Unusual presentations of pleomorphic adenoma and adenoid cystic carcinoma of the lacrimal gland.

PURPOSE: To report two atypical cases of pleomorphic adenoma and adenoid cystic tumours of the lacrimal gland. METHODS: Two case reports are presented. The first is of a 65-year-old female with a long history of right hypoglobus with sudden recent worsening. Computed tomography (CT) showed a round, well-defined lesion in the fossa for the lacrimal gland with an anterior hypodense extension suggestive of possible malignancy in a pleomorphic adenoma. The tumour in the second case, a 35-year-old male, was diagnosed after presentation following a relatively minor periorbital injury. The smooth rounded mass on CT scan was suggestive of a benign lacrimal gland tumour. RESULTS: The lesion in case 1 was excised with a diagnosis of haemorrhage within a pleomorphic adenoma. The lesion in case 2 was excised with a diagnosis of adenoid cystic carcinoma of the lacrimal gland with pseudocapsule. CONCLUSIONS: Haemorrhagic cyst developing beneath the pseudocapsule of a pleomorphic adenoma should be considered in the differential diagnosis of secondary development of malignancy in a pleomorphic adenoma. Adenoid cystic tumours of the lacrimal gland can present with a pseudocapsule.     

复发性泪腺区肿瘤25例临床病理分析

目的：探讨复发性泪腺区肿瘤的临床特征和组织病理学类型。方法：回顾性研究。收集2004-01/2014-02在我科确诊的25例复发性泪腺区肿瘤患者的临床和病理资料,对其临床表现、复发情况、组织病理学分型及预后情况进行分析。结果：所选25例患者中21例为初次复发,1例为3次复发,3例为2次复发。最后一次手术到最近一次复发间隔1mo~28a(中位数1a)。复发后在我院手术时的年龄17.5~70(平均47.9)岁。组织病理检查：多形性腺瘤4例,多形性腺瘤恶变2例,腺样囊性癌10例,上皮-肌上皮癌3例,恶性多形性腺瘤3例,腺癌1例,间叶组织肿瘤1例,黏膜相关淋巴组织淋巴瘤1例。结论：泪腺区复发性肿瘤种类繁多,以腺样囊性癌最多,其次是多形性腺瘤;恶性肿瘤复发率高;多形性腺瘤初次手术完整的切除,对预防术后复发和恶变至关重要。同时早期及长期的随访,对发现肿瘤复发,也非常重要。     

Pleomorphic adenoma of the lacrimal gland in a nine-year-old child

A case of a 9-year-old child with pleomorphic adenoma of the lacrimal gland is described. This tumour is usually found in adults; cases younger than 10 years of age have proved to be extremely rare. Because of its age distribution, pleomorphic adenoma of the lacrimal gland is usually overlooked as a possible cause of unilateral proptosis in children. Also, the history of disease provided by the family may be incorrect, possibly leading to misdiagnosis by the ophthalmologist.     

Pleomorphic adenomas of the lacrimal gland: a clinicopathological analysis

PURPOSE: To analyse retrospectively 32 cases of pleomorphic adenoma of the lacrimal gland diagnosed over a 10-year period in a tertiary referral eye centre from North India. METHODS: The clinical features of 32 cases of pleomorphic adenomas diagnosed between 1991 and 2000 were analysed and the histopathological features reviewed light microscopically. RESULTS: Among the clinical features, a marked male predominance with younger age of presentation and left-sided dominance was noted. The duration of symptoms was less than 10 months in 28% cases. Histopathologically, the features included presence of cystic degeneration (15.6%) and squamous metaplasia (18.7%). Calcification, osteoid formation and malignant transformation (3.1% each) were occasionally observed. CONCLUSIONS: Pleomorphic adenomas of the lacrimal gland may present with duration of symptoms of less than 10 months. Cystic degeneration, squamous metaplasia, calcification or ossification may be observed in lacrimal gland pleomorphic adenomas, as has been described in pleomorphic adenomas of salivary glands. However, the presence of calcification and bone erosion radiologically in a lacrimal gland tumour does not necessarily indicate malignancy.     

