囊性畸胎瘤向血管肉瘤的恶变：病例分析和文献回顾

　　Malignant transformation of a cystic teratoma into an angiosarcoma: A case and review of the treatment literature

　　C. Albertin¹, J. Connor², A. Al-Niaimi²

　　¹University of Wisconsin School of Medicine and Public Health, ²University of Wisconsin Hospitals and Clinics, Madison, WI, USA

　　Introduction: While mature cystic teratomas are the most common ovarian tumors and are usually benign, they carry up to a 2% risk of malignant transformation. An angiosarcoma is a very rare form of this transformation. We present a case of malignant transformation of a mature cystic teratoma into an angiosarcoma and review the literature on treatment options for this aggressive disease.

　　Case report: A 64-year-old female with acute abdominal pain presented with symptoms and signs suggestive of acute peritonitis. A CT scan showed large abdominal masses invading and perforating the sigmoid colon and terminal ileum. Despite debulking and adjuvant paclitaxel, the patient´s disease rapidly progressed and she died 8 weeks after diagnosis.

　　Current NCCN guidelines for intra-abdominal soft tissue sarcomas recommend tumor resection with or without preoperative chemotherapy or radiation therapy. For recurrent disease, a combination of surgery, chemotherapy, radiation therapy, and/or supportive care is indicated. Specific chemotherapies suggested for angiosarcomas include paclitaxel, docetaxel, vinorelbine, sorafenib, sunitinib, and bevacizumab or other chemotherapies used for soft tissue sarcomas of the abdomen and extremities. However, angiosarcomas may have a more robust response to paclitaxel.

　　Conclusion: Angiosarcomas of the ovary are very rare. After debulking, adjuvant paclitaxel is commonly used. The aggressive nature of this cancer warrants exploring the use of the newer anti-angiogenic chemotherapies that may provide longer progression-free survival.

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