生殖器外腺肉瘤：一例病例报告、文献综述及治疗讨论
Extragenital adenosarcoma: a case report, review of the literature, and management discussion.
作者信息
Huang Gloria S, Arend Rebecca C, Sakaris Antoinette, Hebert Tiffany M, Goldberg Gary L
机构信息
Division of Gynecologic Oncology, Department of Obstetrics & Gynecology and Women's Health, Department of Pathology, Albert Einstein College of Medicine and Montefiore Medical Center, Albert Einstein Cancer Center, 1695 Eastchester Road, Suite 601, Bronx, NY 10461, USA.
出版信息
Gynecol Oncol. 2009 Dec;115(3):472-5. doi: 10.1016/j.ygyno.2009.07.033. Epub 2009 Aug 26.
BACKGROUND
Müllerian adenosarcoma is a rare mixed epithelial-mesenchymal tumor. An extragenital site of origin and sarcomatous overgrowth are associated with aggressive clinical behavior.
CASE
We present a rare case of extragenital adenosarcoma with sarcomatous overgrowth and coexistent endometriosis. She was treated with initial cytoreductive surgery and chemotherapy. She underwent a second surgery for management of a high-grade bowel obstruction, due to pathologically confirmed recurrent intraperitoneal adenosarcoma. A complete clinical response was achieved with liposomal doxorubicin, and the patient remains disease-free eighteen months after completion of chemotherapy.
CONCLUSION
Liposomal doxorubicin appears to be an active agent for the treatment of adenosarcoma with sarcomatous overgrowth. In addition, we conclude from our review of all reported cases of extragenital adenosarcoma that concurrent endometriosis may represent a favorable prognostic factor.
背景
苗勒管腺肉瘤是一种罕见的上皮-间叶混合性肿瘤。生殖器外起源部位及肉瘤样过度生长与侵袭性临床行为相关。
病例
我们报告一例罕见的生殖器外腺肉瘤，伴有肉瘤样过度生长及并存的子宫内膜异位症。她最初接受了肿瘤细胞减灭术及化疗。因病理证实腹腔内腺肉瘤复发导致高位肠梗阻，她接受了二次手术。脂质体阿霉素治疗取得了完全的临床缓解，化疗结束18个月后患者仍无疾病复发。
结论
脂质体阿霉素似乎是治疗伴有肉瘤样过度生长的腺肉瘤的有效药物。此外，我们通过对所有已报道的生殖器外腺肉瘤病例的回顾得出结论，并存的子宫内膜异位症可能是一个有利的预后因素。
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