眼眶泪腺上皮性腫瘤的影像學診斷

目的分析34例泪腺上皮性腫瘤的影像學特徵.方法對20例泪腺良性多形性腺瘤(復發性7例),11例腺樣囊性癌(復發性9例),惡性多形性腺瘤2例(均爲復發性)和1例粘液表皮癌的超聲,CT和MRI進行分析.結果良性多形性腺瘤呈圓形,邊界清楚,骨窩形成.腺樣囊性癌體積較大,形狀不規則,骨破壞明顯.結論根據腫瘤的位置,形狀,邊界和繼發性改變(泪腺窩擴大,骨破壞,鈣化,病變向周圍結構蔓延)等影像學表現可作出較準確的術前定性診斷.     

130例原发泪腺上皮性肿瘤的诊断分析

目的分析130例泪腺上皮性肿瘤临床特点,明确诊断依据。设计回顾性病例系列。研究对象130例原发泪腺上皮性肿瘤。方法收集130例经病理检查确诊的泪腺上皮性肿瘤的临床资料、影像学资料和组织病理学资料。主要指标临床表现,影像学表现,组织病理学表现。结果泪腺上皮性肿瘤临床表现主要为眼球突出、视力下降、疼痛、复视等。良性肿瘤中以多形性腺瘤(43．8％)为多,恶性肿瘤占前三位的分别为腺样囊性癌(38．5％),恶性混合瘤(8．5％),腺癌(3．8％)。眼眶B超、CT、MRI都有典型的影像学特征。组织病理学改变是最终诊断依据。结论将影像学表现和临床资料结合起来可对泪腺上皮性肿瘤作出初步诊断,最终诊断要依靠组织病理学检查。     

眼眶泪腺癌26例的CT诊断价值

目的：探讨泪腺腺癌的CT诊断价值。 方法：回顾性分析26例26眼经病理证实眼眶泪腺腺癌患者的CT影像资料。 结果：泪腺腺癌26例均为单眼发病,右眼15例,左眼11例。CT表现：病变呈圆形、卵圆形15例,不规则形、分叶形11例。15例边界尚清楚,11例轮廓呈锯齿状。肿瘤密度不均匀,内见低密度灶或钙化。26例病变包绕并压迫眼球,14例沿眶外壁向眶尖区生长,与眼肌分界不清,并浸润视神经。眼眶骨质虫蚀样破坏21例,溶骨性破坏5例。 结论：泪腺癌有特异性CT征象,其诊断准确率高,是本病最主要影像检查方法。     

In situ adenocarcinoma ex pleomorphic adenoma of the lacrimal gland

A case of an in situ adenocarcinoma of the lacrimal gland that developed in a recurring pleomorphic adenoma is presented. Six years after incomplete resection of a pleomorphic adenoma, the patient complained of a progressive swelling of her right upper eyelid without pain or diplopia. An orbital computerized tomography scan showed an inhomogeneous mass in the right lacrimal gland region without bone involvement. After local excision of the in situ adenocarcinoma ex pleomorphic adenoma, the tumour has been in control for over 6 years.     

Mucoepidermoid carcinoma ex pleomorphic adenoma of the lacrimal gland: A rare presentation

Carcinoma ex pleomorphic adenoma in lacrimal gland is a rare entity unlike its salivary gland counterpart. This rare tumor poses a diagnostic challenge to clinicians as pre-operative diagnosis is difficult and diagnosis is only by careful pathological assessment. We report this uncommon lesion in a 62-year-old lady, wherein the malignant component was mucoepidermoid carcinoma. The elderly patient remained clinically and radiologically free of the tumor for two years after complete excision of the tumor but computed tomography at the end of two and a half years showed a recurrent lesion in the region of the lacrimal gland. This makes long term follow up of patients with these rare lacrimal tumors imperative with a minimum period of at least five years.     

