PubMed2004
Cutaneous vascular proliferations are a vast and complex spectrum. Many appear as hamartomas in infancy; others are acquired neoplasms. Some vascular proliferations are hyperplastic in nature, although they mimic hemangiomas, i.e., neoplasms. The vast majority of the vascular lesions are hemangiomas. Between the hemangiomas and frankly angiosarcomas, there is a group of neoplasms that are angiosarcomas, albeit ones of low grade histologically and, probably, biologically. The term "hemangioendothelioma" has been created to encompass these neoplasms. Vascular proliferations are, fundamentally, composed of endothelial cells. Some hemangiomas, however, contain also abundant pericytic, smooth muscle, or interstitial components, or a combination of them. These heterogeneous cellular components are present usually in hemangiomas. Some of the newly described vascular proliferations, however, are difficult to differentiate from some of the angiosarcomas. Others are markers, occasionally, of serious conditions such as Fabry's Disease (angiokeratoma) and POEM's syndrome (glomeruloid hemangioma). Kaposi's sarcoma continues to be an enigma. The demonstration of Herpes virus 8 in this condition raises doubt about its neoplastic nature. The demonstration of endothelial differentiation of its nodular lesions is tenuous and its true nature remains unresolved. While physicians have known about post-mastectomy angiosarcomas from the origin of the radical mastectomy, a new group of unusual vascular proliferations of the mammary skin are being defined. These lesions arise in the setting of breast-conserving surgical treatment with adjuvant radiation therapy. The incubation period is usually 3 to 5 years, in contrast with the 10, or more, in classical cases of post-mastectomy angiosarcoma. These lesions usually are subtle, both clinically and histologically, in contrast with the "classical," dramatic presentation of mammary angiosarcoma. The spectrum of findings ranges from "simple" lymphangiectasia-like vascular proliferations to unequivocal angiosarcomas. The pathogenesis of these lesions remains a mystery. There are very few clues that allow one to separate hemangiomas from angiosarcomas. The presence of heterologous cellular elements and, particularly, well-developed smooth muscle components tends to favor a hemangioma. Similarly, the presence of thrombosis usually supports hemangioma. Nevertheless, there are no unequivocal or reliable individual diagnostic criteria. A thorough knowledge of the different conditions and their differential diagnoses eventually leads to the proper diagnosis in most cases.