高明暄 李旭升 邵宏斌 陳彥飛 常彥峰 李生貴 郝曉東
. 病例報(bào)告 Case report .
膝關(guān)節(jié)及髕骨內(nèi)色素沉著性絨毛結(jié)節(jié)性滑膜炎一例報(bào)告
高明暄 李旭升 邵宏斌 陳彥飛 常彥峰 李生貴 郝曉東
滑膜炎,色素絨毛結(jié)節(jié)性;巨細(xì)胞瘤;髕骨;膝關(guān)節(jié)
色素沉著性絨毛結(jié)節(jié)性滑膜炎 (pigmented villonodular synovitis,PVNS) 是發(fā)生于關(guān)節(jié)或腱鞘滑膜組織的少見(jiàn)良性腫瘤性疾病,其進(jìn)展后期,病變可自關(guān)節(jié)內(nèi)侵犯、破壞鄰近軟骨及骨組織,但是不破壞關(guān)節(jié)面,在骨內(nèi)直接形成局限病灶的情況未見(jiàn)有報(bào)道[1-3]。我科 2014 年 11 月收治1 例同時(shí)發(fā)生于膝關(guān)節(jié)和髕骨內(nèi)的 PVNS 罕見(jiàn)病例?,F(xiàn)將該病例的病史、影像學(xué)、手術(shù)、病理診斷等臨床資料,以及簡(jiǎn)要文獻(xiàn)綜述報(bào)告如下。
患者,女,37 歲,“左膝酸痛不適 3 年余,加重伴膝后發(fā)現(xiàn)包塊半年”入院。3 年前無(wú)明顯誘因出現(xiàn)左膝勞累后酸痛不適,經(jīng)休息后即減輕,未予以重視和特殊處理。近半年來(lái),上述癥狀漸加重,影響左膝活動(dòng),且無(wú)意中發(fā)現(xiàn)左窩一雞蛋大小包塊,故就診于當(dāng)?shù)蒯t(yī)院,經(jīng)行 B 超檢查,診斷為“左窩包塊待查”后,建議轉(zhuǎn)來(lái)我院診治?;颊咦栽V:左膝未見(jiàn)彌漫性腫脹、發(fā)熱、關(guān)節(jié)絞鎖以及突然劇烈疼痛等情況。既往體健。查體:左膝輕度彌漫性腫脹,膚色如常,左窩較對(duì)側(cè)稍顯飽滿(mǎn);皮溫正常,髕前及窩壓痛陽(yáng)性,髕骨研磨試驗(yàn)陽(yáng)性,浮髕試驗(yàn)陰性;窩正中可及一近圓形包塊,質(zhì)硬,無(wú)活動(dòng)性,與周?chē)M織界限清,直徑約 3 cm;左膝活動(dòng)度 10°~115°,輕度受限;左膝穩(wěn)定性試驗(yàn)均陰性。
化驗(yàn):ESR:8 mm / h,CRP:3.1 mg / L,未見(jiàn)明顯異常。X 線 (圖 1):關(guān)節(jié)對(duì)位關(guān)系正常,股骨及脛骨關(guān)節(jié)面邊緣可見(jiàn)退化、增生表現(xiàn),膝關(guān)節(jié)后側(cè)窩處,軟組織內(nèi)不規(guī)則密度增高影 (圖 1b),髕骨正、側(cè)及軸位均示髕骨內(nèi)側(cè)骨內(nèi)囊性破壞,累及髕骨近 1 / 2,病灶內(nèi)有骨嵴分隔,邊緣骨質(zhì)硬化,邊界清,髕骨關(guān)節(jié)面軟骨下骨及前側(cè)皮質(zhì)尚未穿孔破損 (圖 1)。MRI (圖 2):左側(cè)髕骨內(nèi)可見(jiàn)囊狀異常信號(hào),T1(圖 2a) 加權(quán)呈等低信號(hào),PDWI (圖 2b) 呈稍高信號(hào),邊界清楚,可見(jiàn)長(zhǎng) T1短 T2(圖 2c)信號(hào)硬化邊影。左側(cè)膝關(guān)節(jié)后側(cè)滑膜增厚,并見(jiàn)條形及結(jié)節(jié)樣長(zhǎng) T1短 T2信號(hào)影,關(guān)節(jié)后側(cè)窩區(qū)可見(jiàn)不規(guī)則結(jié)節(jié)樣及囊狀長(zhǎng) T1長(zhǎng) T2信號(hào)影,囊內(nèi)可見(jiàn)短 T2信號(hào)分隔影,增強(qiáng)掃描病灶邊緣可見(jiàn)環(huán)形及片絮狀強(qiáng)化影,邊緣模糊,MRI 影像診斷為 PVNS。 經(jīng)行關(guān)節(jié)穿刺,抽出僅 1 ml 黏稠棕黃色液體,常規(guī)及生化化驗(yàn),紅細(xì)胞,白細(xì)胞計(jì)數(shù)、蛋白定量均未見(jiàn)明顯異常。
