21例眼眶朗格汉斯细胞组织细胞增生症的临床研究

doi:10.3877/cma.j.issn.2095-2007.2020.03.003

目的

探讨眼眶朗格汉斯细胞组织细胞增生症(LCH)的临床特征。

方法

回顾性病例分析。收集2013年1月至2015年6月于中国人民解放军总医院第三医学中心眼眶研究所确诊21例(21只眼)眼眶LCH患者的临床病历资料。其中，男性14例(14只眼)，女性7例(7只眼)；年龄1~51岁，平均年龄(7.4±10.0)岁。检查并记录所有患者的年龄、性别、临床表现、病灶位置、影像学、病理组织学、治疗及预后的结果。临床资料使用频数和百分比进行描述。

结果

所有患者主要临床表现为额部或颧面部肿块，部分患者伴有炎症表现，眼球突出。病变位于右眼眶者8例(8只眼)，占38.10%(8/21)；左眼眶者13例(13只眼)，占61.90%(13/21)；眼眶外上壁交界处者6例(6只眼)，占28.57%(6/21)；外侧壁者5例(5只眼)，占23.81%(5/21)；上壁者9例(9只眼)，占42.56%(9/21)；外下壁交界处者1例(1只眼)，占4.76%(1/21)。电子计算机断层扫描检查发现所有患者有溶骨性骨质破坏，磁共振成像有17例(17只眼)表现为T1WI中信号，T2WI中高混杂信号，增强后多呈不均匀强化，占80.95%(17/21)；部分肿瘤内部可见不规则增强区。病理组织学检查发现了典型的朗格汉斯细胞，且伴有出血。免疫组织化学检查发现S-100蛋白染色在20例(20只眼)中均可见肿瘤细胞浆和细胞膜呈阳性表达；Ki-67抗原阳性者仅1例(1只眼)，占4.76%(1/21)。21例(21只眼)均行手术治疗，切除的肿瘤体积大小不一，形态不规则，无纤维性包膜。随访3个月至1年，仅1例复发，占4.76%(1/21)。

结论

眼眶LCH以眶周软组织肿块或眼球突出为主要表现，影像学检查是重要的诊断方法，确诊需结合免疫组织化学检查。手术切除是首选的治疗方法，眼眶LCH单系统受累患者预后较好，但应密切观察，长期随访。

关键词: 眼眶, 组织细胞增生症, 朗格汉斯细胞, 诊断, 预后

Objective

To explore the clinical features of orbital Langerhans cell histiocytosis (LCH).

Methods

Retrospective cases series. Clinical data of 21 cases (21 eyes) patients with orbital LCH in the Orbital Research Institute of the 3rd Medical Center of the People′s Liberation Army Hospital from January 2013 to June 2015 were selected. Among of them, there were 14 males (14 eyes) and 7 females (7 eyes), aged from 1 to 51 years-old with the average age of (7.4±10.0) years-old. Age, sex, clinical presentation, lesion location, imaging findings, and histopathological results, treatment method and prognosis were recorded. The clinical data were described by using frequency and percentage.

Results

All patients mainly showed lumps at frontal part or cheek, inflammatory infiltration and protopsis. Among of them, 8 cases (8 eyes) accouting for 38.10% (8/21) were found lesions at the right orbit, 13 cases (13 eyes) accouting for 61.90% (13/21) at the left orbit, 6 cases (6 eyes) accouting for 28.57% (6/21) at the upper exterior boundary of orbit, 5 cases (5 eyes) accouting for 23.81% (5/21) at lateral wall, 9 cases (9 eyes) accouting for 42.56% (9/21) at upper wall, and 1 case (1 eye) accouting for 4.76%(1/21) at lower exterior boundary. The osteolytic bone destruction was founded by computed tomography, T1WI middle-intensity signals and T2WI middle-high-intensity mixed signals was founded by magnetic resonance imaging in 17 cases (17 eyes) accouting for 80.95% (17/21) . These signals were unevenly strengthened after the enhancement with irregular strengthened zone of tumor interior. Typical LCH with hemorrhage was found by histopathological examination. 20 cases (20 eyes) were showed tumor cytoplasm and positive cytomembrane after S-100 dyeing. Only one case (one eye) was showed Ki-67 antigen positive rate accouting for 4.76% (1/21). 21 cases (21 eyes) were treated by tumorectomy. The excision tumors had uneven size and shapes without fibroid diolame. After 3 months to 1 year follow-up visits, only one case accouting for 4.76% (1/21) showed recurrence.

