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1、Pigmented Villonodular Synovitis (PVNS)色素沉着绒毛结节性滑膜炎,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 vilionodular synovitis (PVNS) is an uncommon, usually mon
2、oarticular 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
3、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
4、 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, bu
5、t 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 i
6、s 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
7、。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
8、 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 ligm
9、ent. 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。,Diffuse type：T2WI shows multiple low signa
10、l nodules in joint and popliteal space, high signal also can be seen in partial nodules with joint effusion shows hyperintense. Articular surface of tibial platform was invaded and show mixed signal surrounding by low signal ring. Low signal nodule also can be seen in the suprapatellar bursa. 弥漫型PVN
11、S:T2WI 示关节腔内及腘窝多发低信号结节,部分结节内见高信号区,关节腔积液呈高信号。胫骨关节面破坏,呈混杂信号,周围伴低信号环。髌上囊亦见低信号结节. Focal type： T1WI shows well-distributed low signal mass in infrapatellar bursa with a little effusion and joint structures is normal. 局灶型PVNS ,T1WI 示髌下囊内肿块,肿块呈均匀低信号,伴关节腔少量积液,关节骨结构正常,Case 1 35-year-old female patient， progr
12、essive swelling of knee joints for 5 years女，35岁，膝关节进行性肿胀5年,Diffuse type of knee joint:T1WI shows low signal irregular thickened synovium on the anterior and the posterior cruciate ligament. 膝关节弥漫型PVNS。MR T1WI 示前、后交叉韧带表面有不规则增厚的滑膜覆盖,增厚的滑膜呈低信号,T2WI also shows low signal irregular thickened synovium on
13、the anterior and the posterior cruciate ligament. Low signal nodules can be seen in the effusion of suprapatellar bursa. T2WI shows mixed signal of multiple bone destruction, surrounding by a rim of hypointensity. T2WI 示前、后交叉韧带表面有不规则增厚的低信号滑膜覆盖,髌上囊积液内见低信号结节(图5) 。T2WI 示股骨、胫骨多发骨破坏,破坏区高低信号混杂,伴周边低信号环(图6)
14、,Case 2 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: d
15、iffusive 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。,MRI shows nodule on the surface of anterior cruciate ligament and bubble low signal under articular s
16、urface of tibial platform. 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. MRI 示矢状面T1、T2 加权像, 前十字韧带表面结节影, 胫骨关节面下类圆形低信号骨质缺损。HE400滑膜细胞明显增生, 间质浸润
17、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。,Case 326-year-old male patient, diffuse type of right knee joint 男, 26岁,右膝关节弥漫色素沉着绒毛结节性滑膜炎,Figure 1.T1WI: stratified singular signal in the popliteal fossa shows slightly high centre and iso-low rim. Irregular banded low signal can be seen in the suprapatellar b
18、ursa and infrapatellar fat pad. Obviously low signal can be seen below the articular surface of tibia. 图1 右膝关节矢位T1W I示腘窝内见异常信号,呈中心稍高,周边等、低信号的分层状排列,髌上囊及髌下脂肪囊内见形态不规则的片带状低信号影,胫骨前上缘关节面下见小囊状明显低信号影。 Figure 2. fat-suppressed T2WI:stratified singular signal in the popliteal fossa shows obviously high centre
19、 and iso-low rim. Irregular banded high signal can be seen in the suprapatellar bursa and infrapatellar fat pad. Mixed signal can be seen below the articular surface of tibia. 图2右膝关节矢位压脂T2W I示腘窝内异常信号,呈中心明显高、周边等、低信号的分层状排列,髌上囊及髌下脂肪囊内见形态不规则的片带状高信号影,胫骨前上缘关节面下见小囊状高、低混杂信号影。,figure3. fat-suppressed T1WI af
20、ter contrast enhanced: the center of the lesion in the popliteal space shows moderate enhanced, the rim of the lesion、 suprapatellar bursa、 infrapatellar fat pad and popliteal space were obviously enhanced. Obviously annular enhanced lession also be seen below the articular surface of tibia 压脂T1W I增
21、强扫描示腘窝内病灶,中心部分呈中度增强,周边及髌上囊及髌下脂肪囊、腘窝内见形态不规则的片带状明显增强,胫骨前上缘关节面下见环状明显增强。,Case 422-year-old male patient,the PVNS of left hip joint男性, 22岁,右髋关节色素沉着绒毛结节性滑膜炎,The nodular lesion of right hip joint shows hyper-hypo intense-signal on T2WI and iso/hypo mixed-signal on T1WI.The right femoral head was compressiv
22、e deformation and show multiple nodular low signal on T2WI and T1WI. 右髋关节轴位T2WI示右髋关节内见结节状高、低混杂信号,相应区域股骨颈受压变形,股骨头内见多发结节状低信号影.轴位T1W I示右髋关节内见结节状等低混杂信号相应区域股骨颈受压变形,股骨头内见多发结节状低信号影.,Figure6 .enhanced T1WI: the nodular lesion of the right hip joint shows obviously enhanced. 轴位T1W I增强扫描示右髋关节内结节状异常信号明显增强。,Cas
23、e 5 A 43-year-old man presented with pain in the back of the neck radiating to the right upper limb. The pain had progressed over a period of 3 months. There was no weakness of the lower limbs or incontinence of bladder or bowel.43岁男性，颈背部疼痛并放射至右上肢，进行性加重3个月，下肢无症状，无大小便失禁,FIG 1. Lateral radiograph of t
24、he cervical spine shows a soft tissue mass destroying the posterior elements of the lower cervical spine (arrow). There are no foci of calcifications seen within this mass. 图1：颈椎侧位片示软组织肿块破坏颈椎后下部结构，肿块内无钙化点。,FIG 2. Sagittal T1-weighted images before (A) and after (B) contrast dministration show an exp
25、ansile mass lesion involving the posterior elements of C5 and C6 vertebra. This mass shows homogeneous enhancement after contrast material administration.It is seen to displace the thecal sac anteriorly. 图2.矢状位T1WI增强前后示膨胀性的肿块侵犯惊5、6椎体及其附件，肿块呈均匀强化，向前推压硬膜囊。,Axial T1-weighted postcontrast image with fat
26、 suppression shows the exact delineation of the mass with an anteriorly displaced thecal sac (arrow). 轴位增强压脂T1WI示界限明确的肿块向前推压硬膜囊。 Histopathologic section of pigmented villonodular synovitis depicting sheetlike growth within the main tumor mass, which is comprised of mononuclear cells and multinucleat
27、ed giant cells within the collagenized stroma. Hematoxylin and eosin. 组织病理学示变形增殖的滑膜细胞，间以散在的的单核细胞和多核巨细胞。（HE染色）,Differential Diagnosis鉴别诊断,Joint Tuberculosis ：divided into bone tuberculosis and synovial tuberculosis, often occur in hip and knee. Proliferous synovium shows low signal on T1WI and high s
28、ignal 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
29、bone invasion like PVNS, but multiple loose bodies of the joint can help to discern from PVNS. We can see articular cartilage invasion and low signal on T2WI because of hemosiderin deposition . 滑膜软骨瘤病滑膜软骨瘤病可表现为类似PVNS的骨侵蚀,但其多发的关节内游离体(钙化或没钙化)可与PVNS鉴别,同时PVNS可伴有关节软骨破坏及含铁血黄素沉着所形成的特征性T2W I低信号。,Differential Diagnosis鉴别诊断,thanks for your attention!,