外阴阴道复发性侵袭性血管黏液瘤1例伴文献复习

Recurrent aggressive angiomyxoma of vulva and vagina:a case report and a review of literature

目的探讨侵袭性血管黏液瘤的临床表现、病理特点、诊治和预后,加强对其的认知,指导临床,减少误诊。方法采用HE染色及免疫组织化学方法分析侵袭性血管黏液瘤的临床病理学特点。结果 HE染色镜下黏液样背景下梭形或星形细胞疏松排列,肿瘤细胞呈梭形,无核分裂相,瘤细胞在肿瘤中呈弥散均匀分布,其间可见有较为丰富的大小不等的散在血管。免疫组化:Calponin(+)、CD34(血管+)、Desmin(+)、ER(+)、F XIIIA(部分+)、Ki67(<1%+)、MSA(-)、PR(+)、S-100(-)、SMA(少量+)、SOX-10(-)、Myogenin(-)。结论侵袭性血管黏液瘤具有侵袭性及复发性,临床症状、体征缺乏特异性,诊断主要依靠病理学检查,彻底的手术切除是减少复发的主要措施;ER、PR阳性患者,术后可考虑给予GnRH类药物预防复发并严密随访;对于复发性患者,更需强调长期随访的重要性。

Objective To investigate the clinical Feature,pathological characteristics,diagnosis,treatment and prognosis of aggressive angiomyxoma(AAM),so as to strengthen its cognition, guide clinical, reduce misdiagnosis.Methods HE staining and immunohistochemistry were used to analyze the clinicopathological features of aggressive angiomyxoma.Results Under HE staining, the spindle or astrocytes are loosely arranged in the mucus. The tumor cells were fusiform and evenly distributed, with abundant scattered blood vessels of different sizes.Immunohistochemistry showed that Calponin, CD34, Desmin, ER, PR and SMA were positive, F XIIIA was partly positive, Ki67 positive rate was less than 1%,MSA, S-100, SOX-10 and Myogenin all were negative.Conclusion AAM is invasive and recurrent, AAM lacks of specificity in clinical symptoms and signs and its diagnosis mainly depends on pathological examination. and complete surgical resection is the main measure to reduce recurrence. Patients with positive ER and PR can be considered to be given GnRH drugs to prevent recurrence after surgery and to be followed up closely. For recurrent patients, the importance of long-term follow-up should be emphasized.