圖 1 正、側(cè)、軸位 X 線示左側(cè)髕骨一邊累清晰的溶骨性病灶,髕骨內(nèi)側(cè)半基本被病灶所破壞。另外,還有退行性骨贅形成和輕微關(guān)節(jié)腫脹表現(xiàn)Fig.1 Frontal (a), lateral (b) and the axial (c) views of the patella showed a well-defned lytic lesion in the left patella, which had eroded almost all the medial half of the patella. Small degenerative osteophyte formation and slight knee joint effusion were also presented
圖 2 矢狀 T1加權(quán)、矢狀質(zhì)子壓脂以及 T2加權(quán)位核磁共振成像示左膝關(guān)節(jié)廣泛滑膜增生伴結(jié)節(jié)樣病灶。髕骨內(nèi)及窩內(nèi)信號(hào)類(lèi)似病灶,界限清晰,呈 T1低信號(hào),T2中等強(qiáng)度信號(hào)和質(zhì)子壓脂高信號(hào)。病灶內(nèi)間有T1、T2均呈低信號(hào)區(qū)域圖 3 短“S”形膝后側(cè)入路,顯露、分離和切除膝后側(cè)病灶,病灶呈黃棕色,與周?chē)M織界限清,與關(guān)節(jié)囊后側(cè)粘連緊,蒂部與膝內(nèi)滑膜組織連續(xù)Fig.2 Sagittal T1-weighted (a) and sagittal T2-weighted with fat saturation / STIR (b) and axial T2-weighted (c) magnetic resonance images (MRI) of the left knee reveal extensive nodular synovial proliferation with lobulated margins, a well-defned lesion confned to the patella and a lobulated mass-like lesion in the poplitea. The signal intensity of the lesions was low on T1-weighted image, intermediate on T2-weighted and high STIR. There were small signal areas with low signal intensity featuring both on T1and T2Fig.3 A short S-shaped incision was made over the posterior knee joint. The posterior lesion in the poplitea was exposed, dissected and marginally resected
圖 4 關(guān)節(jié)鏡下廣泛增生的滑膜組織,呈現(xiàn)出絨毛樣、色素沉著、結(jié)節(jié)樣增生滑膜。鏡下未見(jiàn)髕骨內(nèi)病灶與關(guān)節(jié)內(nèi)滑膜相連的直接證據(jù),髕骨關(guān)節(jié)面雖然有軟骨軟化,但未見(jiàn)明顯的蠶噬性破損Fig.4 Arthroscopic images (a, b) of the left knee indicating prolifc synovium with coarse villi, heavily pigmented diffuse pigmented villonodular synovitis. No communicating links were found under arthroscopic observation. Although the chondromalacia changes were visible on the articular surface of the patella, it seemed exactly intact without any eroded holes
