ANGIOSARCOMA OF THE OVARY - CLINICOPATHOLOGICAL AND IMMUNOHISTOCHEMICAL ANALYSIS OF 4 CASES WITH A BROAD MORPHOLOGIC SPECTRUM

Angiosarcoma most frequently occurs in the skin of the head and neck r egion of elderly persons, lymphedematous limbs, or in deep soft tissue but only rarely has been described to occur in the female genital tra ct. Four cases of angiosarcoma of the ovary are described herein. They occurred in patients 25 to 42 years old (median, 31 years). The most common clinical presentation was abdominal pain. All of the tumors wer e unilateral, hemorrhagic, and ranged from 3.5 cm to 14 cm (median, 13 cm). The histologic appearance of the tumors was varied, and often th e vascular nature of the tumor was not apparent immediately. Some of t he tumors had a Fascicular growth pat tern composed of spindle-shaped cells with ovoid nuclei and ample eosinophilic cytoplasm closely mimic king leiomyosarcoma. Other tumors resembled ovarian yolk sac tumor wit h a reticular growth pattern, whereas, in other areas, cystic structur es lined by hobnailed hyperchromatic enlarged nuclei simulated clear c ell carcinoma of the ovary. Despite these misleading morphologic findi ngs, all cases were characterized, at least focally, by vasoformative channels or discrete cytoplasmic vacuoles, and all were immunoreactive for Vascular markers. Two patients with spread of tumor outside of th e ovary died 1 month and 2 years after initial diagnosis, respectively . Two patients with tumor confined to the ovary are alive without evid ence of disease 3 and 14 months after diagnosis, respectively. The dif ferential diagnosis of this unusual neoplasm is discussed, and the lit erature is reviewed.