Abstract
Angiosarcoma is a high-grade vascular tumor arising from endothelial cells of blood vessels. It represents less than 1% of the mesenchymal tumors. Uterine angiosarcoma is an extremely rare tumor with less than 25 cases reported in the literature. It usually presents in postmenopausal women as uterine mass and rarely can arise in a leiomyoma. It is included in the group of tumors of aggressive behavior and poor prognosis. Herein, we present a case of primary uterine angiosarcoma in a 56-year-old female patient with a history of fibroids presenting with pelvic pain and weight loss. Abdominal CT scan showed a large uterine mass and enlarged pelvic lymph nodes. Total abdominal hysterectomy with bilateral salpingo-oophorectomy was performed, and on histopathologic examination, it was found to be primary epithelioid angiosarcoma of the uterus. Immunohistochemical stains for CD31, keratin MAK-6, and keratin AE1/AE3 confirmed the diagnosis. In conclusion, uterine angiosarcoma should be suspected in any rapidly growing hemorrhagic uterine mass, and appropriate sampling and immunohistochemical stains should be considered.
Highlights
Angiosarcoma is an aggressive vascular tumor that mainly arises from soft tissue but can affect any part of the body
Later on in 1949, Cohen et al used the term “hemangioendothelioma of the uterus” [6], which was later termed as epithelioid angiosarcoma
The left external pelvic lymph node was positive for metastatic angiosarcoma involving soft tissue while the cervix, bilateral parametria, and bilateral ovaries and fallopian tubes were negative for tumor
Summary
Angiosarcoma is an aggressive vascular tumor that mainly arises from soft tissue but can affect any part of the body. It usually affects elderly males, and the head and neck are the most common sites of diagnosis [1]. The incidence of angiosarcoma in a woman genital tract is extremely low [2]. It can involve the cervix, ovary, vagina, and rarely the uterus [3, 4]. The idea of malignant vascular tumor involving the uterus was first described by Klob of Europe in 1864 [5]. As a term “primary” underlines, the tumor largely develops de novo with no history of radiation therapy and “epithelioid” as those tumors usually stain positive for the epithelial marker and negative for desmin and other mesenchymal markers
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