初步診斷為 PVNS,制訂手術(shù)治療方案,主要為 3 個(gè)部分:(1) 左窩腫物切除;(2) 左膝關(guān)節(jié)鏡探查及病變切除、清理;(3) 左髕骨內(nèi)病變切除、植骨術(shù)。全身麻醉,左大腿根部氣囊止血帶 450 mm Hg。先俯臥位,常規(guī)消毒鋪單,取左膝關(guān)節(jié)后側(cè)入路,逐層顯露,分離保護(hù)神經(jīng)血管束,向外側(cè)牽開(kāi),即可見(jiàn)一連接于左膝后側(cè)關(guān)節(jié)囊的長(zhǎng)條狀黃褐色的腫物,具有包膜,易分離,其蒂部與關(guān)節(jié)囊后側(cè)緊密相連 (圖 3)。將腫物予以分離,完整切除,見(jiàn)腫物內(nèi)腫瘤組織與關(guān)節(jié)腔內(nèi)滑膜組織相連續(xù)。術(shù)中冰凍病檢符合 PVNS。經(jīng)后部對(duì)膝后增生病變滑膜組織進(jìn)行病灶切除。沖洗清點(diǎn)器械后,修補(bǔ)縫合后側(cè)關(guān)節(jié)囊,置引流管,逐層縫合后側(cè)切口。然后,患者改仰臥位,取膝前髕韌帶內(nèi)、外側(cè),并輔以膝關(guān)節(jié)后內(nèi)側(cè)和后外側(cè)膝關(guān)節(jié)鏡標(biāo)準(zhǔn)通道入路,置入關(guān)節(jié)鏡系統(tǒng)。逐一探查髕上囊、內(nèi)側(cè)、外側(cè)間隙、關(guān)節(jié)前后腔。股骨及脛骨關(guān)節(jié)面,前、后交叉韌帶及半月板等結(jié)構(gòu)和組織未見(jiàn)明顯異常等 (圖 4)。股髕關(guān)節(jié)間隙,髕骨關(guān)節(jié)面僅存在輕度軟骨軟化,未見(jiàn)病變滑膜組織累及,也未見(jiàn)穿孔破損 (圖 4c,d)。但是,關(guān)節(jié)后腔內(nèi),肌腱周?chē)そM織增生肥厚,鐵銹色,部分呈絨毛樣間有結(jié)節(jié)增生 (圖 4a,b),用髓核鉗咬取典型病變組織留送病檢后,用刨刀將增生絨毛及結(jié)節(jié)徹底刨削至滑膜外脂肪組織,再用等離子電刀止血同時(shí)對(duì)病變部位進(jìn)行灼燒,關(guān)節(jié)腔清理后,完成關(guān)節(jié)內(nèi)鏡下滑膜病變切除。開(kāi)始行手術(shù)最后一部分,在髕骨前內(nèi)側(cè)取縱向短切口,長(zhǎng)約 4 cm,全層切至髕骨骨膜,在前側(cè)皮質(zhì)開(kāi)骨窗,大小1.5 cm×1.5 cm,翻開(kāi)骨皮質(zhì),見(jiàn)髕骨內(nèi)病變組織呈黃褐色,有包膜 (圖 5a)。完整刮出病變組織,見(jiàn)髕骨腔內(nèi),周?chē)潜谟不轻招纬?,髕骨關(guān)節(jié)面?zhèn)韧旰?,空腔?nèi)注水后未見(jiàn)滲露,提示髕骨病變與關(guān)節(jié)腔并不相通 (圖 5b)。高速磨鉆磨除硬化骨壁至正常骨質(zhì),然后用 50% 氯化鋅燒灼瘤壁,沖洗后,取與髕骨缺損同樣大小的自體髂骨行髕骨缺損重建 (圖 5c),并放回翻開(kāi)骨皮質(zhì),清點(diǎn)后縫合切口。切除左膝關(guān)節(jié)后腫物、關(guān)節(jié)腔內(nèi)滑膜病變組織及髕骨內(nèi)病變分別標(biāo)記,留送常規(guī)病檢。
病理報(bào)告三處不同部位病變均符合 PVNS 的病理特征(圖 6:鏡下,彌漫的滑膜細(xì)胞增殖,同時(shí)伴有多核巨細(xì)胞、黃瘤細(xì)胞及少量的含鐵血黃素沉積)。術(shù)前 30 min 至術(shù)后 24 h 內(nèi),應(yīng)用頭孢唑啉鈉預(yù)防手術(shù)切口感染。術(shù)后24 h 內(nèi)左膝關(guān)節(jié)冰敷,塞來(lái)昔布 50 mg 鎮(zhèn)痛。24 h 拔除引流管后開(kāi)始膝關(guān)節(jié)功能恢復(fù),1 周后非負(fù)重下達(dá)到術(shù)前水平,屈曲 115°,即下地負(fù)重。術(shù)后 12 天拆線,切口 I 期甲級(jí)愈合。醫(yī)囑 3 個(gè)月內(nèi)不得下蹲,避免髕骨病變骨質(zhì)破壞處發(fā)生骨折。術(shù)后 2 周開(kāi)始輔助放療,直線加速器關(guān)節(jié)外照射,總劑量為 20 Gy,每 2 天 1 次 2 Gy,共 10 次,20 天完成。術(shù)后 1 年無(wú)特殊異常,左膝無(wú)疼痛癥狀,膝關(guān)節(jié)活動(dòng)度 -5°~135°,即完全恢復(fù)屈伸活動(dòng)度。
