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.,Pigmented Villonodular Synovitis (PVNS),2015.06.20,.,Introduction,Pigmented villonodular synovitis (PVNS) comprises a group of idiopathic lesions in joints,tendon sheathes and bursae. (色素沉着绒毛结节性滑膜炎主要是指关节、滑囊以及腱鞘内的滑膜特发性的呈结节状或绒毛状进行性增生。) Pigmented villonodular synovitis (PVNS) is an uncommon, usually monoarticular disorder and usually found in adults. The most common sites predilection is knee-joint(80%),then the hip ,ankle, shoulder, elbow in turn. (不常见,好发于成年人,生于膝关节, 占80 % ,髋关节、踝关节、肩关节、肘关节的发病率依次减少),.,Clinical manifestations,The clinical manifestations is lack of specificity. The main manifestations are progressive swelling of joints and hemorrhagic joint effusion. It shows recurrent joint effusions, which can be misdiagnosed as arthritis. Episodes of complete remission may be found between periods of active disease. (本病的临床表现缺乏特异性.临床主要表现为受累关节进行性肿胀,血性关节积液较常见,本病可引起反复积液,各活动期之间可见病变发作的缓解。) The cause of PVNS is not clear, maybe related with tumor、trauma and infection. (本病的病因尚不明,一般认为与肿瘤、外伤、感染有关。),.,Diagnosis:imaging findings,X-ray: the routine X-ray shows major symptoms including joint capsule swelling , soft tissue mass in periphery of joint and invasion of bone which edge shows ossified ring, but joint space is normal, no osteoporosis . X 线表现: 常规X 线平片上主要征象包括关节囊肿胀、关节周围软组织肿块及邻近骨骼的侵蚀,骨缺损边缘有硬化环,但关节间隙保持正常,无骨质疏松,.,Diagnosis:imaging findings,CT findings: shows joint capsule swelling, thickened synovium and obvious joint effusion. CT 表现: 表现为关节囊肿胀,滑膜增厚,关节腔内明显积液,.,Diagnosis:imaging findings,MR findings is very special and shows thickened synovitis and the condition of joint effusion. MRI 表现具有明显的特征性,能清晰的显示滑膜的增厚和积液的程度。 Because of hemosiderin deposition in the synovitis lesions, it shows low signal both on T1WI and T2WI. Partial lesions show bone invasion. 由于病变滑膜组织内含铁血黄素的沉积,故在T1加权像和T2加权像均呈低信号,这是特征性的征象。部分病变可侵蚀骨结构。,.,Histology and Pathology(组织病理学),The histological findings are exuberant synovial proliferation with numerous villi and folds that sometimes fuse into nodules and form locally aggressive intra-articular masses. 组织学表现为活跃增生的滑膜呈绒毛状或皱襞样,常形成结节状侵袭性的关节内肿物。 PVNS can be divided into focal type and diffuse type. PVNS 在病理上分为局灶型和弥漫型2 种。,.,Diffuse type shows exuberant synovial , villiform proliferation and hemosiderin deposition. Exuberant villus can destroy joint capsule and soft tissue , and invade bone by getting through articular cartilage , junction of bone and joint or the attachment of ligament. 弥漫型主要为滑膜广泛增厚、绒毛状增生和含铁血黄素沉着,增殖的绒毛可破坏关节囊,侵犯周边软组织并通过关节软骨、骨与关节交界部或沿韧带附着处侵犯骨组织. Focal type shows mounds of synovial cells, dispersed multinucleated giant cells , foam cells and pigmented hemosiderin deposition. 局灶型为密集成堆的滑膜细胞,间以散在的多核巨细胞和有类脂质积聚的泡沫细胞,以及含铁血黄素沉着。,.,Case 40-year-old female patient, diffuse type PVNS,Figure 4 :T1WI shows diffusive thickened synovium. Suprapatellar bursa become large because of cloddy synovium. Infrapatellar fat pad disappear instead of proliferous synovium, anterior horn of the lateral meniscus was involved. Figure 5 :T2WI: diffusive proliferous synovium shows low signal because of hemosiderin deposition .MRI T1 加权像, 滑膜弥漫性增厚, 髌上囊因被团状的滑膜组织占据而扩大, 髌下脂肪垫内脂肪缺失, 代之以增厚的滑膜组织, 外侧前角半月板受累及。图5 MRI 示T2 加权像, 弥漫增厚的滑膜呈低信号, 系含铁血黄素沉积所致。,.,Histopathologic section shows proliferous synovial cell, interstitial (within tissues) show roundness and polygon. Partial cells have hemosiderin deposition, multinucleated giant cells can be seen locally. HE400滑膜细胞明显增生, 间质浸润的组织呈圆形或多角形, 部分细胞吞噬含铁血黄素, 局部见融合的多核巨细胞。,.,Differential Diagnosis,Joint Tuberculosis :divided into bone tuberculosis and synovial tuberculosis, often occur in hip and knee. Proliferous synovium shows low signal on T1WI and high signal on T2WI,this is different from PVNS. Contrast enhanced scan is helpful, the low signal on T1WI of PVNS shows moderate or obviously enhanced. 关节结核关节结核分为骨结核及滑膜结核,最常发生于髋关节及膝关节。结核增厚的滑膜T1WI呈低信号, T2WI呈高信号,与PVNS的T2低信号有明显差异; 增强扫描两者有明显差异, PVNS T2WI低信号区增强扫描可呈中度至明显,.,Synovial Chondromatosis :it also shows bone invasion like PVNS, but multiple loose bodies of the joint can help to discern from PVNS. We can see articular cartilage i

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