Murphey Mark D, Vidal Jorge A, Fanburg-Smith Julie C, Gajewski Donald A
Department of Radiologic Pathology, Armed Forces Institute of Pathology, Washington, DC 20306, USA.
Radiographics. 2007 Sep-Oct;27(5):1465-88. doi: 10.1148/rg.275075116.
Primary synovial chondromatosis represents an uncommon benign neoplastic process with hyaline cartilage nodules in the subsynovial tissue of a joint, tendon sheath, or bursa. The nodules may enlarge and detach from the synovium. The knee, followed by the hip, in male adults are the most commonly involved sites and patient population. The pathologic appearance may simulate chondrosarcoma because of significant histologic atypia, and radiologic correlation to localize the process as synovially based is vital for correct diagnosis. Radiologic findings are frequently pathognomonic. Radiographs reveal multiple intraarticular calcifications (70%-95% of cases) of similar size and shape, distributed throughout the joint, with typical "ring-and-arc" chondroid mineralization. Extrinsic erosion of bone is seen in 20%-50% of cases. Computed tomography (CT) optimally depicts the calcified intraarticular fragments and extrinsic bone erosion. Magnetic resonance (MR) imaging findings are more variable, depending on the degree of mineralization, although the most common pattern (77% of cases) reveals low to intermediate signal intensity with T1-weighting and very high signal intensity with T2-weighting with hypointense calcifications. These signal intensity characteristics on MR images and low attenuation of the nonmineralized regions on CT scans reflect the high water content of the cartilaginous lesions. CT and MR imaging depict the extent of the synovial disease (particularly surrounding soft-tissue involvement) and lobular growth. Secondary synovial chondromatosis can be distinguished from primary disease both radiologically (underlying articular disease and fewer chondral bodies of variable size and shape) and pathologically (concentric rings of growth). Treatment of primary disease is surgical synovectomy with removal of chondral fragments; recurrence rates range from 3% to 23%. Malignant transformation to chondrosarcoma is unusual (5% of cases) and, although difficult to distinguish from benign disease, is suggested by multiple recurrences and marrow invasion. Recognizing the appearances of primary synovial chondromatosis, which reflect their underlying pathologic characteristics, improves radiologic assessment and is important to optimize patient management.
原发性滑膜软骨瘤病是一种少见的良性肿瘤性病变,在关节、腱鞘或滑囊的滑膜下组织中出现透明软骨结节。这些结节可能会增大并从滑膜上脱落。男性成年人中,膝关节最常受累,其次是髋关节,这是最常见的发病部位和患者群体。由于显著的组织学异型性,其病理表现可能类似软骨肉瘤,通过影像学检查来确定病变起源于滑膜对于正确诊断至关重要。影像学表现通常具有特征性。X线片显示关节内多发大小和形状相似的钙化影(70%-95%的病例),分布于整个关节,具有典型的“环状和弧形”软骨样矿化。20%-50%的病例可见骨的外在侵蚀。计算机断层扫描(CT)能最佳地显示关节内钙化碎片和骨的外在侵蚀。磁共振成像(MR)表现则更具多样性,取决于矿化程度,不过最常见的表现(77%的病例)是T1加权像呈低到中等信号强度,T2加权像呈非常高信号强度且钙化呈低信号。MR图像上这些信号强度特征以及CT扫描上非矿化区域的低衰减反映了软骨病变的高含水量。CT和MR成像可显示滑膜病变的范围(尤其是周围软组织受累情况)以及小叶状生长。继发性滑膜软骨瘤病在影像学上(存在潜在关节疾病且软骨体数量较少、大小和形状各异)和病理上(生长呈同心环)均可与原发性疾病相鉴别。原发性疾病的治疗方法是手术切除滑膜及软骨碎片;复发率为3%至23%。恶变成为软骨肉瘤的情况不常见(5%的病例),虽然难以与良性疾病区分,但多次复发和骨髓侵犯提示恶变可能。认识原发性滑膜软骨瘤病的表现,这些表现反映了其潜在的病理特征,有助于提高影像学评估,对优化患者管理很重要。