圖 5 髕前皮質(zhì)開(kāi)一 1.5 cm × 1.5 cm 骨窗,腫瘤呈黃棕色,刮除后見(jiàn)瘤壁硬化,髕骨關(guān)節(jié)面?zhèn)裙琴|(zhì)完整,取自體髂骨植骨圖 6 病檢:色素沉著性絨毛結(jié)節(jié)性滑膜炎 (H & E 染色:a, 100×;b,200×)。彌漫的滑膜細(xì)胞增殖,同時(shí)伴有多核巨細(xì)胞、黃瘤細(xì)胞及少量的含鐵血黃素沉積Fig.5 A cortical window of 1.5 cm × 1.5 cm was made over the anteromedial aspect of the patella (a). The tumor was brownish (a) and was scooped out. On the inner side of the cavity there was a sclerotic margin (b). Bone defect in the patella was flled with iliac crest bone graft (c)Fig.6 All tissue samples taken from the resected mass in poplitea, (H & E staining: a, 100 ×; b, 200 ×), arthroscopically from the intra-articular knee joint or from the lesion in the patella were defnitively consistent with the PVNS diagnosis, which was characterized by proliferative cells of the synovial membrane, multinucleated giant cells, lipidladen macrophages and deposits of hemosiderin within a fbrous stroma
PVNS,其中部分病例也稱(chēng)作腱鞘巨細(xì)胞瘤,是少見(jiàn)滑膜組織增生性疾病,可發(fā)生于關(guān)節(jié)、腱鞘、滑囊等任何滑膜組織,其發(fā)病率在百萬(wàn)分之二[1-3]。有學(xué)者在描述關(guān)節(jié)滑膜的絨毛樣、結(jié)節(jié)性的增生病變時(shí),于 1941 年首次提出[4],當(dāng)時(shí)并不能確定這樣的病變是炎性、還是腫瘤性增生,而且此爭(zhēng)議自此之后一直存在,至今此病的真正細(xì)胞來(lái)源并不完全明確[5-6]。
患者多以受累關(guān)節(jié)的腫脹或疼痛就診,部分患者因增生滑膜的突然大量出血,積累于相應(yīng)關(guān)節(jié),引起劇烈疼痛[1-3]。PVNS 最典型的 MRI 特征是:在 T1、T2以及質(zhì)子像上均表現(xiàn)為低信號(hào)的結(jié)節(jié)性腫塊,這是由于含鐵血黃素沉積造成的。由于高度的纖維化,其 MRI 表現(xiàn)可類(lèi)似于軟組織,在 T1WI 上呈中等信號(hào),在 T2加權(quán)像上呈高信號(hào)[1,7]。術(shù)前須與一般的軟組織腫瘤進(jìn)行鑒別,比如穿刺活檢以明確診斷。肉眼所見(jiàn)病變是由絨毛和滑膜皺襞構(gòu)成的團(tuán)塊,呈棕褐色。鏡下所見(jiàn) PVNS 的特點(diǎn)是滑膜表面和滑膜下都有滑膜細(xì)胞增生,可形成絨毛和結(jié)節(jié),高倍鏡下可見(jiàn)彌漫的細(xì)胞 (基質(zhì)) 增殖,同時(shí)伴有成纖維組織、多核巨細(xì)胞、黃瘤細(xì)胞、淋巴細(xì)胞及不等量的含鐵血黃素沉積[1-2]。本例患者術(shù)后病理,髕骨內(nèi)、關(guān)節(jié)內(nèi)滑膜病變,關(guān)節(jié)后包塊具備以上特點(diǎn)。
