Cardiac angiosarcoma: histopathologic, immunohistochemical, and cytogenetic analysis of 10 cases

Angiosarcoma (AS) is the most common cardiac sarcoma with differentiation, and is poorly characterized from a molecular genetic standpoint. Prognosis remains poor, owing to several factors including aggressive tumor biology, poor response to adjuvant therapy, and lack of targeted therapy. The clinical, pathologic and molecular cytogenetic features were studied in ten cardiac AS surgically resected at Mayo Clinic (1994-2015) using a whole-genome, single-nucleotide polymorphism-based platform (OncoScan). Mean patient age was 47.8 years, male/female ratio was 1:1.5, and overall median survival was 5.2 months. The most common location was the right atrium (n=7), with one case each occurring in the epicardium, pericardium, and right ventricle. No patients had received thoracic irradiation. The most common morphology was spindle cell (n=8), with one case each of epithelioid and biphasic. ERG was the most sensitive vascular marker, with diffuse immunoreactivity in all cases. Several recurrent (present in at least 3 cases) aberrations were identified, including trisomies in chromosomes 4, 8, 11, 17, 20, as well as 1q+, and homozygous deletion of CDKN2. Patients who received adjuvant therapy had longer overall survival than those who did not (median 13.4 vs 3.2 months; P=.0283). There were no significant associations between tumor location, histology, immunohistochemical findings, cytogenetic profile, and clinical outcome; however, there was a trend towards improved overall survival in patients with tumors harboring 1q+(median 31.8 vs 3.7 months, P=.06). This study confirms recurrent cytogenetic aberrations in cardiac AS, some of which may have prognostic or predictive implications.