Loureiro Felippe José Almeida, Nogueira Wictor Rodrigues, Dutra Mateus José, Stelter Grazielle Oliveira, Schuch Lauren Frenzel, Silveira Felipe Martins, Wagner Vivian Petersen, Martins Manoela Domingues, Martins Marco Antônio Trevizani
Department of Oral Diagnosis, Piracicaba Dental School, Universidade de Campinas, Piracicaba, São Paulo, Brazil.
Department of Stomatology, Porto Alegre Clinics Hospital (HCPA/UFRGS), Porto Alegre, Rio Grande do Sul, Brazil.
Oral Dis. 2025 Jan;31(1):32-38. doi: 10.1111/odi.15153. Epub 2024 Oct 7.
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.
总结已发表的有关颌骨促结缔组织增生性纤维瘤的信息，对该疾病的主要特征进行描述性分析。
按照PRISMA指南进行系统评价。在四个数据库和灰色文献中进行电子检索。纳入病例报告和病例系列。获取频率进行描述性分析。
我们确定了66篇文章，共96例病例。10岁以下（40.6%）的女性患者（55.8%）受影响更严重，平均年龄为18.2岁。下颌骨是最常受累的骨骼，占病例的81.2%。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成像）显示边界清晰的透光性（65.4%）病变。所有病例均采用手术切除治疗。复发率为10.8%，均发生在下颌骨后部。组织病理学特征中常描述在胶原化基质中有梭形细胞成纤维细胞。波形蛋白、平滑肌肌动蛋白和β-连环蛋白是常见的免疫组化标志物。
促结缔组织增生性纤维瘤是一种局部侵袭性病变，常见于儿童颌骨。组织病理学对诊断至关重要，该肿瘤的发病机制应进一步研究。