根據(jù)病變范圍、形態(tài)及進(jìn)展,PVNS 通常粗略分為彌漫型和局限型兩類(lèi)[7-8]。彌漫型好發(fā)于四肢各大關(guān)節(jié),如髖、膝、踝等,其中約 80% 病例見(jiàn)于膝關(guān)節(jié)。局部型常見(jiàn)于手足小關(guān)節(jié)及周?chē)碾烨?。彌漫型病變比局限型多增生活躍,侵襲性更強(qiáng)。假如此例患者膝關(guān)節(jié)內(nèi)后側(cè)病變特點(diǎn),符合彌漫型 PVNS 特點(diǎn)。本例不但在窩內(nèi)存在 PVNS 包塊,其髕骨內(nèi)部還存在一個(gè)類(lèi)似局限型的“孤立”病灶。術(shù)中并未發(fā)現(xiàn)髕骨內(nèi)病變與關(guān)節(jié)內(nèi)病變相連續(xù)的直接證據(jù)。并且,不符合彌漫型 PVNS 的獨(dú)有特征。
文獻(xiàn)報(bào)道發(fā)生于骨組織內(nèi)的局限型的 PNVS 病例非常有限,僅有發(fā)生于椎體、椎弓根、股骨髁等個(gè)別病例[9]。也有腕部、手內(nèi)及足內(nèi)周?chē)浗M織內(nèi)的局限型病灶的少見(jiàn)病例[10]。另外,發(fā)生于膝關(guān)節(jié)內(nèi)連接于前、后交叉韌帶,髕韌帶及髕下脂肪墊的局限性 PVNS 也有報(bào)道[11-13]。在這些病例中,其共同的特點(diǎn)是,病變都鄰近關(guān)節(jié),不難發(fā)現(xiàn)病變與關(guān)節(jié)滑膜間的聯(lián)系。本例患者,術(shù)中見(jiàn)髕骨關(guān)節(jié)面完好,肉眼難以找出髕骨內(nèi)病變與關(guān)節(jié)內(nèi)滑膜病變直接相連的證據(jù)。這一現(xiàn)象,很難解釋為病變自關(guān)節(jié)內(nèi)進(jìn)展,直接蔓延,逐漸破壞骨及軟骨,形成髕骨內(nèi)病變,這樣一個(gè)簡(jiǎn)單的過(guò)程。本例患者髕骨內(nèi)、關(guān)節(jié)內(nèi)滑膜病變,關(guān)節(jié)后包塊及病理均符合 PVNS 診斷。雖然很難直接找出病變之間的組織延續(xù),也不能明確病變發(fā)生發(fā)展的實(shí)際過(guò)程,但是,仍然相信這些不同部位病變的組織來(lái)源相同,也就是說(shuō),髕骨內(nèi)的病變以某種方式來(lái)源于膝關(guān)節(jié)的滑膜組織,這種發(fā)展過(guò)程有待更多類(lèi)似病例的積累進(jìn)行闡釋。此外,本例從癥狀出現(xiàn)到手術(shù),時(shí)間長(zhǎng)達(dá) 3 年,這是解釋髕骨內(nèi)病灶形成與關(guān)節(jié)內(nèi)滑膜病變有關(guān)的惟一線索。
治療膝關(guān)節(jié) PVNS 的具體手術(shù)方法選擇,曾一度存在不同的主張[14-15]。但是,隨著關(guān)節(jié)鏡技術(shù)的發(fā)展,目前已基本形成以下共識(shí)[15-18],采用關(guān)節(jié)鏡下切除關(guān)節(jié)內(nèi)病變,術(shù)后關(guān)節(jié)功能要優(yōu)于開(kāi)放手術(shù),但同時(shí)兩者術(shù)后病變復(fù)發(fā)率相當(dāng);關(guān)節(jié)外及窩病變,需行開(kāi)放手術(shù)輔助切除。本例對(duì)在關(guān)節(jié)鏡不能到達(dá)的關(guān)節(jié)后病變組織,先行開(kāi)放手術(shù)切除。對(duì)髕骨內(nèi)病變,為降低術(shù)后復(fù)發(fā)的風(fēng)險(xiǎn),徹底刮除病變組織后,還特地對(duì)瘤壁進(jìn)行了高速磨鉆磨除,氯化鋅燒灼等處理。術(shù)后隨訪 1 年,未見(jiàn)復(fù)發(fā)。文獻(xiàn)報(bào)道術(shù)后5 年內(nèi)仍存在較高復(fù)發(fā)風(fēng)險(xiǎn)[1,10],故需繼續(xù)密切隨訪。
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(本文編輯:李貴存)
