Localized Angiosarcoma, Not One Disease: A Retrospective Single-Center Study on Prognosis Depending on the Primary Site and Etiology

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.

Prognostic nomograms for predicting overall survival and cancer-specific survival in patients with angiosarcoma, a SEER population-based study

“…Of note, they also found age ≥ 65 related to worse survival 11 . Inna Schott found the negative lymph node status corresponded to the better OS 14 . As for the histologic grade, a recent study concluded AS with grade III histology tended to experience a shorter survival which was consistent with our result 15 .…”

Primary Angiosarcoma of the Breast Diagnosed on Core Needle Biopsy: A Diagnostic Challenge

“…All breast angiosarcomas are high-grade sarcomas with five-year overall survival of 73.7% for primary angiosarcoma and 63.5% for secondary angiosarcoma. 4 The prognostic factors include tumor size, surgical margins, lymph node status, age, and etiology 19,20 In a 58 cases series, local recurrence occurred in a greater proportion of patients with margins <5 mm than those with margins ≥5 mm. 4 Mastectomy was the main surgical therapy, and breast-conserving surgery is considered if the tumor is small, allowing for excision with negative margins and a favorable cosmetic reconstruction.…”

Pediatric Lymphedema: Study of 180 Patients Referred to a Tertiary Lymphedema Clinic

“…6 No patient had angiosarcoma in our cohort, which has been described in 5 young girls, and 19 adults overall in congenital primary lymphedema 42 ; angiosarcoma remains an unusual but aggressive complication affecting adults with secondary lymphedema. 43…”

“…6 No patient had angiosarcoma in our cohort, which has been described in 5 young girls, and 19 adults overall in congenital primary lymphedema 42 ; angiosarcoma remains an unusual but aggressive complication affecting adults with secondary lymphedema. 43 Functional impairment and pain in pediatric lymphedema have been underestimated. In our cohort, about one out of four patients experienced pain, and one fifth had functional impairment, the latter close to what Watt et al described in their cohort.…”