【分享】滑膜骨软骨瘤病

天**客

关于滑膜骨软骨瘤病：

　【发病机制】―――不明，目前认为是滑膜受创伤或炎症**后的反应性增生而形成．滑膜细胞发生化生而形成的软骨细胞，在滑膜内沉积，并有较多的血管长入，变成骨化中心，不断的生长，呈息肉状的长蒂状并与滑膜相连，随之增大脱落于关节腔内形成游离体．失去血液{MOD}后，外围的软骨犹如包壳，依靠骨液可以继续生存生长，形成较大较多的瘤体。本病由于侵犯关节，可引起关节疼痛、肿胀，导致功能障碍，所以应早期诊断，早期手术治疗。此时由于创伤小而出血少，故粘连及瘢痕少．有利于术后关节功能的恢复和疼痛症状消失．术中应严格无菌操作，严密上血，避免周围组织再次损伤，彻底情除关节内的游离体及病灶组织，防上复发．感染，术后粘连和脱位，影响功能恢复。

【病理改变】―――为滑膜充血，增厚，绒毛增殖，表现散布大小不等结节，小者如粟粒，大者直径可达 Icm以上。结节质硬，呈白色或半透明状，偶有呈淡紫色者；结节以阔底或窄蒂与滑膜相连，或脱落入关节腔，或位于滑膜囊及关节附近的腱鞘内，成为游离体。其形状各异，以圆形或卵圆形居多。剖面为软骨，内可钙化或骨化，其中心部可有脂性骨髓。镜下可见血管增生，淋巴细胞聚集，滑膜表层细胞及纤维组织增生，有时可见成片的成纤维细胞化生为软骨细胞，形成软骨小体并钙化和骨化等不同阶段。 M lie ram通过病理形态观察把本病分为三个阶段：①急性期：滑膜改变明显，但关节内无游离体；②中间期．过渡性滑膜病变合并滑膜骨软骨瘤及游离体；③骨膜病变终止期：滑膜恢复正常或仅有轻度炎症，多个游离体形成。

　【临床表现】―――与一般慢性关节炎和关节内游离体的表现相同．但病程缓慢．多数患者早期可无症状，持续数月，数年，甚至数十年后，受累关节渐渐出现疼痛、肿胀、功能受限，活动时关节内可有异物感及绞锁现象，有时可钢及活动性包块。

　【好发人群、部位】　－由以上病例可知，本病例多发于成年人，多为女性。几乎全为大关节发病，其中膝关节发病占到65%，髋关节发病占到28%，肩关节也有发病。
　　【典型影像表现】    x线―改变为关节部位有多个圆形、卵圆形不透明阴影，密度可呈匀质性增高，也可周边密度高．而中心透亮。关节间隙和关节面一般保持正常。晚期病人可见关节面边缘骨质增生的退性变。X线平片约有1／3病人无钙化或骨化改变，如做充气造影，可见多个负性阴影。。
CT-－表现大体类似于平片所见，只是从断面上可见游离体与关节构成骨分界清楚，关节构成骨多无破坏。
MRI－MRT1WI、T2WI及FSEIR均与股骨中央黄骨髓组织信号一致，病灶中央可见点状T1低信号及FSEIR高信号应考虑为未骨化的软骨组织。

　　

爱**魂

[ Pathogenesis ] ¨d¨d¨d is unclear, after at present thinks is 滑膜 stimulates the wound or the inflammation the reactivity proliferation forms. 滑膜 the cell has the metaplasia forms 软骨细胞, in 滑膜 inside deposits, and has more blood vessels long enters, turns 骨化 the center, the unceasing growth, assumes 息肉 the shape long peduncle shape and with 滑膜 is connected, increases along with it falls off forms in the joint cavity drifts away the body. After loses the blood supply, the pe**hery cartilage just like the package of shell, depends upon the bone fluid may continue to survive grows, forms the comparatively greatly more lumps body. This gets sick because encroaches upon the joint, may cause the joint ache, swelling, causes the function barrier, therefore should 早期诊断, the early surgery treat. This time small bleeds as a result of the wound few, therefore the adhesion and the scar are few. Is advantageous the joint function restoration and the ache symptom vanishes after the technique. In the technique should the strict aseptic operation, on the strict blood, avoid pe**hery organizing once more to damage, 彻 底情 drifts away the body except the joint in and the sickness stove organization, guards against recurs. The infection, after the technique the adhesion and 脱位, affects the function restoration.

[ Pathology change ] ¨d¨d¨d for 滑膜 the hyperemia, the accumulation, 绒毛 the multiplication, the performance spreads the size does not wait 结节, the small like millet grain, the big diameter may reach above Icm. 结节 the nature is hard, assumes the white or half transparency, the partner has assumes the lavender; 结节 with 滑膜 is connected by the extravagant bottom or the narrow peduncle, either falls off into the joint cavity, either locates 滑膜 in the nei**orhood of the pouch and the joint 腱鞘 inside, becomes drifts away the body. Its shape respectively different, is in the majority by the circular or the egg circular. The section plane is the cartilage, inside may 钙化 or 骨化, it 中心部 may have the fat marrow. Under the mirror obviously the blood vessel proliferation, the lymphocyte gathers, 滑膜 the surface layer cell and the fiber structure proliferation, sometimes obviously Large expanse of becomes the ciliary cell metaplasia for 软骨细胞, forms 软骨小体 and 钙化 and 骨化 and so on the different stage. M lie ram divides into through the pathology shape observation this sickness three stages: (1) Acute time: 滑膜 the change is obvious, but in the joint not drifts away the body; (2) 中间期. The transition 滑膜 pathological change merge 滑膜 骨软骨瘤 and drifts away the body; (3) 骨膜 the pathological change terminates the time: 滑膜 restores normal or only has the mild inflammation, many drift away the body formation.

[ 临床表现 ] ¨d¨d¨d drifts away the body performance with in the common chronic arthritis and the joint is same. But the course of an illness is slow. The most patients early time may do not have the symptom, continues several months, several years, after even several ten years, exhaust oneself the joint gradually have the ache, swelling, the function receive limit, when activity in the joint may have the foreign matter feeling and twist locks the phenomenon, sometimes may the steel and 活动性 the package of block.

[ Good sends crowd, spot ] - may know by the above case of illness, this case of illness sends much to the **, are many is the female. Nearly all arises for the big joint, knee joint arises accounts for 65%, the hip joint arises accounts for 28%, the shoulder joint also has arises.
[ Typical image performance ] x ¨D change has the many circulars, the egg circular opaque shadow for the joint spot, the density may assume the uniform nature advances, also may the pe**heral density be high. But the center is translucent. The joint gap and 关节面 the general maintenance is normal. Later period the patient obviously 关节面 edge ossein proliferation draws back the change in sexuality. X even piece approximately has 1 / 3 patients does not have 钙化 or 骨化 the change, if makes the gasification radiography, obviously many negative shadows.
The CT- - performance roughly is similar to the even piece institute sees, only is obviously drifts away from the cross section the body and the joint constitution bone dividing line is clear, the joint constitution bone does not have the destruction.
MRI - MRT1WI, T2WI and FSEIR is all consistent with the thi**one central yellow marrow organization signal, the sickness stove central obviously selects the shape T1 low signal and the FSEIR high signal should consider for not 骨化 the cartilage organization.