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.     

Primary ductal adenocarcinoma of the lacrimal gland with changing genetic analysis mutations

Primary ductal adenocarcinoma of the lacrimal gland is a rare but highly aggressive epithelial malignancy with a poor prognosis. Early diagnosis, along with genetic testing of these tumors, is imperative for proper management. We present a case of a 54-year-old man with decreasing vision over the past three years and increasing proptosis in his right eye over the past three months, secondary to a lacrimal gland mass diagnosed as primary ductal adenocarcinoma. The diagnosis was made using histological and immunohistochemical profiles (positivity for cytokeratin AE1/3, CAM5.2, androgen receptor, human epidermal growth factor receptor 2, and gross cystic disease fluid protein 15) seen in previous cases, alongside a tumor genetic profile that showed actionable mutations. Uniquely in this case, after failing traditional chemotherapy, repeat biopsy revealed a change in genetics with the malignancy no longer showing actionable mutations. These findings show that these immunohistochemical findings can act as diagnostic biomarkers, while genetic testing can reveal actionable mutations for targeted therapy.     

Stones in the lacrimal gland: a rare condition

Purpose: Stones (dacryoliths) in the lacrimal drainage system are relatively common. However, stones in the lacrimal gland itself are very rare. We present three cases of lacrimal gland stones and describe the clinical and histopathological characteristics and composition of the stones. Methods: Three patients presented with swelling in the lateral canthal region of several months’ duration. Clinical examination in each case revealed a mass adjacent to the lacrimal gland. We carried out surgical excision, histological examination and Raman spectroscopy. Results: Histopathological examination of the excised material showed an eosinophilic, amorphic material organized in lamellae and localized in a ductule. The findings were consistent with a stone in the lacrimal gland in all three cases. All stones were composed of proteins of similar types. After surgery, the three patients healed without complications. Conclusions: Lacrimal gland stones represent a very rare but relevant differential diagnosis when a patient presents with unilateral persistent conjunctivitis or with a tumour in the lateral canthus. Treatment is excision under local anaesthesia.     

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.     

Intraocular lacrimal gland choristoma

There have been nine previously reported cases of intraocular lacrimal gland choristoma. This case report is of an infant with an intraocular lacrimal gland choristoma which was managed conservatively for a 19-month period until the onset of glaucoma. Tumour biopsy was initially performed because until this time the tumour's behaviour suggested it was not malignant. The latter was confirmed on biopsy, however hypotony resulted following the surgical intervention.     

泪腺脱垂复位并重睑成形治疗泪腺脱垂

目的 探讨泪腺脱垂复位并重睑成形治疗泪腺脱垂的临床效果.方法 对18例(36眼)泪腺脱垂按照重睑成形术设计手术切口,根据上睑皮肤松弛和眼睑饱满程度决定皮肤及脂肪的去除量,用4-0线褥式缝合泪腺前缘及泪腺筋膜将脱垂的眶部或睑部泪腺分别固定于眶上缘内及眶外侧壁的骨膜上,并将切开的眶隔拉紧加固间断缝合,上睑皮肤以重睑成形方式缝合,外侧皮肤间断缝合.术后随访6个月～3年.结果 17例34眼(94.44％)术后泪腺复位良好,无复发.1例2眼(5.56％)在术后2年轻度复发,但症状较术前明显改善.18例36眼全部双眼睑裂对称,重睑形态良好自然.结论 泪腺脱垂复位、眶隔加固并重睑成形术治疗泪腺脱垂效果良好,实现了功能治愈与美容效果的双重目的.     

The role of biopsy in lacrimal gland inflammation: A clinicopathologic study

Purpose: To determine the causes of lacrimal gland inflammation based on histopathology and systemic evaluation.

Methods: This is a retrospective case series study. From the University of British Columbia Orbit Clinic between January 1976 and December 2008, we reviewed the medical records of 60 patients who presented with inflammatory features of the lacrimal gland (i.e., erythema, edema, or tenderness) in which the diagnoses were not possible clinically and on imaging alone. As was our routine practice, all these patients underwent lacrimal gland biopsy before starting any treatment.

Results: The histopathologic findings of the 60 patients showed that 37 (61.7%) had identifiable types of lacrimal inflammation including 10 with Sjogren’s syndrome, seven with sarcoidal reaction, six with feature of granulomatosis with polyangiitis (formerly known as Wegener’s granulomatosis), five with lymphoma, two with sclerosing inflammation, two with IgG4-related dacryoadenitis, and one patient each with infectious dacryoadenitis, myoepithelial carcinoma, xanthogranuloma, eosinophilic angiocentric fibrosis, and eosinophilic allergic granulomatous nodule. The histopathologic findings of the remaining 23 (38.3%) patients showed nonspecific inflammation of the lacrimal gland. 23 patients (38.3%) had associated systemic diseases. 48 patients (80%) were treated successfully and 10 (16.7%) had recurrence of inflammation.

Conclusions: We recommend that in patients presenting with lacrimal gland inflammation (i.e., erythema, edema, tenderness) in which the specific diagnosis cannot be made clinically and on imaging, biopsy is warranted for accurate diagnosis and appropriate treatment. We found that the majority of these patients (61.7%) had specific histopathology, and 38% had systemic diseases.     