E-cadherin和β-catenin在泪腺多形性腺瘤和腺样囊性癌中的表达

目的研究E-cadherin及β-catenin在泪腺多形性腺瘤和腺样囊性癌中的表达。方法选取经病理检查确诊为泪腺多形性腺瘤的患者11例11眼和腺样囊性癌的患者6例6眼,分别行E-cadherin和β-catenin免疫组织化学染色。选取泪腺脱垂患者的泪腺组织6例作为正常对照组。结果正常泪腺组织中E-cadherin呈强阳性表达,主要位于细胞膜;而在泪腺多形性腺瘤中E-cadherin呈弱阳性表达,主要位于细胞质。E-cadherin在腺样囊性癌中未见表达。3组中E-cadherin阳性表达的样本数差异有统计学意义（H=16.492,P=0.000）;正常泪腺组E-cadherin阳性表达的样本数明显高于多形性腺瘤组和腺样囊性癌组,差异均有统计学意义（U=-3.561,P=0.000;U=-3.108,P=0.002）,多形性腺瘤组中E-cadherin阳性表达的样本数明显高于腺样囊性癌组,差异有统计学意义（U=-2.147,P=0.032）。正常泪腺β-catenin细胞膜呈中度或强阳性表达,多形性腺瘤和腺样囊性癌中β-catenin的表达较正常泪腺组织表达阳性程度差异无统计学意义（P〉0.05）,腺样囊性癌组中β-catenin细胞核阳性的比例（33.3%,2/6）较多形性腺瘤组（72.7%,7/11）低。结论 E-cadherin表达的下降是参与泪腺上皮性肿瘤发生及癌变的重要因素,而E-cadherin的进一步下降甚至缺失,以及β-catenin的核转位可能是参与泪腺多形性腺瘤恶性转化的因素之一。     

常见泪腺上皮性肿瘤中EGFL7的表达及其与血管生成和细胞增殖活性的相关性研究

目的：检测常见泪腺上皮性肿瘤中类表皮生长因子结构域 7(EGFL7)、微血管密度(MVD)、Ki67的表达,探究EGFL7与MVD、Ki67之间的相互关系。

方法：应用免疫组化法,对10例正常泪腺组织(取自因炎性假瘤、Mikulicz病等良性泪腺肿瘤切除后,标本中的部分正常泪腺组织)、20例泪腺多形性腺瘤、12例多形性腺癌、14例腺样囊性癌组织中的EGFL7蛋白、Ki67进行检测,并应用CD34标记计数MVD。

结果：EGFL7主要在泪腺多形性腺癌、腺样囊性癌的肿瘤细胞的细胞浆中表达,免疫组化染色结果显示,在正常泪腺组织中EGFL7无阳性表达,泪腺多形性腺瘤组织中EGFL7的阳性率为5%(1/20),泪腺多形性腺癌中EGFL7的阳性表达率83%(10/12),泪腺腺样囊性癌中EGFL7的阳性表达率为86%(12/14),两种恶性肿瘤中的表达率明显高于多形性腺瘤和正常泪腺组织(P<0.001)。CD34可使肿瘤微血管染色呈棕黄色的单个或成簇细胞群。在泪腺多形性腺癌(32.58±14.46)及腺样囊性癌(43.43±4.60)中CD34的表达明显高于多形性腺瘤(4.20±1.19)(P<0.001); Ki67在具有增殖活性的细胞核呈棕褐色着色,在泪腺多形性腺癌(44.83±13.68)及腺样囊性癌(26.29±8.44)中Ki67的表达明显高于多形性腺瘤(2.80±3.14)及正常组织(0.40±0.70)(P<0.001)。在两种恶性肿瘤中EGFL7的表达分别与MVD、Ki67呈明显正相关(r_s=0.897,P<0.001; r_s=0.837,P<0.001)。

结论：EGFL7在泪腺上皮性肿瘤中的表达与MVD、Ki67呈正相关性,提示EGFL7不仅在泪腺上皮性肿瘤血管生成中起重要作用,而且参与泪腺上皮性肿瘤的增殖。     

Basal cell cystadenoma of the lacrimal gland: diagnostic pitfalls of a basaloid pattern in lacrimal tumours

An 85-year-old male experienced a painless swelling along the left lateral orbit for one year. A computed tomography scan demonstrated a cystic mass in the orbit adjacent to the lacrimal gland. There was a concern for malignancy considering the large size and the patient’s age, so the tumour was excised. Histopathology of the tumour showed nests with basaloid patterns, but a definitive diagnosis was not rendered. The uncertainty of tissue diagnosis coupled with the basaloid pattern, which carries a grim prognosis in some salivary gland tumours, led us to refer this case to an authority on lacrimal gland pathology, who suggested that this tumour be called a basal cell cystadenoma. To the best of our knowledge, a basal cell cystadenoma of the lacrimal gland has not been reported in the literature. We present histopathological features that distinguish this tumour from malignant tumours with a basaloid pattern. We also discuss the management differences associated with basaloid patterns in lacrimal tumours.     

