Primary ovarian angiosarcoma is a very rare gynaecological sarcoma, with poor prognosis. These tumors are though to arise from carcinosarcomas, teratomas, or the ovarian vasculature and occur at any age. There are only a few cases reported in the international literature, most commonly associated to surface epithelial-stromal or germ cell tumours. Herein, the authors report the clinicopathologic features of an angiosarcoma arising in an ovarian fibroma. A 65-year-old patient was admitted with a palpable mass in the hypogastrium. Grossly, the removed ovary was completely replaced by a solid tumor mass. On histological analysis, the lesion revealed the typical histological features of angiosarcoma with sinusoidal patterns and anaplastic cells, admixed with spindle-shaped cells arranged in fascicles or in a storiform pattern, compatible with a fibroma. The vascular component was strongly immunopositive for CD31 and CD34. The patient was submitted to chemotherapy, and she was alive for two months after surgical proceedings. 1. Introduction Mesenchymal neoplasms of the ovary include all primary ovarian neoplasms of connective tissue origin, and not of teratomatous or surface epithelial-stromal origin. This mode of origin cannot be excluded in a number of cases in view of the possibility of one-sided differentiation of a teratoma or of a carcinosarcoma of the ovary. Mesenchymal neoplasms of the ovary can be benign or malignant and are classified on the basis of their line of differentiation. This uncommon group of neoplasms is not specific to the ovary, determining difficult problems in diagnosis, histogenesis, behavior, and therapy . Angiosarcoma is a very rare malignant mesenchymal ovarian neoplasm. The tumor is usually unilateral, with uncertain histogenesis. It may originate from the vascular tissue present in the ovary or from a teratoma in which the vascular component has overgrown the other parts of the tumor. Patients usually have symptoms related to the presence of a lower abdominal mass, which may be associated with torsion of the tumor and hemorrhage [2–5]. Fibroma is the most common ovarian neoplasms of connective tissue origin and constitutes 3%–5% of ovarian neoplasms. The histogenesis of ovarian fibroma is controversial. The neoplasm most likely arises from mesenchymal cells of the ovarian stroma, which differentiate in the fibroblastic direction. Ovarian fibroma is bilateral in 4%–8% of patients and multiple in 10% of cases. Patients with ovarian fibroma frequently are asymptomatic, or they manifest with abdominal pain, urinary symptoms,
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