Primary Epithelioid Angiosarcoma of the Uterus: A Rare Tumor with Very Aggressive Behavior

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.

Primary epithelioid angiosarcoma of endometrium

“…As observed in our case, anastomosing vascular structures, tumoral cells with rounded nuclei, prominent nucleoli and eosinophilic cytoplasm, nuclear pleomorphism, and frequent mitosis are frequently observed features in these tumors [2,10]. However, the histopathological appearance of angiosarcomas can vary, and differential diagnosis might be difficult due to mimickers such as benign proliferative lesions or inflammatory pathologies.…”

“…Therefore, further immunohistochemical and/or molecular examinations are very useful for the diagnosis. CD31 is the most specific marker for this tumor, ERG and cytokeratin positivity are also frequently observed [10,11]. Since the expression pattern of CD34 in angiosarcomas is variable [11], this marker can be positive or negative, it should be kept in mind which its positivity does not make a definitive diagnosis, and negativity does not rule out this diagnosis.…”

“…Angiosarcomas may give symptoms in line with their anatomical localizations, endometrial angiosarcomas often present with vaginal bleeding in postmenopausal women, as in the current case. Radiology is helpful in identifying uterine tumors, but distinguishing angiosarcomas from other uterine tumors may not be feasible with imaging alone [10]. Thus, histopathological studies are needed to confirm the accurate diagnosis.…”

Uterine Sarcoma Presenting as Hypercapnic Respiratory Failure

“…AE1/3 indicates carcinoma, which would also be negative. Less than 25 cases of uterine angiosarcoma have been reported in literature [18][19][20]. Angiosarcomas stain positive for von Willebrand factor (factor VIII), CD34, CD31, and VEGF [21].…”