125 I粒子治疗泪腺恶性和复发多形性腺瘤

目的：观察 125 I粒子植入治疗泪腺恶性肿瘤和复发的多形性腺瘤的临床效果和并发症。方法：回顾性系列病例研究。收集2008年7月至2016年2月于郑州大学第一附属医院眼科收入院治疗并保留完整病历资料的泪腺上皮性肿瘤16例,其中泪腺腺样囊性癌8例,黏液表皮样癌1例,癌在多形性腺瘤中2例,复发的多形性腺瘤5例。所有患者均经过手术和病理检查确诊,恶性肿瘤和复发的多形性腺瘤在术后1个月行 125 I粒子植入治疗。临床和影像学检查随访项目包括肿瘤局部控制情况和并发症。 结果： ①肿瘤控制情况：11例1次 125 I粒子植入有效控制病变;3例2次植入有效控制病变;1例3次、1例4次植入有效控制病变。②并发症情况：16例均未见眼球、视神经和颅脑穿刺损伤;13例在3个月内出现眼表刺激征,其中1例眼睑浅部植入粒子出现重度刺激征;8例出现干眼症表现;6例出现轻度的结膜充血、睑缘肥厚、睫毛或眉毛脱落、角膜水肿、角膜上皮脱失;4例出现皮肤表面红肿和色素沉着。并发症多在3个月后逐渐缓解和恢复正常。结论：泪腺恶性肿瘤和复发的多形性腺瘤,术后给予 125 I粒子植入内照射治疗,肿瘤局部控制效果好,且并发症较少,被认为是治疗易复发的泪腺上皮性肿瘤的有效方法。     

原发性泪腺上皮性肿瘤患者97例诊治分析

目的　探讨泪腺上皮性肿瘤的临床表现、影像学特征、诊断方法及治疗原则。方法　对２００３年１月至２０１４年１０月在河南省眼科研究所就诊的９７例泪腺上皮性肿瘤的临床表现、影像学检查、治疗方法、术后视力、及随访情况等临床资料进行回顾性分析。采用前路开眶１１例,常规外侧开眶１９例,改良“Ｓ”形皮肤切口并外侧开眶６７例。结果　９７例泪腺上皮性肿瘤中,泪腺多行性腺瘤６５例（６７．０１％）,腺样囊性癌１６例（１６．４９％）,多形性腺癌１２例（１２．３７％）,腺癌２例（２．０６％）,黏液表皮样癌１例（１．０３％）,肌上皮瘤１例（１．０３％）。术后视力提高３７例,无变化５４例,下降６例。１３例出现眼球内陷,早期上睑下垂１１例,４例出现外展运动受限、水平复视。术后６个月随访:上睑下垂４例;１例出现永久性外展不足、水平复视。结论　泪腺上皮性肿瘤具有典型的临床表现和特征性的影像学改变,术前通过准确的定位来选择适宜的手术进路,可顺利并完整地切除肿瘤,避免肿瘤复发和出现严重并发症。     

Space-occupying lesions of the lacrimal gland at one tertiary eye center in China: a retrospective clinical study of 95 patients

AIM: To investigate the treatment status and prognosis of space-occupying lacrimal gland lesions at one tertiary eye center in China. METHODS: A retrospective clinical study was performed on 95 patients with space-occupying lesions of the lacrimal gland surgically treated at the Eye & ENT Hospital of Fudan University from 2003 to 2007. The reviewed clinical data included age, gender, side of the lesion, duration of signs and symptoms, histopathological diagnosis, treatment modality, recurrence (local, regional, and distant metastasis) and survival. RESULTS: Of the 95 cases (99 eyes), pleomorphic adenomas were the most common lesions (43 cases), followed by lymphoid disorders (14), inflammatory pseudotumors (11), carcinoma ex-pleomorphic adenomas (11), and adenoid cystic carcinomas (ACC, 6). There were 8 patients with relapsed pleomorphic adenomas. Five of these 8 cases had malignant pathological changes. All patients with ACC had metastasis and three of them died during their follow-up. CONCLUSION: Our study indicated that the most common lacrimal gland lesions were pleomorphic adenomas. Multiple recurrence and surgical procedures may increase the risk of tumor progression. ACC had a high incidence of tumor metastasis and a poor prognosis.     