Conclusions

Orbital LCH was manifested by orbital lumps or exophthalmos. Imaging examination was an important diagnosis method, and accurate diagnosis was based on immunohistochemical examination. Excision was the first choice in the treatment. Patients with orbital single-system LCH have good prognosis, but follow-up analysis is needed.

Key words: Orbit, Histiocytosis, Langerhans cells, Diagnosis, Prognosis

图1 患者眼眶电子计算机断层扫描和磁共振成像　图A和B示患者左眶电子计算机断层扫描成像图，可见左侧额骨不规则破坏和缺失，左眶上方有不规则软组织密度影，与颅内沟通；图C~E示患者左眶磁共振成像图，可见颅顶及颅底不规则占位，T1中信号，T2中高信号，内有不规则增强区；图F示患者右眶电子计算机断层扫描成像图，可见外侧壁骨侵蚀，呈虫噬样改变，周围软组织肿胀，边界不清；图G示患者右眶磁共振成像图，可见右眶外侧占位病变，眶外壁局部骨侵蚀缺损，周围软组织肿胀，边界不清

图2 眼眶朗格汉斯细胞组织细胞增生症肿瘤组织外观彩色照像与光学显微镜下病理组织细胞的显微结构图　图A示刮除的肿瘤标本，形态不规则，呈胶冻样，灰红色，无包膜；图B和图C示眼眶朗格汉斯细胞组织细胞(苏木精-伊红染色)。其中，图B中可见片状肿瘤细胞，其周围散在分布嗜酸性粒细胞(细胞小，胞浆红)和多核巨细胞(左下角)(×200)；图C中可见肿瘤细胞体积大，呈圆形、椭圆形和多角形，核染色质较少，核呈圆形、卵圆形及肾形，核仁明显，胞浆丰富呈淡红色，左上角见多核巨细胞(×400)；图D示眼眶朗格汉斯细胞组织细胞S-100蛋白(免疫组织化学染色)，可见肿瘤细胞表达S-100抗体，细胞核和胞浆呈棕黄色(×400)；图E示眼眶朗格汉斯细胞组织细胞Ki-67抗原(免疫组织化学染色)，可见少数肿瘤细胞表达Ki-67抗体，细胞核呈棕色(细胞核大)(×200)

