BACKGROUND: Uterine epithelioid angiosarcoma is extremely rare. Evidence-based advice regarding optimal management is lacking. METHOD: We report the 22nd case in the world literature and review all the cases reported in English since 1900. An attempt is made to generate information that may help in the management based on the available literature. RESULTS: A 54-year-old woman was found to have an enlarged uterus on routine clinical examination. Although, she had no other symptoms, an endometrial biopsy was performed, which indicated a high-grade sarcoma. She was taken for a total abdominal hysterectomy and bilateral salpingo-oophorectomy and final histology returned a diagnosis of epithelioid angiosarcoma of the uterus. Two months later, she had tumor recurrence and was started on gemcitabine and taxotere later changed to albumin-bound paclitaxel (ABI-007, Abraxane) and bevacizumab. She was still alive with no evidence of disease at the time of this report, 12 months after diagnosis. Literature review suggests that surgical resection followed by chemotherapy is a reasonable management approach. CONCLUSION: Uterine epithelioid angiosarcoma is an uncommon but very aggressive disease. Surgical resection followed by sequential chemotherapy is a reasonable management approach, and modern chemotherapy and anti-angiogenic agents may have merit.
BACKGROUND: Uterine epithelioid angiosarcoma is extremely rare. Evidence-based advice regarding optimal management is lacking. METHOD: We report the 22nd case in the world literature and review all the cases reported in English since 1900. An attempt is made to generate information that may help in the management based on the available literature. RESULTS: A 54-year-old woman was found to have an enlarged uterus on routine clinical examination. Although, she had no other symptoms, an endometrial biopsy was performed, which indicated a high-grade sarcoma. She was taken for a total abdominal hysterectomy and bilateral salpingo-oophorectomy and final histology returned a diagnosis of epithelioid angiosarcoma of the uterus. Two months later, she had tumor recurrence and was started on gemcitabine and taxotere later changed to albumin-bound paclitaxel (ABI-007, Abraxane) and bevacizumab. She was still alive with no evidence of disease at the time of this report, 12 months after diagnosis. Literature review suggests that surgical resection followed by chemotherapy is a reasonable management approach. CONCLUSION: Uterine epithelioid angiosarcoma is an uncommon but very aggressive disease. Surgical resection followed by sequential chemotherapy is a reasonable management approach, and modern chemotherapy and anti-angiogenic agents may have merit.