髂骨彌漫性腱鞘巨細(xì)胞瘤一例報告

吳長坤 姜鑫 劉建永 楊波

. 病例報告 Case report .

髂骨彌漫性腱鞘巨細(xì)胞瘤一例報告

吳長坤 姜鑫 劉建永 楊波

巨細(xì)胞瘤；腱 T；髂骨；腫瘤

彌漫性腱鞘巨細(xì)胞瘤 ( diffuse-type tenosynovial giant cell tumor，D-TGCT ) 起源于腱鞘及滑囊的滑膜細(xì)胞，是一種滑膜樣單個核細(xì)胞破壞樣增生，其組織學(xué)特性與色素沉著絨毛結(jié)節(jié)性滑膜炎相同，呈浸潤性生長[1]，且復(fù)發(fā)率較高，易誤診為惡性[2-4]。D-TGCT 病因不明，常見于 20～50歲女性患者，主要累及膝、踝、髖等負(fù)重關(guān)節(jié)，也有少數(shù)發(fā)生于顳下頜關(guān)節(jié)、脊柱小關(guān)節(jié)等部位的報道[5-6]。2016年 11月 28日我科收治 1例髂骨 D-TGCT 患者，現(xiàn)報道如下。

臨床資料

患者，女，12歲，因右臀部疼痛 3天入院，疼痛以夜間為主，活動不加重疼痛。體格檢查：右臀部較對側(cè)膨隆，局部皮膚正常，皮溫略高，可觸及一大小約 4cm× 3cm×1cm 腫物，質(zhì)硬，壓痛，推之不動，髖關(guān)節(jié)活動正常。

影像學(xué)檢查：術(shù)前雙側(cè)髖關(guān)節(jié)正位 X 線片示右側(cè)骶髂關(guān)節(jié)部見高低混雜信號影 ( 圖 1)。

雙側(cè)髖關(guān)節(jié) CT 平掃示右側(cè)髂骨骶髂關(guān)節(jié)層面骨質(zhì)破壞，局部略膨脹，下部關(guān)節(jié)面皮質(zhì)破壞中斷 ( 圖 2)。

雙側(cè)髖關(guān)節(jié) MR 平掃示 T2WI 冠狀位示：右側(cè)髂骨骶髂關(guān)節(jié)區(qū)骨質(zhì)破壞，邊緣清晰，內(nèi)側(cè)破壞骨皮質(zhì)，內(nèi)見混雜長 T2WI 及短 T2WI 信號。T2WI 抑脂冠狀位示：右側(cè)髂骨骶髂關(guān)節(jié)區(qū)骨質(zhì)破壞，病變邊緣清晰，內(nèi)側(cè)局部骨皮質(zhì)中斷，病變內(nèi)見混雜長 T2WI 及短 T2WI 信號 ( 圖 3)。

患者入院后行血、尿常規(guī)，乙肝五項，血生化，女性腫瘤標(biāo)志物，血沉、C 反應(yīng)蛋白，心電圖，胸片等檢查，檢查結(jié)果無明顯異常。于入院第 2天行右髂骨細(xì)針穿刺活檢術(shù)，取標(biāo)本行病理學(xué)檢查。

病理檢查結(jié)果：大體：灰紅帶骨組織一條，總體積約5mm×2mm×2mm，組織切面灰紅、灰黃、質(zhì)韌。CD68多核巨細(xì)胞 ( + )，腫瘤細(xì)胞由彌漫性增生大單核細(xì)胞、小單核細(xì)胞、泡沫狀組織細(xì)胞、多核巨細(xì)胞構(gòu)成；大單核細(xì)胞胞質(zhì)豐富，核偏位，胞質(zhì)內(nèi)有假包涵體，被吞噬的含鐵血黃素位于大單核細(xì)胞胞質(zhì)假包涵體周圍；小單核細(xì)胞卵圓形，有核仁。免疫組化：LCA ( 部分＋)、Vimentin (＋)、S-100( 組織細(xì)胞＋)、CD68( 組織細(xì)胞＋) 、CD38( - )、CD138(－)、Kappa (＋)、Lambda ( 部分＋)、Desmin (－)、P63(－)、CD1ɑ (－) 、Ki-67指數(shù) ( 5% )，病理學(xué)診斷：髂骨 D-TGCT ( 圖 4)。

全麻后行右髂骨近中線側(cè)切口，逐層暴露至髂骨腫瘤部位，術(shù)中見腫瘤位于髂骨外側(cè)皮質(zhì)受侵蝕變薄，腫瘤侵蝕右側(cè)骶髂關(guān)節(jié)，瘤體周圍形成硬化骨竇，大小約 4cm×2.5cm×1cm，邊界不清，形狀不規(guī)則，質(zhì)韌。手術(shù)沿瘤壁外側(cè) 2mm 完整清除腫瘤組織，用碘酊、無水酒精滅活瘤腔，并用蒸餾水浸泡沖洗后行同種異松質(zhì)骨顆粒植骨。手術(shù)順利，術(shù)后復(fù)查 X 線片見瘤腔松質(zhì)骨顆粒植骨飽滿 ( 圖 5)。于 2016年 12月 16日康復(fù)出院。術(shù)后半年( 圖 6) 復(fù)查髖關(guān)節(jié) CT 平掃未見復(fù)發(fā)及轉(zhuǎn)移。

