髂骨弥漫性腱鞘巨细胞瘤一例报告

吴长坤 姜鑫 刘建永 杨波

. 病例报告 Case report .

髂骨弥漫性腱鞘巨细胞瘤一例报告

吴长坤 姜鑫 刘建永 杨波

巨细胞瘤；腱 T；髂骨；肿瘤

弥漫性腱鞘巨细胞瘤 ( diffuse-type tenosynovial giant cell tumor，D-TGCT ) 起源于腱鞘及滑囊的滑膜细胞，是一种滑膜样单个核细胞破坏样增生，其组织学特性与色素沉着绒毛结节性滑膜炎相同，呈浸润性生长[1]，且复发率较高，易误诊为恶性[2-4]。D-TGCT 病因不明，常见于 20～50岁女性患者，主要累及膝、踝、髋等负重关节，也有少数发生于颞下颌关节、脊柱小关节等部位的报道[5-6]。2016年 11月 28日我科收治 1例髂骨 D-TGCT 患者，现报道如下。

临床资料

患者，女，12岁，因右臀部疼痛 3天入院，疼痛以夜间为主，活动不加重疼痛。体格检查：右臀部较对侧膨隆，局部皮肤正常，皮温略高，可触及一大小约 4cm× 3cm×1cm 肿物，质硬，压痛，推之不动，髋关节活动正常。

影像学检查：术前双侧髋关节正位 X 线片示右侧骶髂关节部见高低混杂信号影 ( 图 1)。

双侧髋关节 CT 平扫示右侧髂骨骶髂关节层面骨质破坏，局部略膨胀，下部关节面皮质破坏中断 ( 图 2)。

双侧髋关节 MR 平扫示 T2WI 冠状位示：右侧髂骨骶髂关节区骨质破坏，边缘清晰，内侧破坏骨皮质，内见混杂长 T2WI 及短 T2WI 信号。T2WI 抑脂冠状位示：右侧髂骨骶髂关节区骨质破坏，病变边缘清晰，内侧局部骨皮质中断，病变内见混杂长 T2WI 及短 T2WI 信号 ( 图 3)。

患者入院后行血、尿常规，乙肝五项，血生化，女性肿瘤标志物，血沉、C 反应蛋白，心电图，胸片等检查，检查结果无明显异常。于入院第 2天行右髂骨细针穿刺活检术，取标本行病理学检查。

病理检查结果：大体：灰红带骨组织一条，总体积约5mm×2mm×2mm，组织切面灰红、灰黄、质韧。CD68多核巨细胞 ( + )，肿瘤细胞由弥漫性增生大单核细胞、小单核细胞、泡沫状组织细胞、多核巨细胞构成；大单核细胞胞质丰富，核偏位，胞质内有假包涵体，被吞噬的含铁血黄素位于大单核细胞胞质假包涵体周围；小单核细胞卵圆形，有核仁。免疫组化：LCA ( 部分＋)、Vimentin (＋)、S-100( 组织细胞＋)、CD68( 组织细胞＋) 、CD38( - )、CD138(－)、Kappa (＋)、Lambda ( 部分＋)、Desmin (－)、P63(－)、CD1ɑ (－) 、Ki-67指数 ( 5% )，病理学诊断：髂骨 D-TGCT ( 图 4)。

全麻后行右髂骨近中线侧切口，逐层暴露至髂骨肿瘤部位，术中见肿瘤位于髂骨外侧皮质受侵蚀变薄，肿瘤侵蚀右侧骶髂关节，瘤体周围形成硬化骨窦，大小约 4cm×2.5cm×1cm，边界不清，形状不规则，质韧。手术沿瘤壁外侧 2mm 完整清除肿瘤组织，用碘酊、无水酒精灭活瘤腔，并用蒸馏水浸泡冲洗后行同种异松质骨颗粒植骨。手术顺利，术后复查 X 线片见瘤腔松质骨颗粒植骨饱满 ( 图 5)。于 2016年 12月 16日康复出院。术后半年( 图 6) 复查髋关节 CT 平扫未见复发及转移。

