PDF(Pubmed)
Abstract：
Deep angiomyxoma is a rare, infiltrative, hormone-dependent, benign-mesenchymal neoplasm that occurs in the deep soft tissues of the perineal regions. In total, 33% females with newly diagnosed deep angiomyxoma will typically relapse within 5 years after the standard treatment of radical resection. Postoperative hormone therapy is frequently administered to prevent recurrence, but the role of prophylactic oophorectomy in premenopausal women remain to be fully elucidated. In the present report, a 42-year-old Japanese woman was referred for a refractory Bartholin\'s cyst that is 14 cm in diameter. Based on the results of imaging (unenhanced CT and MRI) and histopathology, deep angiomyxoma was suspected, but no definitive diagnosis was possible. Tumor resection and bilateral salpingo-oophorectomy were performed before the postoperative diagnosis was confirmed to be deep angiomyxoma. The patient received an aromatase inhibitor (2.5 mg letrozole daily) as adjuvant hormonal therapy. There was no evidence of recurrence at the 1-year postoperative follow-up. In conclusion, prophylactic oophorectomy and postoperative adjuvant therapy with aromatase inhibitors may be a promising treatment option for deep angiomyxoma to optimize the outcome of surgical treatment. Long-term follow-up is required to monitor for the late and/or local recurrence of deep angiomyxoma and possible adverse effects of adjuvant hormonal therapy.
摘要：
深部血管黏液瘤是一种罕见的，渗透，激素依赖性,发生在会阴区深部软组织的良性-间充质肿瘤。总的来说,新诊断为深部血管粘液瘤的33％女性通常会在根治性切除术的标准治疗后5年内复发。术后经常进行激素治疗以防止复发，但是预防性卵巢切除术在绝经前妇女中的作用仍有待完全阐明。在本报告中，一名42岁的日本妇女因直径14厘米的难治性Bartholin囊肿而被转诊。根据影像学（未增强CT和MRI）和组织病理学结果，怀疑是深部血管黏液瘤,但无法明确诊断.在术后诊断为深部血管黏液瘤之前，进行肿瘤切除和双侧输卵管切除。患者接受芳香酶抑制剂（每天2.5mg来曲唑）作为辅助激素治疗。术后1年随访无复发迹象。总之,预防性卵巢切除术和芳香化酶抑制剂术后辅助治疗可能是治疗深部血管黏液瘤的一个有前景的选择,以优化手术治疗的结果.需要长期随访以监测深部血管粘液瘤的晚期和/或局部复发以及辅助激素治疗的可能不良反应。
