Primary Angiosarcoma of Chest Wall and Cutaneous Metastasis: A Systematic Review and Pooled Analysis of Published Reports

Angiosarcomas represent a family of malignant vascular tumors arising from the endothelial lining of blood vessels. Vascular tumors arising in the chest wall are rare, and primary chest wall angiosarcomas (PACW) are even rarer. They have high propensity to metastasize to distant organs, but metastatic colonization of the skin by primary chest wall tumors has never been reported. Presented is a systematic review of the literature to identify cases of PACW reporting skin metastasis. A systematic review was conducted in accordance with PRISMA guidelines. A PubMed, Scopus, Web of Science, and manual search through references of relevant publications was used to identify all published case reports of PACW. Data extracted from each case included age, sex, symptomatology, immunohistochemistry markers, metastasis, management, follow-up, and outcome. The systematic review identified 9 publications reporting 11 cases of PACW. Mean age at presentation was 35 years (range, 8-84 years). Treatment strategies included surgical excision alone (n = 5), surgical excision and adjuvant radiotherapy and chemotherapy (n = 3), and radiotherapy and chemotherapy alone (n = 2). Death was the final outcome in 44.44% of patients. There was no report of cutaneous metastasis in any of the included cases; however, nonskin metastasis was reported in 2 cases. Based on this systematic review, there was no case found of PACW metastasizing to the skin.