Schott I, Liffers ST, Farzaliyev F, Falkenhorst J, Steinau HU, Treckmann JW, Podleska LE, Pöttgen C, Schildhaus HU, Ahrens M, Dirksen U, Murat FZ, Siveke JT, Bauer S, and Hamacher R
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., Competing Interests: LEP reports consultation for Belpharma outside of the submitted work. CP reports honoraria from Roche, Boehringer Ingelheim, and AstraZeneca outside the submitted work. JTS reports personal fees from AstraZeneca, Aurikamed, Bayer, Baxalta, BMS, Celgene, Falk Foundation, Immunocore, iOMEDICO, Lilly, Novartis, Roche, and Shire; grants and personal fees from Bristol Myers Squibb, Celgene, and Roche; has minor equity in iTheranostics and Pharma15 (<3%); and is a member of the Board of Directors for Pharma15, all outside the submitted work. SB reports personal fees from Bayer, Eli Lilly, Novartis, Pfizer, and PharmaMar; serves in an advisory/consultancy role for ADC Therapeutics, Bayer, Blueprint Medicines, Daiichi Sankyo, Deciphera, Eli Lilly, Exelixis, Janssen-Cilag, Nanobiotix, Novartis, PharmaMar, Plexxikon, and Roche; receives research funding from Novartis; and serves as a member of the External Advisory Board of the Federal Ministry of Health for “Off-label use in oncology,” all outside the submitted work. RH reports travel grants from Lilly, Novartis, and PharmaMar, as well as personal fees from Lilly outside the submitted work. All other authors declare no conflicts of interest concerning this specific publication., (Copyright © 2021 Inna Schott et al.)