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Introduction: Angiosarcomas are rare mesenchymal tumors showing endothelial differentiation and comprise less than 1% of all sarcomas. They show variable degrees of vascular formation and range from well-differentiated tumors resembling hemangioma to highly anaplastic solid tumors that may be mistaken for poorly-differentiated carcinomas or sarcomas. They are no longer separated into hemangiosarcomas and lymphangiosarcomas. Angiosarcomas are highly aggressive tumors with a poor prognosis.
Locations: Tumors arising in skin and superficial soft tissues (cutaneous angiosarcomas) make up about 50% of cases. Another 40% of cases involve parenchymal organs (breast, heart, spleen, liver, bone). The remaining 10% occur in deep soft tissues. The peak incidence is seen in the 7th decade. Angiosarcomas are rare in children; but when they do occur, they tend to involve deep soft tissues of head and neck, mediastinum, and internal organs more frequently than in adults. This wide location resection specimen of angiosarcoma from scalp shows a red-purple, multinodular hemorrhagic lesion. The pathologic extent of the tumor was far beyond the clinical size and the resection margins were focally positive.
Clinical Subtypes: On the basis of clinical presentation, pathogenesis, and prognosis, angiosarcomas are subdivided into several groups:
Locations: Tumors arising in skin and superficial soft tissues (cutaneous angiosarcomas) make up about 50% of cases. Another 40% of cases involve parenchymal organs (breast, heart, spleen, liver, bone). The remaining 10% occur in deep soft tissues. The peak incidence is seen in the 7th decade. Angiosarcomas are rare in children; but when they do occur, they tend to involve deep soft tissues of head and neck, mediastinum, and internal organs more frequently than in adults. This wide location resection specimen of angiosarcoma from scalp shows a red-purple, multinodular hemorrhagic lesion. The pathologic extent of the tumor was far beyond the clinical size and the resection margins were focally positive.
Clinical Subtypes: On the basis of clinical presentation, pathogenesis, and prognosis, angiosarcomas are subdivided into several groups:
- Cutaneous Angiosarcoma (unassociated with radiation or lymphedema) - scalp, face, forehead, and neck
- Lymphedema-associated Angiosarcoma (including Stewart-Treves Syndrome)
- Post-radiation Angiosarcoma
- Angiosarcoma of Parenchymal Organs (breast, heart, liver, spleen, bones)
- Angiosarcoma of Deep Soft Tissues