李志成,馬毅民,閆東,程曉光*
LI Zhi-cheng1,2, MA Yi-min1, YAN Dong1, CHENG Xiao-guang1*
股骨頭軟骨母細胞瘤一例Chondroblastoma of femoral head:a case report
李志成1,2,馬毅民1,閆東1,程曉光1*
LI Zhi-cheng1,2, MA Yi-min1, YAN Dong1, CHENG Xiao-guang1*
病史:患者,女,21歲,22個月前出現(xiàn)左髖關(guān)節(jié)無誘因疼痛,程度可耐受,不伴發(fā)熱、夜間痛、盜汗,未服用藥物。查體見左下肢輕度跛行,左髖關(guān)節(jié)皮膚不紅,未見靜脈曲張,皮溫不高、未觸及腫脹、包塊,局部深壓痛,未及血管雜音,關(guān)節(jié)被動及主動活動可,四肢感覺運動可,肢端血運活動可,病理征未引出。
影像學(xué)表現(xiàn):左髖關(guān)節(jié)正側(cè)位X線片示左側(cè)股骨頭、頸部限局性骨質(zhì)破壞灶,邊界清晰、硬化,病灶內(nèi)密度欠均勻,未見明確軟組織腫塊形成(圖1)。CT表現(xiàn):左側(cè)股骨頭、頸部溶骨性骨質(zhì)破壞,邊界清晰、硬化,病變內(nèi)未見明顯鈣化、成骨改變,未見明確軟組織腫塊突破骨皮質(zhì),增強掃描示病變不均勻強化(圖2)。MRI表現(xiàn):左側(cè)股骨頭、頸部骨破壞,呈不均勻低T1、高T2信號,內(nèi)可見多發(fā)液-液平面,邊緣增厚低信號帶提示骨質(zhì)硬化,增強掃描示病變明顯不均勻強化(圖3)。
手術(shù)記錄:縱行切開股外側(cè)肌,顯露股骨粗隆外側(cè),G型臂引導(dǎo)下開窗,經(jīng)過股骨粗隆刮除股骨頭頸部病變,用高速磨鉆磨除周圍骨質(zhì)直至顯露正常骨,用大量生理鹽水脈沖沖洗后,以同種異體骨股骨頭(同種骨材料,深凍股骨頭)制成異體松質(zhì)骨顆粒植于股骨近端缺損處。
病理:鏡下呈軟骨母細胞瘤結(jié)構(gòu)及動脈瘤樣骨囊腫結(jié)構(gòu),可見增生的軟骨母細胞伴大片狀軟骨樣基質(zhì);亦見單一的纖維性囊壁及纖維細胞、纖維母細胞、少量多核巨細胞,伴出血及含鐵血黃素沉著。病理診斷:軟骨母細胞瘤(圖4~6)。
討論 軟骨母細胞瘤,又稱Codman腫瘤、鈣化性巨細胞瘤等,是一種發(fā)生于骨骼成熟之前的良性病變,占原發(fā)骨腫瘤不到1%。該病好發(fā)于10~25歲,男性多于女性,典型好發(fā)部位為長骨骨骺內(nèi),如肱骨、脛骨及股骨骨骺,發(fā)生于干骺端的病變極少,但可有骨骼發(fā)育成熟后干骺端繼發(fā)受累。此外,少見發(fā)病部位為脊柱椎體[1]和長骨骨皮質(zhì)內(nèi)[2]。臨床表現(xiàn)以關(guān)節(jié)疼痛和活動受限為主,部分可以有關(guān)節(jié)積液[3]。典型的軟骨母細胞瘤X 線平片和CT表現(xiàn)為發(fā)生于骨骺的病變,可跨越骺板、累及干骺端,多呈分葉狀或類圓形溶骨性骨破壞,內(nèi)見斑點狀、條狀、團狀和片絮狀鈣化[4], 或無明顯鈣化灶[5],移行帶窄,可有或無薄
圖1 數(shù)字化X線攝影示左側(cè)股骨頭、頸部限局性骨質(zhì)破壞灶(黑色箭頭),邊界清晰、硬化,病灶內(nèi)密度欠均勻;左側(cè)股骨近端未見明確軟組織腫塊顯示Fig. 1 Digital radiography images show localized bone destruction lesion in the left femoral head and neck(black arrow), with clear, sclerotic boundary, and inhomogeneous density inside; There is no signifcant sign for the existence of soft tissue masses.
圖2 A:CT平掃骨窗示左側(cè)股骨頭、頸部溶骨性骨質(zhì)破壞,邊界清晰、硬化,病變內(nèi)未見明顯鈣化、成骨改變;B:CT平掃軟組織窗示病灶內(nèi)軟組織密度稍欠均勻,未見明確軟組織腫塊突破皮質(zhì);C、D:CT增強掃描軟組織窗示左側(cè)股骨上端病變軟組織成分不均勻強化Fig. 2 A: Routine computed tomography(CT) scan image(in bone window) shows lytic bone destruction lesion in the left femoral head and neck. The lesion has clear, sclerotic margin, and there is no significant calcifcation or ossifcation inside the lesion; B: Routine CT scan image(in soft tissue window) shows inhomogeneous soft tissue density inside the lesion. No signifcant soft tissue mass formed outside the host bone; C, D:Contrast enhancement CT scan images show that the lesion located in the left femoral head and neck was inhomogeneously enhanced.