Multifocal pigmented villonodular synovitis arising separately in the articular knee joint and the patella: 1 case report
GAO Ming-xuan, LI Xu-sheng, SHAO Hong-bin, CHEN Yan-fei, CHANG Yan-feng, LI Sheng-gui, HAO Xiaodong. General Hospital of Lanzhou Military Area Command, Lanzhou, Gansu, 730050, PRC
HAO Xiao-dong, Email: xushengli1968@163.com
Objective To describe a rare case of pigmented villonodular synovitis (PVNS) with a nodular lesion in the left patella, diffuse affected synovial tissue in the left knee and an extra-articular mass in the left poplitea. Methods A 37-year-old female with complaints of intermittent left knee pain (3 years) and a painful mass palpated in her left poplitea was analyzed. Results On plain radiograph, an osteolytic lesion was evident in the left patella, ESR: 8 mm / h, CRP: 3.1 mg / L, no obvious abnormal signals. Magnetic resonance imaging (MRI) of left knee showed lesions with same signal intensity coexist both in the inner left patella, left intra-articular knee joint and left extraarticular poplitea. Diagnosis of PVNS was made. Combined with arthroscopic synovectomy, open resection of lesions and iliac crest bone graft for patellar bone defect were performed. The postoperative pathological diagnoses of the resected mass from the poplitea, the affected synovial tissue and the lesion in the patella were consistent with PVNS. The postoperative clinical course was uneventful. No signs of recurrence were found at 1-year follow-up. Conclusions This is the frst report of PVNS occurring both in the inner left patella, left intra-articular knee joint and left extraarticular poplitea.
Synovitis, pigmented villonodular; Giant cell tumors; Patella; Knee joint
10.3969/j.issn.2095-252X.2016.07.017中圖分類(lèi)號(hào):R684
730050 蘭州軍區(qū)蘭州總醫(yī)院骨科
郝曉東,Email: xushengli1968@163.com
2016-03-21)
中國(guó)骨與關(guān)節(jié)雜志2016年7期