Abstract
Angiosarcomas are aggressive tumors of vascular endothelial origin, occurring sporadically or in association with prior radiotherapy. We compared clinicopathologic and biologic features of sporadic angiosarcomas (SA) and radiation-associated angiosarcomas (RAA). Methods. From a University of Michigan institutional database, 37 SA and 11 RAA were identified. Tissue microarrays were stained for p53, Ki-67, and hTERT. DNA was evaluated for TP53 and ATM mutations. Results. Mean latency between radiotherapy and diagnosis of RAA was 11.9 years: 6.7 years for breast RAA versus 20.9 years for nonbreast RAA (P = 0.148). Survival after diagnosis did not significantly differ between SA and RAA (P = 0.590). Patients with nonbreast RAA had shorter overall survival than patients with breast RAA (P = 0.03). The majority of SA (86.5%) and RAA (77.8%) were classified as high-grade sarcomas (P = 0.609). RAA were more likely to have well-defined vasoformative areas (55.6% versus 27%, P = 0.127). Most breast SA were parenchymal in origin (80%), while most breast RAA were cutaneous in origin (80%). TMA analysis showed p53 overexpression in 25.7% of SA and 0% RAA, high Ki-67 in 35.3% of SA and 44.4% RAA, and hTERT expression in 100% of SA and RAA. TP53 mutations were detected in 13.5% of SA and 11.1% RAA. ATM mutations were not detected in either SA or RAA. Conclusions. SA and RAA are similar in histology, immunohistochemical markers, and DNA mutation profiles and share similar prognosis. Breast RAA have a shorter latency period compared to nonbreast RAA and a significantly longer survival.
Highlights
Angiosarcomas are rare, aggressive tumors of endothelial origin that account for less than 1% of all soft tissue sarcomas
Clinical and followup information were obtained by chart review and the Social Security Death Index (SSDI)
sporadic angiosarcomas (SA) patients were most commonly treated with a combination of surgery and radiotherapy, but trimodality and single modality therapy, including surgery or chemotherapy alone, were given
Summary
Angiosarcomas are rare, aggressive tumors of endothelial origin that account for less than 1% of all soft tissue sarcomas. They arise sporadically or secondary to predisposing conditions such as environmental toxins, chronic lymphedema, foreign bodies, or previous radiation therapy [1,2,3,4]. Given the association of these aggressive malignancies with radiation therapy, there is an important need to understand the biology of these secondary tumors as radiotherapy becomes increasingly utilized in cancer care. Radiation-associated angiosarcomas (RAA) were first clinically reported by Calnan and Cowdell in 1959 in a patient who developed an abdominal wall angiosarcoma six years after radiotherapy for penile
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