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 exten- sion 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.     

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。     

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.     

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。     

Carcinosarcoma of the lacrimal gland arising from a pleomorphic adenoma

PURPOSE: To report a case of the lacrimal gland tumor diagnosed as carcinosarcoma (true mixed tumor) arising from a pleomorphic adenoma. DESIGN: Interventional case report. METHODS: An 80-year-old Japanese woman was referred with a well-circumscribed tumor in the lacrimal fossa. RESULTS: The tumor was excised, but the deepest portion remained because of heavy bleeding. Histopathologically, the tumor consisted of osteosarcomatous cells. No epithelial or myoepithelial component was identified by immunohistochemistry. Two months later, the tumor progressed further and was excised completely with lateral orbitotomy. The tumor consisted primarily of osteosarcomatous component but also had epithelial components including carcinoma and pleomorphic adenoma. Examinations did not detect local recurrence and metastasis at 10 post-operative months. CONCLUSION: The tumor was diagnosed as true carcinosarcoma of the lacrimal gland arising from a pleomorphic adenoma. When lacrimal gland tumors containing sarcomatous components are encountered, carcinosarcoma should be included in the differential diagnosis.     

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。     

Atypical presentations of pleomorphic adenoma of the lacrimal gland

PURPOSE: To report 3 cases of pleomorphic adenoma of the lacrimal gland with atypical features. METHODS: The medical records, radiographic imaging, operative reports, and tumor histopathology of 3 patients with unusual presentations of pleomorphic adenoma of the lacrimal gland were reviewed. RESULTS: All 3 patients were females ranging in age from 18 to 64 years. The first patient presented with abrupt orbital inflammation mimicking orbital cellulitis. The second patient presented with a painful subcutaneous nodule. The last patient demonstrated calcification with bony erosion on orbital imaging. Orbitotomy was performed in all cases. Histopathologic evaluation of all specimens with light microscopy was consistent with pleomorphic adenoma of the lacrimal gland. CONCLUSIONS: Pleomorphic adenoma is the most common epithelial tumor of the lacrimal gland. A higher degree of suspicion must be present to make the correct diagnosis in cases with atypical features. Pleomorphic adenoma may present abruptly with orbital inflammation mimicking orbital cellulitis, as a painful subcutaneous nodule, or demonstrate calcification with bony erosion on orbital imaging. Complete surgical excision of the lesion within its pseudocapsule is recommended to prevent local recurrences and, although less likely, the possibility of malignant transformation.     

Calcified orbital cyst simulating a malignant lacrimal gland tumor in an adult

A 56-year-old woman with left periocular pain was discovered on CT to have a partially calcified, circumscribed cystic lesion in the lacrimal gland fossa, suggestive of a malignant lacrimal gland tumor. Adjacent bone fossa formation without erosion was noted. Histopathologic examination of the excised mass disclosed a cyst with chronic granulomatous inflammation and calcification of the cyst wall. The combination of chronic pain, cystic changes, and calcification in a lacrimal gland fossa mass in an adult should suggest a malignant neoplasm, but a long-standing orbital cyst with calcification of its rim can produce the same features.     

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。     

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.     

泪腺上皮性肿瘤261例的临床和组织病理学特点分析

目的研究泪腺上皮性肿瘤的病理类型和临床特征。方法收集261例经病理诊断为泪腺上皮性肿瘤患者的273份石蜡切片标本,结合患者的组织病理学和临床相关资料进行归纳分析,探讨不同类型泪腺上皮性肿瘤的病理学特点及临床表现。结果泪腺上皮性肿瘤中各种类型所占比例由高向低排列依次为多形性腺瘤(混合瘤)(62．3％)、泪腺囊腺癌(22．3％)、多形性腺癌(8．8％)及泪腺腺癌(4．0％)等。术后复发12例,总复发率为4．6％;死亡4例,死亡率为1．5％。结论泪腺上皮性肿瘤死亡率较低,但复发率较高。应根据临床资料和病理诊断决定治疗用药和术后处理,而以手术治疗为主。     

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.     

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。     

Adenoid cystic carcinoma of the lacrimal sac

Adenoid cystic carcinoma is the most common epithelial malignancy of the lacrimal gland and minor salivary glands. Its occurrence in the lacrimal drainage system is extremely rare. A 57-year-old woman presented with epiphora and a mass in the lacrimal sac, which proved to be an adenoid cystic carcinoma. Histologically, it appeared to arise from the wall of the lacrimal sac, and both the lacrimal gland and maxillary antrum were uninvolved by tumor. Despite aggressive surgical intervention, recurrence and metastasis developed. Adenoid cystic carcinoma must be considered in the differential diagnosis of epiphora.     

