总结已发表的有关颌骨促纤维增生性纤维瘤的信息，对该病症的主要特征进行描述性分析。根据 PRISMA 指南进行了系统评价。在四个数据库和灰色文献中进行了电子检索。包括病例报告和病例系列。获得频率用于描述性分析。我们识别了 66 篇文章，总共 96 个案例。平均年龄为 18.2 岁的 10 岁以内 (40.6%) 的女性患者 (55.8%) 受影响更大。下颌骨是受影响最严重的骨骼，占 81.2% 的病例。主要临床特征是无痛性肿胀（54.2%）。大多数影像学检查（放射学、计算机断层扫描和磁共振）显示明确的射线可透性（65.4%）病变。所有病例的治疗均为手术切除。复发率为10.8%，且全部发生在下颌后牙区。胶原化基质中的梭形细胞成纤维细胞经常被描述为组织病理学特征。波形蛋白、平滑肌肌动蛋白和 β-连环蛋白是常见的免疫组织化学标记物。促纤维增生性纤维瘤是一种局部侵袭性病变，通常影响儿童的颌骨。组织病理学对于诊断至关重要，应进一步研究该肿瘤的发病机制。© 2024 Wiley periodicals LLC。

To summarize published information about the desmoplastic fibroma of the gnathic bones into a descriptive analysis of the main features of this condition.A systematic review according to the PRISMA guidelines was conducted. Electronic search was performed in four databases and in the gray literature. Case reports and case series were included. Frequencies were obtained for descriptive analysis.We identified 66 articles, for a total of 96 cases. Female patients (55.8%) in the first decade of life (40.6%) with a mean age of 18.2 years were more affected. The mandible was the most affected bone with 81.2% of the cases. The main clinical feature was painless swelling (54.2%). Most of the imaging examinations (radiological, computed tomography, and magnetic resonance) showed well-defined radiolucencies (65.4%) lesions. The treatment was surgical removal in all cases. The recurrence rate was 10.8% and all in the posterior mandible. Spindle cell fibroblasts in a collagenized stroma were often described in the histopathological features. Vimentin, smooth muscle actin, and β-catenin were common immunohistochemical markers.Desmoplastic fibroma is a locally aggressive lesion that commonly affects the jaws in children. Histopathology is essential for diagnosis, and the pathogenesis of this tumor should be further investigated.© 2024 Wiley Periodicals LLC.