Infantile hemangioendothelioma of the ovary: a monodermal teratoma or a neoplasm of ovarian somatic cells?

Vascular tumors of the female genital tract are uncommon, and only a few cases have been reported in the ovary. We describe herein, an unusual tumor of the ovary: infantile hemangioendothelioma (cellular hemangioma of infancy) in a newborn. The tumor consisted of well-formed blood vessels and proliferating endothelial cells that were arranged in solid cordlike structures. The tumor permeated the ovarian stroma and entrapped normal ovarian follicles. By immunohistochemistry the neoplastic cells expressed factor VIII, CD34, and alpha smooth-muscle actin, and ultrastructurally they had the features of endothelial cells that were focally associated with pericytes. We examined simple sequence repeat (SSR) polymorphic markers in the tumor tissue, as well as in the patient's and parents' blood. The informative SSR markers were found to be identical in the tumor and in the patient's somatic cells. We suggest that the tumor described herein is a congenital infantile hemangioendothelioma arising from ovarian parenchymal cells rather than a teratoma originating from germ cells. A similar morphologic lesion has been described recently in the ovary and interpreted as monodermal teratoma composed of vascular tissue.