TY  - JOUR
A1  - Schott, Inna
A1  - Liffers, Sven-Thorsten
A1  - Farzaliyev, Farhad
A1  - Falkenhorst, Johanna
A1  - Steinau, Hans-Ulrich
A1  - Treckmann, Jürgen Walter
A1  - Podleska, Lars Erik
A1  - Pöttgen, Christoph
A1  - Schildhaus, Hans-Ulrich
A1  - Ahrens, Marit
A1  - Dirksen, Uta
A1  - Murat, Fatma-Zehra
A1  - Siveke, Jens
A1  - Bauer, Sebastian
A1  - Hamacher, Rainer W.
T1  - Localized angiosarcoma, not one disease: a retrospective single-center study on prognosis depending on the primary site and etiology
T2  - Sarcoma
N2  - 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 ( = 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.
Y1  - 2021
UR  - http://publikationen.ub.uni-frankfurt.de/frontdoor/index/index/docId/79285
UR  - https://nbn-resolving.org/urn:nbn:de:hebis:30:3-792855
SN  - 1369-1643
VL  - 2021
IS  - 9960085
PB  - Hindawi
CY  - London
ER  -