Hu Xinyu, Gong Yichen, Zhang Zhongyi, Wu Zhipeng, Tong Peijian, Liu Yan, Lv Shuaijie
Department of Orthopedics and Traumatology, The First Affiliated Hospital of Zhejiang Chinese Medical University (Zhejiang Provincial Hospital of Chinese Medicine),, 54 Youdian Road, Hangzhou, Zhejiang Province, 310053, China.
BMC Musculoskelet Disord. 2025 Jul 7;26(1):662. doi: 10.1186/s12891-025-08565-4.
Low-grade myofibroblastic sarcoma (LGMS) is a rare malignant tumor with a predilection for soft tissue and bone. This is a rare malignant tumor with diagnostic challenges, often leading to misdiagnosis as other similar diseases. As a result, its exact incidence remains unclear. While pathologic studies have been reported, systematic clinical, radiological, and therapeutic evaluations remain limited.
This study presented a case of LGMS located in the left distal femur treated with distal femoral resection and total knee arthroplasty using a tumor knee joint system. A review of 24 published cases was conducted for comparative analysis, focusing on clinical presentations, radiological features, immunohistochemical findings, treatment strategies, and long-term outcomes.
Radiological analysis revealed a tumor confined to the distal femoral medullary cavity without cortical destruction or lytic changes. Immunohistochemical staining demonstrated: SMA (+), p53 (+), Ki-67 (30% +), CD163 (+), and CD68 (+), confirming the diagnosis of LGMS. Postoperatively, the patient reported significant pain relief and functional recovery at five months, with VAS, WOMAC, and HSS scores indicating improved joint function. The literature review highlighted LGMS's predilection for local recurrence, emphasizing the importance of early diagnosis and surgical intervention.
LGMS demonstrates unique radiological and pathological features depending on its site of origin. Our case underscores the utility of wide excision and TKA in managing bone-involved LGMS. Further studies are needed to evaluate long-term outcomes and optimize management strategies.
低度恶性肌纤维母细胞肉瘤(LGMS)是一种罕见的恶性肿瘤,好发于软组织和骨骼。这是一种诊断具有挑战性的罕见恶性肿瘤,常导致误诊为其他类似疾病。因此,其确切发病率尚不清楚。虽然已有病理研究报道,但系统的临床、放射学和治疗评估仍然有限。
本研究报告了一例位于左股骨远端的LGMS病例,采用肿瘤膝关节系统行股骨远端切除和全膝关节置换术进行治疗。对24例已发表病例进行回顾以作对比分析,重点关注临床表现、放射学特征、免疫组化结果、治疗策略和长期预后。
放射学分析显示肿瘤局限于股骨远端髓腔,无皮质破坏或溶骨性改变。免疫组化染色显示:平滑肌肌动蛋白(SMA)(+)、p53(+)、Ki-67(30%+)、CD163(+)和CD68(+),确诊为LGMS。术后5个月,患者报告疼痛明显缓解,功能恢复,视觉模拟评分(VAS)、西安大略和麦克马斯特大学骨关节炎指数(WOMAC)及美国特种外科医院膝关节评分(HSS)表明关节功能改善。文献回顾强调LGMS易局部复发,强调早期诊断和手术干预的重要性。
LGMS根据其起源部位表现出独特的放射学和病理学特征。我们的病例强调了广泛切除和全膝关节置换术在治疗累及骨骼的LGMS中的作用。需要进一步研究以评估长期预后并优化管理策略。