外阴侵袭性纤维瘤病1例并文献复习

A Case of Aggressive Fibromatosis of Vulva and Literature Review

摘要: 目的：探讨侵袭性纤维瘤(Aggressive fibromatosis, AF)的病因、临床表现、病理特征、诊断及治疗方法和预后。方法：对1例外阴侵袭性纤维瘤患者的病例资料及相关文献进行回顾性分析。结果：侵袭性纤维瘤病是一类临床少见的软组织肿瘤，可发生于全身各处，外阴部位发病罕见。肿瘤易局部复发，无远处转移能力，发病与内分泌、创伤、遗传等多因素相关。该病常表现为无痛性肿块，可压迫或局部侵犯周围组织产生相应临床症状。MRI是明确肿瘤大小、位置、与周围组织关系的首选影像学检查。该病具有较明确的病理特征：多无包膜，边界不清，质硬韧，镜下见束状平行排列的呈长梭形成纤维细胞和肌纤维母细胞，细胞呈浸润性生长，无病理性核分裂及异型性。结论：侵袭性纤维瘤病因不明，发病部位不一，临床表现无明显特异性。病理学检查为诊断的金标准。治疗方法主要为手术切除，术后复发率较高，应定期随访。

Abstract: Objective: The objective is to explore the etiology, clinical manifestations, pathological features, diagnosis, treatment methods and prognosis of aggressive fibromatosis (AF). Methods: Case data and related literature of a patient with aggressive fibromatosis of vulva were retrospectively analyzed. Results: Aggressive fibromatosis is a kind of clinically rare soft tissue tumor that can occur throughout the body, and the onset of the vulva site is rare. Tumors are prone to local recurrence, without distant metastatic ability, and the pathogenesis is associated with endocrine, genetic, trauma and other factors. The disease often presents as a painless mass, which can compress or locally invade the surrounding tissue to produce the corresponding clinical symptoms. MRI is the preferred imaging examination to define the tumor size, location and relationship with the surrounding tissues. The disease has clear pathological characteristics: mostly no capsule, unclear boundary, hard toughness, microscopic parallel arrangement of bundles to form fibroblast cells and myofibroblast cells. Cells show invasive growth, no pathological nuclear division and atypia. Conclusions: Aggressive fibromatosis has unknown etiology, variable onset sites, and no obvious clinical specificity. Pathological examination is the gold standard for diagnosis. The main treatment is surgical resection, with a high postoperative recurrence rate, and postoperative follow-up is required.