Olawaiye Alexander B, Morgan Jeffrey A, Goodman Annekathryn, Fuller Arlan F, Penson Richard T
Gynecologic Oncology Division, Department of Obstetrics, Gynecology and Reproductive Biology, Massachusetts General Hospital/Harvard Medical School, 55 Fruit Street, Boston, MA 02114, USA.
Arch Gynecol Obstet. 2008 Nov;278(5):401-4. doi: 10.1007/s00404-008-0727-z. Epub 2008 Jul 19.
Uterine epithelioid angiosarcoma is extremely rare. Evidence-based advice regarding optimal management is lacking.
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.
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.
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.
子宫上皮样血管肉瘤极为罕见。目前缺乏关于最佳治疗方案的循证建议。
我们报告了世界文献中的第22例病例，并回顾了自1900年以来英文报道的所有病例。我们试图根据现有文献生成有助于治疗的信息。
一名54岁女性在常规临床检查中发现子宫增大。尽管她没有其他症状，但仍进行了子宫内膜活检，结果显示为高级别肉瘤。她接受了全腹子宫切除术和双侧输卵管卵巢切除术，最终组织学诊断为子宫上皮样血管肉瘤。两个月后，她出现肿瘤复发，开始使用吉西他滨和多西他赛治疗，后来改为白蛋白结合型紫杉醇（ABI - 007，Abraxane）和贝伐单抗。在本报告时，即诊断后12个月，她仍然存活，无疾病证据。文献综述表明，手术切除后进行化疗是一种合理的治疗方法。
子宫上皮样血管肉瘤是一种罕见但极具侵袭性的疾病。手术切除后序贯化疗是一种合理的治疗方法，现代化疗和抗血管生成药物可能具有优势。