[1]	Hidayat AA, Mafee MF, Laver NV, et al. Langerhans cell histiocytosis and juvenile Xanthogranuloma of the orbit: elinicopathologie, CT and MRI features[J]. Radiol Clin North Am, 1998, 36(6): 1229-1240.
[2]	Lichtenstein L. Histocytosis X: Integration of eosinphilic granuloma of bone，"Letter-Swie disease" ，and "Schuller-Christian disease" an related manifestation of a single nosologic entity[J]. Arch Pathol, 1953, 56(1): 84-102.
[3]	Herwig MC, Wojno T, Zhang Q, et al. Langerhans cell histiocytosis of the orbit: five Clinicopathologic cases and review of the literature [J]. Surv Ophthalmol, 2013, 58(4): 330-340.
[4]	Bhanage AB, Katkar AD, Ghate PS. Langerhans cell histiocytosis with presentation as orbital disease [J]. J Pediatr Neurosci, 2015, 10(2): 162-165.
[5]	Anton M, Holousová M, Rehurek J, et al. Histiocytosis X and the orbit in children[J]. Cesk Oftalmol, 1992, 48(3): 176-180.
[6]	Cochrane LA, Prince M, Clarke K．Langerhans cell histiocytosis in the paediatric population：Presentation and treatment of head and neck manifestations[J]. J Otolaryngol, 2003, 32(1): 33-37.
[7]	Boston M, Derkay CS. Langerhans′cell histiocytosis of the temporal bone and skull base [J]. Am J Otolaryngol, 2002, 23(4): 246-248.
[8]	Vosoghi H, Rodriguez-Galindo C, Wilson MW.Orbital involve-ment in Langerhans cell histiocytesis [J]. Ophthal Plast Recon-str Surg, 2009, 25(6): 430-433.
[9]	Willman CL. Detection of clonal histiocytes in Langerhans cell histiocytosis: biology and clinical significance [J]. Br J Cancer Suppl, 1994，23: S29-S33.
[10]	罗清礼. 组织细胞增多症X[M]. 北京：人民卫生出版社，2005：1047-1051.
[11]	Yashoda-Devi B, Rakesh N. Agarwal M．Langerhans cell histiocytosis wioral manifestations：a rare and unusual case report[J]．J Clin Exp Dent，2012, 4(4): e252-e255.
[12]	Moore AT, ritchard J, Taylor DS. Histiocytosis X: an ophthalmological review[J]. Br J Ophthalmol, 1985, 69(1): 7-14.
[13]	Margo CE, Goldman DR. Langerhans cell histiocytosis[J]. Sury Phthalmol, 2008, 53(4): 332-358.
[14]	Chen HC, Shen WC, Chou DY, et al. Langerhans cell histocytosis of the skull complicated with an epidural hematona[J]. AJNR, 2002, 23(3): 493-495.
[15]	汤静燕，王耀平. 组织细胞增生症[J]. 临床儿科杂志，2002，20(7):445-447.
[16]	刘彤华，李维华. 骨和关节[M]. 北京：人民卫生出版社，1994：817-820.
[17]	Woo KI.Harris GJ.Eosinophilic granuloma of the orbit：understanding the paradox of aggressive destruction responsive to minimal intervention[J]. Ophthalmic Plastic & Reconstructive Sugery, 2003, 19(6): 429-439.
[18]	Azouz EM, Saigal G, Rodriguez MM, et al. Langerhans′cell histiocytosis: pathology, imaging and treatment of skeletal involvement[J]. Pediatr Radiol, 2005, 35(2): 103-115.
[19]	李凤鸣. 眼科全书(上册)[M]. 北京：人民卫生出版社，1996：1236-1237.
[20]	周晓冬，宋国祥，何颜津. 眼眶组织细胞增生症X临床分析[J]. 中华眼科杂志，2003，39(11):63-67.
[21]	肖利华. 现代眼眶病诊断学[M]. 北京：北京科学技术出版社，2006：406-408.
[22]	Howarth DM, Gilchrist GS, Mullan BP, et al. Langerhans cell histiosytosis diagnosis, natural history, management, and outcome[J]. Cancer, 1999, 85(10): 2278-2290.
[23]	Wu C, Li K, Hei Y, et al. MR imaging features of orbital langerhans cell histiocytosis[J]. BMC Ophthalmology, 2019, 19(1): 263-273.
[24]	杨本涛，王振常，鲜军舫，等. 颞骨朗格尔罕斯细胞组织细胞增生症的CT和MRI诊断[J]. 中华放射学杂志，2002，36(3):254-257.
[25]	张斯佳，皇甫幼田，弓莉. 颅骨朗格罕斯细胞组织细胞增生症的CT和MRI诊断[J]．临床放射学杂，2010，29(3):320-322.
[26]	傅筱敏，韩本谊. 颅骨嗜酸性肉芽肿CT和MRI诊断[J]. 临床放射学杂志，2007，26(3):248-250.
[27]	Sieni E, Barba C, Mortilla M, et al. Early diagnosis and monitoring of neurodegenerative Langerhans cell histiocytosis [J]. PLoS One, 2015, 10(7):316-335.
[28]	Das JK, Soibam R, Tiwary BK, et al. Orbital manifestations of Langerhans cell histiocytosis: a report of three cases [J]. Oman J Ophthalmol, 2009, 2(3): 137-140.
[29]	Shetty SB, Mehta C. Langerhans cell histiocytosis of the orbit [J]. Indian J Ophthalmol, 2001, 49(4): 267-268.
[30]	程虹，金木兰，李增山，等. 软组织与骨肿瘤病理学和遗传学[M]. 北京：人民卫生出版社，2006：406-407.
[31]	Addenan M, May CM, Hooi TK, et al. A rare case of solitary unifocal Langerhans cell histiocytosis with orbital extension: diagnostic dilemma [J]. Oman J Ophthalmol, 2018，11(3): 284-287.
[32]	Vazquez E, Lucaya J, Castellote , et al. Neuroimaging in pediatric leukemia and lymphoma: differential diagnosis[J]. Radiographics, 2002, 22(6): 1411-1428.
[33]	宋国祥. 眼眶病学[M]．北京：人民卫生出版社，1999：312-315.

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Clinical features of 21 patients with orbital Langerhans cell histiocytosis

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Clinical features of 21 patients with orbital Langerhans cell histiocytosis