目的:侵袭性血管黏液瘤是一种少见的间叶性肿瘤,其特点是高复发率,通常在育龄期妇女的下生殖道中观察到。
方法:收集2007年1月至2021年12月在北京朝阳医院经病理证实的侵袭性血管黏液瘤17例。我们收集了临床数据,并总结了临床和免疫组织化学特征。
结果:所有17名患者均为女性,年龄在23至57岁之间(平均,37.7年;中位数,42年)。14例新诊断,3例复发。肿瘤位于外阴(58.8%),阴道(23.5%),臀部(11.8%),和子宫颈(5.9%)。肿瘤最大尺寸为2至15cm(平均8±4.4cm,中位数6厘米)。有9名患者的随访数据,范围从25到124个月(平均,82个月;中位数,80个月)。在后续行动结束时,未报告其他复发或转移。免疫组织化学分析显示雌激素(10/11)和孕激素(8/11)受体具有免疫反应性,desmin(6/8),平滑肌肌动蛋白(4/10),和波形蛋白(4/4),S-100(1/8)和CD34(1/7)。5例患者的Ki67水平低于5%。
结论:AAM是一种激素敏感,明显的罕见间充质肿瘤,局部复发发生率高。手术是首选治疗方法,完全切除是将复发风险降至最低的基本前提。
OBJECTIVE: Aggressive angiomyxoma is an uncommon mesenchymal neoplasm characterized by a high recurrence rate, usually observed in the lower genital tract of women during their reproductive age.
METHODS: Seventeen cases of aggressive angiomyxoma confirmed by pathology from January 2007 to December 2021 in Beijing Chao-yang Hospital were included. We collected clinical data and summarized the clinical and immunohistochemical features.
RESULTS: All seventeen included patients were females, aged between 23 and 57 years (mean, 37.7 years; median, 42 years). Fourteen patients were newly diagnosed and three were recurrent. The tumors were located in vulva (58.8 %), vagina (23.5 %), buttock (11.8 %), and cervix (5.9 %). The tumors size were 2 to 15 cm in greatest dimension (mean 8 ± 4.4 cm, median 6 cm). Follow-up data was available for nine patients, which ranged from 25 to 124 months (mean, 82 months; median, 80 months). At the end of follow-up, no other recurrence or metastasis was reported. Immunohistochemical analysis showed immunoreactive for estrogen (10/11) and progesterone (8/11) receptor, desmin (6/8), smooth muscle actin (4/10), and vimentin (4/4), S-100 (1/8) and CD34 (1/7). The Ki67 level was less than 5 % in five cases.
CONCLUSIONS: AAM is a hormone-sensitive, distinct rare mesenchymal neoplasm with high incidence of local recurrence. Surgery is the preferred treatment, with complete resection being an essential prerequisite for minimizing the risk of recurrence.