MALIGNANT VASCULAR TUMORS OF THE SOFT-TISSUES - CLASSIC AND NEW ENTITIES - REVIEW

Soft tissue vascular tumors display a wide spectrum of clinical and pathological features. The histopathological assessment of soft tissue vascular tumors is generally considered difficult and the main issue remains the distinction between benign and malignant lesions. The degree of malignancy, on the other hand, is also of importance; because it will strongly influence the choice of treatment and supposedly also the prognosis. While many studies of classic malignant vascular tumors, such as angiosarcoma and hemangiopericytoma have been reported, vascular tumors of intermediate malignancy have also recently been described. These tumors are relatively rare; however, it is essential for both pathologists and clinicians to be aware of these new entities. Due to the low-grade nature of these tumors, complete and, ideally, wide local excision without adjuvant therapy should be offered to patients and close follow-up is needed. The prediction of malignancy in hemangiopericytomas has been rended difficult by the idiosyncratic behavior of this tumor and the need for prolonged clinical follow-up. Classic forms of angiosarcoma and malignant hemangiopericytoma should be treated in a similar manner to other high-grade sarcomas,vith radical local excision and possibly also adjuvant chemotherapy.