圖3 A:MR T1WI平掃。左側(cè)股骨頭、頸部不均勻長T1信號灶,邊緣增厚低信號帶提示骨質(zhì)硬化;B、C:MR T2WI平掃(B)/壓脂(C)。病灶內(nèi)見不均勻長T2信號灶,多發(fā)液-液平面;D:MR增強掃描示病變明顯不均勻強化Fig. 3 A: Routine magnetic resonance(MR) T1WI image shows inhomogeneous low intensity signal located in left femoral head and neck,the thickened low intensity signal band surrounding the lesion suggests bone sclerosis; B, C: Routine MR T2WI image(B) and T2 SPAIR fat-suppressed image(C) show inhomogeneous high intensity signal inside the lesion, with multiple fuid-fuid level formed; D: Contrast enhancement MR scan image shows that the lesion is inhomogeneously enhanced, signifcantly.
圖4 動脈瘤樣骨囊腫結(jié)構(gòu):單一的纖維性囊壁及纖維細胞、纖維母細胞、少量多核巨細胞構(gòu)成的囊壁結(jié)構(gòu)(HE ×10)Fig. 4 Structure of aneurysmal bone cyst with fibrous cystic walls and walls formed by fbroblasts and polykaryocytes,is shown above(HE ×10).
圖5 軟骨母細胞瘤結(jié)構(gòu):增生的軟骨母細胞伴大片狀軟骨樣基質(zhì),少量多核巨細胞(HE ×20)Fig. 5 Image shows chondroblastoma structure: proliferated chondrocytes with large amounts of cartilage-like matrix, and a small number of polykaryocytes(HE ×20).
圖6 動脈瘤樣骨囊腫高倍鏡觀察:可見纖維細胞、纖維母細胞、少量多核巨細胞構(gòu)成,伴出血及含鐵血黃素沉著(HE ×20)Fig. 6 Structure of aneurysmal bone cyst under high power microscopic view: a large amount of fiber cells, fibroblasts and a small number of polykaryocytes, with hemorrhage and hemosiderosis occurred(HE ×20).
侵襲性軟骨母細胞瘤可穿過生長板侵入干骺端或關(guān)節(jié)腔,出現(xiàn)較厚的骨膜反應(yīng)和軟組織腫塊[8]。
此例發(fā)生于股骨頭頸部,相對少見,易誤診。當非長骨骨骺部位病變具有軟骨母細胞瘤的影像征象、且病變位于少見部位(長骨骨骺以外相對好發(fā)部位)時,應(yīng)考慮到本病的可能[10]。X線平片對診斷具有一定價值;CT克服了常規(guī)X線平片組織結(jié)構(gòu)重疊的缺點,對病變的破壞、鈣化及灶周硬化邊的顯示較X線清晰、敏感;MR能充分顯示病灶成分、瘤周骨髓水腫、周圍軟組織情況,對合并有ABC的軟骨母細胞瘤的診斷方面具有更高價值[6]。
鑒別診斷:(1)骨巨細胞瘤:骨巨細胞瘤多位于干骺端,呈偏心性囊性擴張改變,可侵蝕骨皮質(zhì),或從皮質(zhì)呈氣球狀凸出,內(nèi)可見粗厚骨嵴,近骨干側(cè)皮質(zhì)顯著增厚。MRI上表現(xiàn)為:T1WI低信號,T2壓脂序列上明顯高信號,CT/MRI增強掃描強化明顯,MRI可以很好顯示腫瘤是否侵及鄰近關(guān)節(jié)或周圍軟組織[10]。(2)內(nèi)生軟骨瘤(單發(fā)):內(nèi)生軟骨瘤發(fā)病高峰年齡段為20~40歲,多位于長管狀骨干骺端,腫瘤形態(tài)不規(guī)則,缺乏明確的硬化邊、骨膜增生和軟組織腫脹征象[6]。(3)透明細胞型軟骨肉瘤:多見于年長人群,病變較大且比軟骨母細胞瘤更易侵及骨骺以外的區(qū)域,T2WI多呈顯著高信號,低信號成分少見,多缺乏硬化邊[10]。
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27 Jun 2016, Accepted 31 Jul 2016
Femoral head; Chondroblastoma; Magnetic resonance imaging
股骨頭;軟骨母細胞瘤;磁共振成像
北京市優(yōu)秀人才培養(yǎng)資助項目(編號:2015000021467G177);北京市衛(wèi)生系統(tǒng)高層次衛(wèi)生技術(shù)人才培養(yǎng)項目(編號:2009-2-03)
1.北京積水潭醫(yī)院放射科,北京 100035
2.大理白族自治州中醫(yī)醫(yī)院放射科,大理 671000
1Department of Radiology, Beijing Jishuitan Hospital, Beijing 100035, China
2Department of Radiology, Dali Bai Autonomous Prefecture Hospital of Traditional Chinese Medicine, Dali 671000, China
程曉光,E-mail:xiao65@263.net
*Correspondence to: Cheng XG, E-mail:xiao65@263.net液-液平面[7]。MR圖像上,由于病變富含軟骨基質(zhì),而在T1WI上呈低信號,T2WI上呈不均勻中等信號[8],液-液平面顯示較CT更加清晰。MR亦層硬化緣,通常無膨脹性改變[6],而出現(xiàn)骨膨脹就應(yīng)考慮合并動脈瘤樣骨囊腫(aneurysmal bone cyst,ABC)[3],合并ABC的病變可在CT及MR上見到可清晰顯示骨髓和周圍軟組織的水腫[9]。
2016-06-27 接受日期:2016-07-31
R445.2;R738.3
B
10. 12015/issn.1674-8034.2016.08.012
ACKNOWLEDGMENTS This work was part of Beijing Talents Fund (No. 2015000021467G177); High level health technical personnel training project of Beijing health system (No. 2009-2-03).