圖1 術(shù)前雙側(cè)髖關(guān)節(jié)正位 X 線片示右側(cè)骶髂關(guān)節(jié)部見高低混雜信號影圖2 術(shù)前雙側(cè)髖關(guān)節(jié) CT 平掃示右側(cè)髂骨骶髂關(guān)節(jié)層面骨質(zhì)破壞，局部略膨脹，下部關(guān)節(jié)面皮質(zhì)破壞中斷圖3 雙側(cè)髖關(guān)節(jié) MR 平掃示 T2WI 冠狀位示：右側(cè)髂骨骶髂關(guān)節(jié)區(qū)骨質(zhì)破壞，邊緣清晰，內(nèi)側(cè)破壞骨皮質(zhì)，內(nèi)見混雜長 T2WI 及短 T2WI信號。T2WI 抑脂冠狀位示：右側(cè)髂骨骶髂關(guān)節(jié)區(qū)骨質(zhì)破壞，病變邊緣清晰，內(nèi)側(cè)局部骨皮質(zhì)中斷，病變內(nèi)見混雜長 T2WI 及短 T2WI 信號圖4 CD68多核巨細(xì)胞 ( + )，腫瘤細(xì)胞由彌漫性增生大單核細(xì)胞、小單核細(xì)胞、泡沫狀組織細(xì)胞、多核巨細(xì)胞構(gòu)成；大單核細(xì)胞胞質(zhì)豐富，核偏位，胞質(zhì)內(nèi)有假包涵體，被吞噬的含鐵血黃素位于大單核細(xì)胞胞質(zhì)假包涵體周圍；小單核細(xì)胞卵圓形，有核仁 ( HE ×100)圖5 術(shù)后雙側(cè)髖關(guān)節(jié) X 線片見瘤腔松質(zhì)骨顆粒植骨飽滿圖6 右側(cè)髂骨內(nèi)見腎形低密度影，局部骨質(zhì)不連續(xù)，內(nèi)見團狀高密度灶，右側(cè)骶髂關(guān)節(jié)欠連續(xù)Fig.1Preoperative bilateral hip anteroposterior X-ray showed high and low mixed signals of the right sacroiliac jointFig.2Preoperative bilateral hip joint CT scan showed that the right sacroiliac joint layer was destructed, slight local inf l ation. The lower articular surface cortex was destructedFig.3Coronal T2WI showed the bone destruction in the right iliac sacroiliac joint, clear boundary, medial destruction of the cortical bone, mixed long T2WI and short T2WI signals. Coronal fat-bearing T2WI showed bone destruction in the right iliac sacroiliac joint, clear boundary, local destruction of the cortical bone, mixed long T2WI and short T2WI signalsFig.4HE staining: CD68multinucleated giant cells ( + ), tumor cells consisted diffuse hyperplastic mononuclear cells, small mononuclear cells, foam cells, multinucleated giant cells. Large mononuclear cells were abundant in the cytoplasm, and the nuclear translocation was located in the cytoplasmic fake inclusion bodies. Small mononuclear cells were oval and had nucleolus ( HE ×100)Fig.5Postoperative bilateral hip X-ray fi lm showed well bone graftingFig.6In the right ilium, there was a low density of renal shape. The local bone mass was discontinuous and there was a group of high density foci. The right sacroiliac joint was not continuous

討 論

D-TGCT 可于關(guān)節(jié)外形成單個或多個彌漫性或浸潤性腫塊，也可累及毗鄰關(guān)節(jié)。往往隱匿起病，生長緩慢，疼痛并不常見，可表現(xiàn)為患肢輕微疼痛，疼痛可能于活動后加重，休息后減輕，少數(shù)患者可于體表捫及包塊，關(guān)節(jié)受累后可出現(xiàn)關(guān)節(jié)活動受限[2,7]。本例患者右臀部疼痛3天，夜間疼痛較重，疼痛程度未受活動影響，查體于右臀部可捫及包塊。

