence-free survival (LRFS), and metastasis-free survival (MFS) were estimated using the Kaplan–Meier method. To identify prognostic factors, univariate and multivariate analyses were performed based on Cox regressions. Results The median age was 67 years (19–72.8 years). Primary sites were cutaneous (27.2%), breast (38.2%), and deep soft tissue (34.6%). The majority was primary angiosarcomas (55.9%) followed by postradiation (40.4%) and chronic lymphedema angiosarcomas (2.9%). Prognosis significantly differed depending on the primary site and etiology. Shortest median OS and MFS were observed in deep soft tissue angiosarcomas, whereas cutaneous angiosarcomas, angiosarcomas of the breast, and radiation-associated angiosarcomas displayed worse median LRFS. Univariate analyses showed better OS for tumor size <10 cm (p = 0.009), negative surgical margins (p = 0.021), and negative lymph node status (p = 0.007). LRFS and MFS were longer for tumor size <10 cm (p = 0.012 and p = 0.013). In multivariate analyses, age <70 years was the only independent positive prognostic factor for OS in all subgroups. For LRFS, secondary AS of the breast was a negative prognostic factor (HR: 2.35; p = 0.035). Conclusions Different behaviors and prognoses depending on the primary site and etiology should be considered for the treatment of this heterogeneous disease. In cutaneous angiosarcomas of the head/neck and postradiation angiosarcomas of the breast, local recurrence seems to have a crucial impact on OS. Therefore, improved local therapies and local tumor staging may have to be implemented. However, in deep soft tissue angiosarcomas, distant recurrence seems to have a major influence on prognosis, which indicates a benefit of additional perioperative chemotherapy.
Highlights
Angiosarcomas are very rare and malignant tumors that share markers of differentiation with endothelial cells of blood or lymphatic vessels [1]. ese tumors account for only 2–5% of soft tissue sarcomas with an incidence of only 1.5 to 2.8 per million per year [1,2,3,4,5]
Depending on the anatomic location and etiology, they are typically subdivided into cutaneous angiosarcomas, mainly on the head and neck, radiation-induced angiosarcomas, mainly of the breast, lymphedema-associated angiosarcomas, primary breast angiosarcomas, and soft tissue angiosarcomas [1]. e prognosis of angiosarcomas is generally poor with median overall survival reported between 30 and 50 months and 5year overall survival rates between 10% and 50%, while localized angiosarcomas range around 40%, and metastasized disease drops to around 15% [1, 5,6,7,8]. e impact of the type of angiosarcoma on prognosis is not well studied, and generic staging classifications appear to have limited value in angiosarcomas
The distribution of angiosarcoma subtypes is comparable to other studies, a high variability can be observed between studies: cutaneous angiosarcomas (13–75%), angiosarcomas of the breast (16–56%), deep soft tissue angiosarcomas (5–42%), and primary (46–71%), radiation-associated (17–63%), and lymphedema-associated (3–22%) angiosarcomas [5, 6, 8, 17,18,19,20,21]. e overall survival for localized angiosarcomas varies in other studies with a median time from 20.8 to >60 months and 5-year survival rates from 17.9% to 74.8%, whereas our cohort ranges somehow in the middle [5, 6, 8, 17,18,19, 21]
Summary
Angiosarcomas are very rare and malignant tumors that share markers of differentiation with endothelial cells of blood or lymphatic vessels [1]. ese tumors account for only 2–5% of soft tissue sarcomas with an incidence of only 1.5 to 2.8 per million per year [1,2,3,4,5]. Depending on the anatomic location and etiology, they are typically subdivided into cutaneous angiosarcomas, mainly on the head and neck, radiation-induced angiosarcomas, mainly of the breast, lymphedema-associated angiosarcomas, primary breast angiosarcomas, and soft tissue angiosarcomas [1]. Primary sites were cutaneous (27.2%), breast (38.2%), and deep soft tissue (34.6%). Shortest median OS and MFS were observed in deep soft tissue angiosarcomas, whereas cutaneous angiosarcomas, angiosarcomas of the breast, and radiation-associated angiosarcomas displayed worse median LRFS. Different behaviors and prognoses depending on the primary site and etiology should be considered for the treatment of this heterogeneous disease. In cutaneous angiosarcomas of the head/neck and postradiation angiosarcomas of the breast, local recurrence seems to have a crucial impact on OS
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
