Department of Pathology, Wake Forest University School of Medicine, Winston-Salem, North Carolina 27157, USA.
Arch Pathol Lab Med. 2012 Feb;136(2):217-21. doi: 10.5858/arpa.2011-0056-RS.
Aggressive angiomyxoma is a rare mesenchymal tumor that most commonly arises in the vulvovaginal region, perineum, and pelvis of women. The term aggressive emphasizes the often infiltrative nature of the tumor and its frequent association with local recurrence. Patients often present with nonspecific symptoms which are frequently misdiagnosed with more common entities, such as a Bartholin cyst, lipoma, or hernia. Histologic examination reveals a hypocellular and highly vascular tumor with a myxoid stroma containing cytologically bland stellate or spindled cells. The tumor cells are characteristically positive for estrogen and progesterone receptors, suggesting a hormonal role in the development of the tumor. Chromosomal translocation of the 12q13-15 band involving the HMGA2 gene has been described. Surgical excision is the treatment of choice, although treatment with gonadotropin-releasing hormone agonists is an emerging therapy. Metastases are exceedingly rare, and overall, the prognosis is good.
侵袭性血管黏液瘤是一种罕见的间叶性肿瘤，最常发生于女性的外阴阴道区域、会阴和骨盆。“侵袭性”一词强调了肿瘤常具有浸润性和局部复发的倾向。患者常表现为非特异性症状，常被误诊为更常见的疾病，如巴氏腺囊肿、脂肪瘤或疝。组织学检查显示肿瘤细胞稀疏，富含血管，黏液样基质中含有细胞学上呈星状或梭形的细胞。肿瘤细胞通常对雌激素和孕激素受体呈阳性，提示肿瘤的发生与激素有关。已描述了涉及 HMGA2 基因的 12q13-15 带的染色体易位。手术切除是首选治疗方法，尽管促性腺激素释放激素激动剂治疗是一种新兴的治疗方法。转移极为罕见，总体预后良好。