A clinicopathologic study of primary adenoid cystic carcinoma of the lacrimal gland

A retrospective study of 26 patients with the diagnosis of primary adenoid cystic carcinoma of the lacrimal gland was performed with use of randomly selected tissue from each tumor. An attempt was made to determine any associations among clinical presentation, survival, and tumor histologic patterns. We found that lower tumor grades were associated with a predominantly Swiss-cheese (cribriform) pattern. Both lower tumor grade and Swiss-cheese pattern were associated with longer survival. The basaloid (solid) pattern or bone invasion was not associated with a shorter survival. Between men and women, there were no differences in clinical presentation, histologic patterns, or survival. Survival was not affected by surgical or radiation therapy.     

眶内泪腺良性肿瘤的CT诊断临床研究

目的:探讨CT对泪腺良性肿瘤的诊断价值。方法:回顾性分析30例经病理证实良性泪腺肿瘤临床资料和CT影像资料。结果:泪腺良性肿瘤患者30例中26例位于泪腺眶部,泪腺睑部4例。肿瘤椭圆形18例,不规则形4例,边缘不规则呈现分叶状或结节状8例。CT表现大多数为密度均匀,眼眶皮质受压,凹陷18例,骨皮质破坏伴硬化肥厚12例。结论:泪腺良性肿瘤CT有一定特征,诊断准确率高。     

Amyloidosis of lacrimal gland

Primary localized amyloidosis of lacrimal gland is a rare occurrence. This report describes a female patient with isolated amyloidosis of the lacrimal gland. A 45-year-old Indian woman presented with a swelling over the left lacrimal gland region. Computed tomography showed uniform enlargement of the lacrimal gland. A lacrimal gland biopsy revealed amyloidosis. No systemic involvement was detected on further investigation. To our knowledge, this is the first report of lacrimal gland amyloidosis from India and our report also highlights the importance of lacrimal gland biopsy in diagnosing lacrimal gland masses.     

泪腺良性淋巴上皮病变免疫组织化学研究

目的 通过研究9种单克隆抗体在泪腺良性淋巴上皮病变中的表达,探讨其免疫病理本质及发病机制,为临床治疗及预后提供理论依据.方法 使用CD4、CD8、CD21、CD34、CD45、CD68、IgG、IgG4和增殖细胞核抗原这9种分子的单克隆抗体,应用免疫组织化学染色SP法对25例泪腺良性淋巴上皮病变的石蜡标本进行免疫组织化学染色.结果 B淋巴细胞表面抗原(CD21)、T淋巴细胞表面抗原(CD4、CD8和CD45)、黏附分子CD34及IgG在25例样本中均呈阳性表达,巨噬细胞表面抗原CD68、增殖细胞核抗原和IgG4这3种分子在25例样本中均有一定比例的阳性表达,表达率分别为12％、8％和80％.结论 泪腺良性淋巴上皮病变是一种主要以B淋巴细胞和T淋巴细胞激活为主要病理免疫基础的疾病,绝大多数属于IgG4相关性疾病的范畴,同时有发生恶变的潜能,临床治疗后的密切随访很有必要.     

Giant pleomorphic adenoma of the lacrimal gland: A surgical challenge

Pleomorphic adenoma is the most common epithelial neoplasm of the lacrimal gland. The tumor typically presents with a superotemporal mass with inferonasal displacement of the globe. They generally measure less than 3 cm in size and can be removed comfortably via a lateral orbitotomy approach. Pleomorphic adenoma left unattended for a long period grows up to humongous proportions and poses a surgical challenge both for its complete removal and globe salvage. We report a rare case of pleomoprphic adenoma of the lacrimal gland in an adult male, who did not seek any medical advice for 20 years allowing the tumor to expand enormously in all dimensions, with complete obscuration of the globe. The tumor was excised completely and globe could be salvaged.     

泪腺病变的CT分析

目的探讨不同泪腺病变的CT表现,提高泪腺病变的诊断水平。方法20例23眼泪腺病变患者,比较分析其CT表现特点。结果10例11眼泪腺炎性假瘤表现为泪腺增大,与周围结构如眼环分界不清,部分边缘锐利,其中1例伴有外直肌及上直肌增厚。1例1眼泪腺淋巴细胞反应性增生表现为右侧泪腺体积增大,边界清楚。5例5眼泪腺混合瘤,主要表现为泪腺区类圆形软组织肿块,密度均匀。2例4眼良性淋巴上皮性病变表现为对称性双侧泪腺弥漫性肿大,与周围组织分界清楚。2例2眼泪腺癌表现为眼眶外上方软组织肿块影,侵犯邻近组织结构并破坏眶壁骨质。结论根据CT表现特点,多数泪腺病变可明确诊断。     

Carcinoid tumour in the lacrimal gland

A case of carcinoid tumour in the lacrimal gland is described, thought to be a metastasis from a known primary lesion in the mediastinum. The results of light and transmission electron microscopic examination are presented, and the possibility of the lesion representing a second primary tumour discussed.     

泪腺窝眶缘骨孔固定泪腺治疗泪腺脱垂临床观察

目的:探讨泪腺窝眶缘骨孔固定泪腺治疗泪腺脱垂临床效果。方法:对21例42眼泪腺脱垂患者采用重睑切口,泪腺窝眶缘制作骨孔,缝线穿孔固定泪腺手术。术后随访3～36mo。结果:全组21例42眼,显效19例38眼,显效率90.5%,有效2例4眼,有效率9.5%,无效0例,总有效率100%。结论:泪腺窝眶缘骨孔固定泪腺能有效治疗泪腺脱垂,实现患者外观与功能的双重恢复。     

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.