图1 术前双侧髋关节正位 X 线片示右侧骶髂关节部见高低混杂信号影图2 术前双侧髋关节 CT 平扫示右侧髂骨骶髂关节层面骨质破坏，局部略膨胀，下部关节面皮质破坏中断图3 双侧髋关节 MR 平扫示 T2WI 冠状位示：右侧髂骨骶髂关节区骨质破坏，边缘清晰，内侧破坏骨皮质，内见混杂长 T2WI 及短 T2WI信号。T2WI 抑脂冠状位示：右侧髂骨骶髂关节区骨质破坏，病变边缘清晰，内侧局部骨皮质中断，病变内见混杂长 T2WI 及短 T2WI 信号图4 CD68多核巨细胞 ( + )，肿瘤细胞由弥漫性增生大单核细胞、小单核细胞、泡沫状组织细胞、多核巨细胞构成；大单核细胞胞质丰富，核偏位，胞质内有假包涵体，被吞噬的含铁血黄素位于大单核细胞胞质假包涵体周围；小单核细胞卵圆形，有核仁 ( HE ×100)图5 术后双侧髋关节 X 线片见瘤腔松质骨颗粒植骨饱满图6 右侧髂骨内见肾形低密度影，局部骨质不连续，内见团状高密度灶，右侧骶髂关节欠连续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

讨 论

D-TGCT 可于关节外形成单个或多个弥漫性或浸润性肿块，也可累及毗邻关节。往往隐匿起病，生长缓慢，疼痛并不常见，可表现为患肢轻微疼痛，疼痛可能于活动后加重，休息后减轻，少数患者可于体表扪及包块，关节受累后可出现关节活动受限[2,7]。本例患者右臀部疼痛3天，夜间疼痛较重，疼痛程度未受活动影响，查体于右臀部可扪及包块。

D-TGCT 病因不明，术前诊断困难，临床及影像学检查缺乏特异性，约 50% 的患者 X 线片可发现局灶性软组织内阴影，偶尔有骨溶解、骨膜反应以及病变内钙化等表现，CT 可以更清楚的评价骨破坏的程度和范围；MRI 上T1WI 病灶区表现为与肌组织相近或更高信号，而 T2WI 表现为高低混杂的不均匀信号[8-9]。影像学检查仅作为辅助诊断手段，确诊尚需依赖病理学检查，D-TGCT 主要由单核细胞组成，伴有数量不等的多核巨细胞、泡沫细胞、慢性炎细胞以及含铁血黄素[3-4]。细针穿刺活检，病理报告：肿瘤由多核巨细胞、泡沫细胞和炎症细胞与单核细胞混合组成；诊断为 D-TGCT ( 图 4)。

D-TGCT 呈侵袭性生长，诊断明确后，治疗以手术切除为主，肿瘤侵蚀骨关节者应将瘤组织彻底刮除，切除不彻底者约 40%～50% 复发，复发后可出现恶性肿瘤表现，并发生远处转移。当 D-TGCT 侵犯骨关节，破坏关节软骨，关节间隙变窄时行人工关节置换术效果较好。放疗可用于切除不彻底或术后复发的病例，其临床效果存在争议，且放疗可出现关节僵硬、股骨颈骨折等并发症[2,7-8]。本例行肿瘤完整切除，然后用碘酊、无水酒精灭活瘤腔并蒸馏水浸泡冲洗后行同种异体松质骨颗粒植骨。虽然通常认为 D-TGCT 是良性肿瘤，但其生长呈侵袭性，并有转移表现，因此，在肿瘤切除后应密切随访[5,10]。本例术后半年门诊随访，无局部压痛，双侧骶髂关节部皮温无差异，局部无隆起，骶髂关节活动良好，无受限。骶髂关节 CT平扫示：右侧髂骨内见肾形低密度影，局部骨质不连续，内见团状高密度灶，右侧骶髂关节欠连续。后期疗效尚待随访证实。

综上所述，D-TGCT 病因不明，主要累及膝、踝、髋等负重关节，累及髂骨部位的罕见报道，影像学检查特异性不高，诊断主要依赖病理学检查，明确诊断后应手术切除；放疗及化疗效果不确定，术后复发率较高，需严密随访。

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( 本文编辑：李贵存 )

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 山东，潍坊医学院临床医学院研究生处 ( 吴长坤 )；261641 山东，潍坊市人民医院关节外科 ( 姜鑫、刘建永、杨波 )

杨波，Email: boshandebo@sina.com

2017-04-17)