The Spectrum of Vascular Lesions of the Upper Female Genital Tract: A Report of 55 Cases

Upper female genital tract vascular proliferations are rare and generally not well characterized. We evaluated types, differences in distribution, and associations of such lesions. Fifty-five vascular lesions were identified: 42 benign (ovary 24; uterine corpus 11; para-adnexal 4; fallopian tube 1; ovaries, fallopian tubes, and corpus 1; ovary and fallopian tube 1) and 13 angiosarcomas. Patients with benign vascular lesions had a mean age of 55 (range: 13-82) yr. Twenty-six lesions were incidental findings, and 11 were associated with clinical manifestations. They had a mean size of 2.0 (range: <1-13) cm, and often were grossly cystic and hemorrhagic. Uterine benign vascular lesions included 6 arteriovenous malformations, 3 venous hemangiomas/malformations, 2 cavernous hemangiomas, and 1 mixed venous-cavernous hemangioma. In the ovary, there were 10 anastomosing hemangiomas, 8 arteriovenous malformations, 6 venous (2 in mature cystic teratomas, 1 bilateral in a patient with Klippel-Trenaunay syndrome), and 2 cavernous hemangiomas. Anastomosing hemangiomas were frequently associated with peripheral stromal luteinization/hilar cell hyperplasia; intravascular growth, extramedullary hematopoiesis, and one with adipocytic metaplasia. Venous hemangiomas/malformations were noted at a younger age in the ovary when compared to the uterine corpus. Patients with angiosarcomas had a mean age of 32 (range: 12-58) yr and a mean tumor size of 9.7 (range: 1.5-23) cm. Eight presented with a pelvic mass. Most angiosarcomas were grossly hemorrhagic and/or necrotic. Eleven arose in the ovary, 4 of them were associated with mature cystic teratoma, 1 with adenosarcoma with sarcomatous overgrowth, and 1 was part of a malignant mesenchymoma. Five were predominantly spindled, 3 epithelioid, 2 spindled and epithelioid, and one pleomorphic. Both uterine angiosarcomas were epithelioid. Follow-up was available for 8 patients: 7 died of disease between 6 and 43 mo, and 1 was alive and well at 106 mo. Vascular lesions in the upper female genital tract are uncommon, morphologically heterogeneous, and more frequent and clinically evident in the adnexa. Anastomosing hemangioma is the most common benign vascular lesion in the ovary and may be misdiagnosed as a steroid cell tumor due to associated stromal luteinization/hilar cell hyperplasia. Arteriovenous malformation is the most common benign vascular lesion in the uterine corpus. Angiosarcomas may be associated with another neoplasm, more commonly mature cystic teratoma, and have a poor prognosis.