Angiosarcoma of the visceral organs: A morphological, immunohistochemical, and C-MYC status analysis

Visceral angiosarcomas are rare malignant tumors with poor prognosis. The pathogenesis remains unclear. Herein, we describe the morphological, immunohistochemical, and C-MYC status of a series of visceral angiosarcomas. We evaluated the clinicopathologic and C-MYC status of visceral angiosarcomas (n = 12) and compared them to a control series of angiosarcomas arising in cutaneous (n = 15) and soft tissue structures (n = 15). Clinical follow-up data were obtained for all patients and exhibited high metastasis and mortality rates. Malignant endothelial cells displayed a range of morphological features including nonepithelioid, epithelioid, and mixed features, forming vasoformative (n = 6), solid (n = 4) or mixed (n = 2) architectures. Epithelioid morphology was present in 4/12 tumors. Mitoses ranged from 3 to 60 per 10 high-power fields. Necrosis was observed in 10/12 tumors. By immunohistochemistry, all angiosarcomas expressed at least 2 markers of endothelial differentiation, including CD31, CD34, vWF, ERG, and Fli-1. Eight cases of C-MYC amplification and 5 cases of C-MYC translocation were detected. Our data showed that visceral angiosarcoma is more common in women, and the clinical presentations of patient age and tumor size were significantly different between the study and control groups. No significant difference in staining between the visceral angiosarcoma and control groups was observed for endothelial markers, while different C-MYC statuses were detected.