D-TGCT 病因不明，術(shù)前診斷困難，臨床及影像學(xué)檢查缺乏特異性，約 50% 的患者 X 線片可發(fā)現(xiàn)局灶性軟組織內(nèi)陰影，偶爾有骨溶解、骨膜反應(yīng)以及病變內(nèi)鈣化等表現(xiàn)，CT 可以更清楚的評價骨破壞的程度和范圍；MRI 上T1WI 病灶區(qū)表現(xiàn)為與肌組織相近或更高信號，而 T2WI 表現(xiàn)為高低混雜的不均勻信號[8-9]。影像學(xué)檢查僅作為輔助診斷手段，確診尚需依賴病理學(xué)檢查，D-TGCT 主要由單核細(xì)胞組成，伴有數(shù)量不等的多核巨細(xì)胞、泡沫細(xì)胞、慢性炎細(xì)胞以及含鐵血黃素[3-4]。細(xì)針穿刺活檢，病理報告：腫瘤由多核巨細(xì)胞、泡沫細(xì)胞和炎癥細(xì)胞與單核細(xì)胞混合組成；診斷為 D-TGCT ( 圖 4)。

D-TGCT 呈侵襲性生長，診斷明確后，治療以手術(shù)切除為主，腫瘤侵蝕骨關(guān)節(jié)者應(yīng)將瘤組織徹底刮除，切除不徹底者約 40%～50% 復(fù)發(fā)，復(fù)發(fā)后可出現(xiàn)惡性腫瘤表現(xiàn)，并發(fā)生遠處轉(zhuǎn)移。當(dāng) D-TGCT 侵犯骨關(guān)節(jié)，破壞關(guān)節(jié)軟骨，關(guān)節(jié)間隙變窄時行人工關(guān)節(jié)置換術(shù)效果較好。放療可用于切除不徹底或術(shù)后復(fù)發(fā)的病例，其臨床效果存在爭議，且放療可出現(xiàn)關(guān)節(jié)僵硬、股骨頸骨折等并發(fā)癥[2,7-8]。本例行腫瘤完整切除，然后用碘酊、無水酒精滅活瘤腔并蒸餾水浸泡沖洗后行同種異體松質(zhì)骨顆粒植骨。雖然通常認(rèn)為 D-TGCT 是良性腫瘤，但其生長呈侵襲性，并有轉(zhuǎn)移表現(xiàn)，因此，在腫瘤切除后應(yīng)密切隨訪[5,10]。本例術(shù)后半年門診隨訪，無局部壓痛，雙側(cè)骶髂關(guān)節(jié)部皮溫?zé)o差異，局部無隆起，骶髂關(guān)節(jié)活動良好，無受限。骶髂關(guān)節(jié) CT平掃示：右側(cè)髂骨內(nèi)見腎形低密度影，局部骨質(zhì)不連續(xù)，內(nèi)見團狀高密度灶，右側(cè)骶髂關(guān)節(jié)欠連續(xù)。后期療效尚待隨訪證實。

綜上所述，D-TGCT 病因不明，主要累及膝、踝、髖等負(fù)重關(guān)節(jié)，累及髂骨部位的罕見報道，影像學(xué)檢查特異性不高，診斷主要依賴病理學(xué)檢查，明確診斷后應(yīng)手術(shù)切除；放療及化療效果不確定，術(shù)后復(fù)發(fā)率較高，需嚴(yán)密隨訪。

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( 本文編輯：李貴存 )

Giant cell tumor of the diffuse tendon sheath: 1case report

WU Chang-kun, JIANG Xin, LIU Jian-yong, YANG Bo.
School of Postgraduate, School of Clinical Medicine, Weifang Medical University, Weifang, Shandong, 261041, China Corresponding author: YANG Bo, Email: boshandebo@sina.com

Objective To analyze the epidemiology, diagnosis and treatment of the giant cell tumor of the diffuse tendon sheath ( D-TGCT ). Methods A rare clinical study of iliac bone D-TGCT was retrospectively analyzed and the foreign literatures in recent years were reviewed. Results A 12-year-old female with 3days’ right hip pain was admitted to the hospital. After imaging and preoperative examinations, the right iliac bone needle biopsy was performed on the right side. Pathological diagnosis: iliac D-TGCT. Cancellous bone allograft grafting was applied in the tumor cavity. During the operation, it was found that tumors were near the right sacroiliac joint, soft bone cortex, inward destruction of the sacroiliac joint, tenacious sclerotic bone antrum around the iliac bone, unclear boundary, irregular shape. Tumors were removed 2mm against the wall. The tumor cavity was inactivated by iodine and ethanol, and rinsed with distilled water. Allogeneic cancellous bone grafting was conducted. No recurrence or metastasis occurred in the 6months’ follow-up. Conclusions D-TGCT involving the iliac bone is rare. The specif i city of imaging is not high and the diagnosis mainly depends on pathology and immunohistochemistry. Radiotherapy and chemotherapy effects are uncertain. Postoperative recurrence rate is high and the patient is in close follow-up.

Giant cell tumors; Tendons; Ilium; Neoplasms

10.3969/j.issn.2095-252X.2017.08.016

R738.1

261041 山東，濰坊醫(yī)學(xué)院臨床醫(yī)學(xué)院研究生處 ( 吳長坤 )；261641 山東，濰坊市人民醫(yī)院關(guān)節(jié)外科 ( 姜鑫、劉建永、楊波 )

楊波，Email: boshandebo@sina.com

2017-04-17)