Pleomorphic adenoma of a subconjunctival ectopic lacrimal gland

This report describes a case of pleomorphic adenoma of an ectopic lacrimal gland arising subconjunctivally in the lateral fornix in a 13-year-old girl. The tumor was removed surgically in toto with the capsule. This is probably the first reported case.     

回顾191例泪腺占位性病变临床分析及术后随访观察

目的：探讨191例泪腺占位性病变的临床特点及术后随访情况。

方法：选取2011-01/2015-08我科收治的191例221眼泪腺占位患者,总结其临床特征,并结合病史、影像、病理资料、地域特色进行分析。所有患者行泪腺肿瘤摘除,术后随访1a。

结果：患者191例221眼中,男44例49眼,女147例172眼。炎症性病变171眼,依次是IgG₄硬化性泪腺炎66眼、慢性泪腺炎27眼、泪腺脱垂伴炎性肿大54眼、Grave''s病24眼。淋巴组织增生性病变16眼,依次是恶性淋巴瘤6眼、良性淋巴组织增生10眼。上皮性病变34眼,依次是多形性腺瘤26眼、多形性腺癌2眼、腺样囊性癌3眼、腺癌3眼。泪腺占位性病变以IgG₄硬化性泪腺炎、泪腺脱垂伴炎性肿大多见,其中汉族159眼、维族36眼、哈萨克族16眼、蒙古族10眼。手术后主要表现为眼部干涩,哭时无泪,以双侧泪腺摘除者明显,但局部使用人工泪液可以缓解,无严重不良反应。

结论：病史及影像特点对泪腺占位性病变的诊断和鉴别诊断有很大的帮助,新疆地区泪腺占位,以非上皮性病变最为常见,其次是上皮性病变,多发生于汉族、维族患者,而其它民族少有发生,手术后眼部干涩及哭时无泪为主要症状。对病程短且有干眼倾向的患者需延迟摘除。     

泪腺上皮性肿瘤α-1抗胰蛋白酶的免疫组化研究

应用α－１抗胰蛋白酶多克隆抗体对１０例多形性腺瘤和１０例腺样囊腺癌进行原位免疫标记研究。结果表明，１０例多形性腺瘤均呈阳性反应；１０例腺样囊腺癌中，７例呈阴性反应，３例阳性反应。但腺样囊腺癌的阳性反应程度明显低于多形性腺瘤。说明泪腺腺样囊腺癌的蛋白酶抑制物活性明显降低是该肿瘤具有较强的组织浸润力的原因之一。而且，α－１抗胰蛋白酶的免疫组化测定有助于泪腺上皮性肿瘤的良、恶性判断。     

Pleomorphic adenoma of a deep orbital ectopic lacrimal gland

Ectopic lacrimal gland, being one of the choristomas, is comprised of lacrimal gland tissue outside the lacrimal gland fossa in the fronto-lateral part of the orbital roof. Ectopic lacrimal gland is a rare condition where the gland may be found in the orbit, eyelids, ocular adnexa or within the globe. Neoplastic transformation of such tissue may occur. A sixty-two-year old male patient presented with right eye proptosis and slight nasal displacement of the globe. Computerized tomography scan revealed a well-defined hypodense lesion of size 19 x 18 x 20 mm supero-lateral to lateral rectus muscle, with mild proptosis and thinning of the right lateral orbital wall. Excisional biopsy was performed through a lateral orbitotomy approach. A well circumscribed globular mass was removed from the right orbit, well behind the fossa for the lacrimal gland in the retrobulbar space. Histopathology was suggestive of pleomorphic adenoma of lacrimal gland. Pleomorphic adenoma is an epithelial tumor of the lacrimal gland which is extremely rare from an ectopic lacrimal gland and only few cases have been reported in literature till date.     

Correlation of computed tomographic and histopathologic features in malignant transformation of benign mixed tumor of lacrimal gland

A 65-year-old man had painless progressive proptosis of five years' duration in his left eye. Sudden increase of the proptosis required a lateral canthotomy. A lateral orbitotomy was performed and a large, encapsulated, globoid mass was totally excised. Histopathologic diagnosis was adenoid cystic carcinoma arising in and surrounding a benign mixed tumor of the lacrimal gland. The computerized tomographic features correlated quite accurately with the gross appearance and histopathologic findings observed in the lacrimal gland mass. The authors discuss the clinical and radiographic features that should alert the clinician to the possibility of malignant transformation in a benign mixed tumor of the lacrimal gland.