Solid dendritic cell angiosarcoma: Reinterpretation of extraskeletal sarcoma resembling Ewing's sarcoma

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Abstract
Twenty [human] cases of a type of extraskeletal tumor resembling Ewing''s sarcoma were studied. The tumor affected mainly soft tissues in a variety of sites. Throughout the long follow-up period, there was only 1 instance of non-recurrence after excision. Most of the tumors became generalized within 3 yr. One case developed general spread after 13 yr. The tumor shows predominantly expansive growth and a solid architecture which, in places, changes to reticular or sinusoidal structures. The predominant dendritic tumor cells show multiple junction complexes. Weibel-Palade granules can occasionally be demonstrated in their cytoplasm. In foci of a peritheliomatous pattern the tumor cells become elongated, contain an abundance of endoplasmic reticulum and give rise to pericellular basement membranes, although typical collagen was rarely found. The tumor apparently is a form of angiosarcoma, mostly of the endothelial type but with pericytic variations. Since neither its cellular features nor its biological behavior correspond to those of Ewing''s sarcoma, a change of name is proposed.