Background: Bone desmoplastic fibroma (DF) is a rare, locally aggressive, benign tumor. Due to its low incidence, studies on the topic have been limited to case reports and a few case series. This review aims to summarize modern literature on bone DF and provide an overview of its clinical presentation and prognostic horizons after surgical treatment. Methods: We systematically searched for articles reporting on DFs treated surgically. Our research was conducted according to the PRISMA guidelines, including PubMed, Embase, and Scopus articles between 1958 and 2025. Lesions’ location and size, tumors’ symptoms, the surgical treatment of choice, and the recurrence rates at patients’ latest follow-up were recorded. Results: A total of 97 articles and 187 cases were included. The mean age was 24.3. There was no gender difference. The lower limb was the most common localization (87 cases; 47%), followed by the upper limb (48; 26%), mandible (27; 14%), spine (17; 9%), and other sites (8; 4%). Pain was detected in 73% of cases and swelling in 53%. Seventeen patients (11%) had pathological fractures. Focal resections and curettage were the most common surgical treatments. Thirty-eight cases (23%), most treated with intralesional curettage, had local recurrence after a mean follow-up of 63 months. Conclusions: Despite its low incidence, desmoplastic fibromas should be considered in the differential diagnosis of symptomatic osteolytic bone lesions. Although curettage can be considered as a reasonable solution for selected cases, wide resections are recommended when feasible to minimize the risk of local recurrence.
背景:骨促结缔组织增生性纤维瘤是一种罕见的局部侵袭性良性肿瘤。由于其发病率低,相关研究仅限于病例报告和少量病例系列。本综述旨在总结关于骨促结缔组织增生性纤维瘤的现代文献,概述其临床表现及手术治疗后的预后情况。 方法:我们系统检索了报道手术治疗的促结缔组织增生性纤维瘤的文献。研究遵循PRISMA指南,涵盖1958年至2025年间PubMed、Embase和Scopus数据库中的文章。记录病变位置与大小、肿瘤症状、选择的手术治疗方案以及患者末次随访时的复发率。 结果:共纳入97篇文章,涉及187例病例。患者平均年龄24.3岁,无性别差异。下肢是最常见发病部位(87例,47%),其次为上肢(48例,26%)、下颌骨(27例,14%)、脊柱(17例,9%)及其他部位(8例,4%)。73%的病例出现疼痛症状,53%出现肿胀。17例患者(11%)发生病理性骨折。病灶切除和刮除术是最常用的手术治疗方式。平均随访63个月后,38例(23%)出现局部复发,其中多数曾接受病灶内刮除治疗。 结论:尽管发病率较低,对于有症状的溶骨性骨病变进行鉴别诊断时,应考虑促结缔组织增生性纤维瘤的可能性。虽然刮除术可作为特定病例的合理治疗方案,但建议在可行情况下实施广泛切除术,以最大限度降低局部复发风险。