泪腺上皮性肿瘤113例临床病理学分析

目的 探讨泪腺上皮性肿瘤组织病理学类型和临床特征.方法 回顾性病例研究重新复习天津眼科医院病理科自1980年至2009年间收检的113例泪腺上皮性肿瘤的临床和病理学资料,参照WHO关于唾液腺肿瘤的分类和诊断标准,分析泪腺上皮性肿瘤的类型和临床病理学特点.结果 113例泪腺上皮性肿瘤中,多形性腺瘤73例(64.6%),腺样囊性癌23例(20.4%),癌在多形性腺瘤中9例(8.0%),其他类型的上皮性肿瘤均为恶性,共8例(7.0%).结论 泪腺上皮性肿瘤有不同的病理类型,其临床特点、治疗和预后有很大区别,正确和详细的病理诊断可对临床治疗起到指导意义.     

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。     

Beware the empty curette!

BACKGROUND AND OBJECTIVE Chalazions are a common occurrence in the eyelids due to chronic inflammation in the tarsal plate. Treatment of non-resolving cases may involve incision and curettage. Chalazions that are recurrent should arouse suspicion. We present a case of a pleomorphic adenoma of the palpebral portion of the lacrimal gland, which was misdiagnosed as a chalazion. PATIENT AND METHODS A 41-year-old Caucasian lady initially presented with a swelling in the outer part of her right upper eyelid. A diagnosis of chalazion was made and two attempts at incision and curettage failed to resolve the lesion. She was then referred to the oculoplastics/adnexal unit. A more detailed examination revealed involvement of the palpebral part of her lacrimal gland. A CT-scan confirmed this and she proceeded to have a lateral orbitotomy to remove the tumour. RESULT Histological confirmation of a pleomorphic adenoma of the palpebral portion of the lacrimal gland was obtained. The lacrimal gland had been completely excised. CONCLUSION Persistent or recurrent meibomian cyst should be treated with suspicion. A careful orbital examination including lid eversion should always be performed to outrule a more sinister pathology.     

Cystic carcinoma ex pleomorphic adenoma of the lacrimal gland

In a 48-year-old male patient, a well-circumscribed, round tumor in the lacrimal fossa was detected by CT. During total excision of the tumor, a wall of the tumor ruptured, resulting in leakage of the fluid contents. The wall of the tumor formed a cyst that contained residual yellowish fluid. Histologically, the cyst wall included an adenocarcinomatous component and a focus of pleomorphic adenoma. The tumor was diagnosed as a carcinoma ex pleomorphic adenoma of the lacrimal gland. The carcinoma cells showed positive immunoreactivity for androgen receptor and BRST-2, indicating that the tumor was equivalent to a salivary duct carcinoma. Postoperatively, the patient underwent involved field irradiation. This case demonstrates that a carcinoma ex pleomorphic adenoma of the lacrimal gland may develop in a cyst.     

Primary malignant neoplasms of the lacrimal gland.

The clinical characteristics and outcome of 50 primary malignant neoplasms of the lacrimal gland are reviewed: 38 (76%) adenoid cystic carcinomas, six (12%) carcinomas arising in pleomorphic adenoma, and six (12%) adenocarcinomas or other types of carcinoma. Most patients presented with a short history and pain, though pain tended to occur less often and later with adenocarcinoma than with adenoid cystic carcinoma. Pain was unrelated to the duration of symptoms, invasion of bone, loss of trigeminal nerve function, or the frequency and time of tumour recurrence. The estimated disease-free survival for patients with adenoid cystic carcinoma was significantly (p less than 0.01) reduced where half or more of the biopsy specimen showed basaloid differentiation. Eleven patients underwent extended cranio-orbital resection, and the others received a combination of total dacryoadenectomy adenectomy and/or radiotherapy. Survival after adenoid cystic carcinomas appears to be significantly (p less than 0.05) greater when tumour resection is combined with radiotherapy than after radiotherapy alone. At present, however, the rate of disease-free survival after treatment of adenoid cystic carcinoma appears unaltered by cranio-orbital resection, though these latter patients form a relatively greater proportion of those surviving for more than 10 years. Further long-term follow-up is needed to see if this technique does influence survival.