Clinical characteristics, radiologic features, and histopathology of biopsied lacrimal gland tumors.

Herein, we described the clinicopathologic and radiologic features of biopsied lacrimal gland tumors. A retrospective case series of 79 patients treated between 2004 and 2021 was reviewed. The median age was 48.9 years (range 18.3-88.3 years), with 51.9% females. The histopathologic diagnoses were as follows: immunoglobulin G4-related disease (IgG4-RD) = 23, reactive lymphoid hyperplasia = 14, lymphoma = 14, nonspecific inflammation = 10, adenoid cystic carcinoma (ACC) = 9, and pleomorphic adenoma = 9. The proportion of histopathologic diagnoses did not differ significantly over the range of symptom durations (≤ 1 month, > 1-3 months, > 3 months). Patients with ACC had significantly shorter symptom duration and more frequent proptosis than those with pleomorphic adenoma (p = 0.040 and p = 0.009, respectively). Patients with IgG4-RD were older (median 54.3 years) than those with nonspecific inflammation (36.2 years; p = 0.046). Patients with ACC were more likely to present with diplopia than those with lymphoma (p < 0.001). The superior wedge sign increased the likelihood of ACC compared with that of non-epithelial non-malignant lacrimal gland tumors (relative risk ratio = 13.44, p = 0.002). The overall survival of patients with ACC and lymphoma did not differ significantly. Although these patients present with a short symptom duration, urgent orbital imaging, tissue biopsy, and prompt treatment should be performed in patients with lacrimal gland tumors.

Causes of Tearing in Patients With Chemotherapy: Meibomian Gland Dysfunction Versus Lacrimal Drainage Obstruction.

Numerous ocular toxicities that have been associated with the use of chemotherapeutic agents present as problems with the ocular surface, ocular adnexa, and lacrimal system, and many chemotherapeutic agents have tearing as a side effect. In this study, 34 eyes from 17 patients with a mean age of 62.4±14.8 years were analyzed. Chemotherapy was administered for a mean of 13.8±7.6 months. Chemotherapeutic agents of the following types were included: titanium silicate-1 (58.8%), Docetaxel (23.5%), Paclitaxel (11.8%), and 5-fluorouracil (5.9%). Tearing began 9.1 to 10.9 months after chemotherapy treatment. Within 3 months of beginning chemotherapy, tearing occurred in 9 patients (52.9%), and within 6 months, it occurred in 11 patients (64.7%). Mean tear break-up time was 5.4±2.6 sec. Ten eyes (29.4%) had normal fluorescein dye disappearance test findings (within grade 1), and the mean fluorescein dye disappearance test was 1.91±0.87. Among the 34 eyes, 24 (70.6%) had normal puncta and 9 (26.5%) and 1 (2.9%) had stenosis and blockage, respectively. Ten eyes (29.4%) showed total regurgitation, 19 eyes (55.9%) showed partial regurgitation, and 5 eyes (14.7%) showed no regurgitation upon syringing. Four eyes (11.8%) and 30 eyes (88.2%), respectively, showed soft and hard stops upon probing. Dacryoscintigraphy confirmed that 6 eyes (17.6%) were normal, 8 eyes (23.5%) showed post-sac delay or obstruction, and 20 eyes (58.8%) showed pre-sac delay or obstruction. The mean meiboscores for the upper and lower eyelids on LipiView were 2.15±0.86 and 2.53±0.79, respectively. The difference in meiboscores between the upper and lower eyelids was significant ( P=0.004 ). Obstruction of the lacrimal drainage system is a significant contributing factor to tearing in chemotherapy patients. However, reflex tearing because of meibomian gland dysfunction should also be fully considered to effectively manage the tearing because of the high incidence of accompanying meibomian glands loss when the lacrimal drainage system is obstructed.

Normative lacrimal gland dimensions by magnetic resonance imaging in an Australian cohort.

PURPOSE: To report the normative dimensions of the lacrimal gland on fat-suppressed contrast-enhanced magnetic resonance imaging (MRI) in an Australian cohort. METHODS: Retrospective review of patients who underwent 3 T orbital MRI is presented. Two hundred eleven orbits were used to conduct lacrimal gland measurements. Orbits were excluded if there was ipsilateral orbital or lacrimal gland disease, prior surgery, or poor image quality. The length and width of the lacrimal gland were measured in axial and coronal sections using the largest image. RESULTS: The mean lacrimal gland axial length was 14.6 mm in the right orbit and 14.3 mm in the left orbit. The mean axial width was 4.9 mm in both orbits. Coronal lengths averaged 16.2 mm in the right orbit and 16.4 mm in the left orbit. The coronal width averaged 4.8 mm in both orbits. A significant negative correlation was found between age and the right axial length (r = -0.26, p < .01) and the left axial length (r = -0.26, p < .01) of the lacrimal gland. No statistically significant difference was found between genders or laterality. CONCLUSION: This study presents the normal lacrimal gland dimensions on fat-suppressed contrast-enhanced MRI in an Australian cohort. An inverse relationship exists between age and the axial length of the lacrimal gland. These data may be used to help diagnose enlargement of the lacrimal gland.

Lymphoproliferative disorders in the lacrimal caruncle: report of three cases and review of literature.

This report included three cases of lymphoproliferative disorders developing from the lacrimal caruncle. The first case was an 11-year-old boy with reactive lymphoid hyperplasia in the left lacrimal caruncle. The second case was an 80-year-old woman with reactive lymphoid hyperplasia in the right lacrimal caruncle. The third case was a 77-year-old man with follicular lymphoma in the left lacrimal caruncle. Our literature review of cases with lacrimal caruncular lesions showed 11 reported cases with reactive lymphoid hyperplasia and 17 with malignant lymphoma. There had been no previous report on follicular lymphoma in the lacrimal caruncle.

A rare case of mucoepidermoid carcinoma ex pleomorphic adenoma of the lacrimal gland.

Carcinoma ex pleomorphic adenoma (CEPA) of the lacrimal gland is a rare malignant tumor that arises from a pre-existing pleomorphic adenoma. Lacrimal gland CEPA with mucoepidermoid histological subtype is exceedingly rare. Diagnosis can be aided by radiographic findings, though the gold standard is histopathological analysis following excisional biopsy. Management options include complete surgical excision with or without adjuvant radiation therapy based on tumor grade and invasiveness. We present a 76-year-old woman with 6 months of diplopia and unilateral proptosis. Her initial exam was remarkable for hypoglobus, proptosis, and limited elevation of the right eye. Computed tomography (CT) scan demonstrated a superior, well-circumscribed, extraconal orbital mass. An excisional biopsy was performed, and histopathological findings were consistent with mucoepidermoid carcinoma ex pleomorphic adenoma with positive margins in the tumor capsule. The patient received radiation therapy and remains markedly improved with no disease recurrence at 5 months post-operatively.

Unexpected recurrence and rapid progression of lacrimal gland adenoid cystic carcinoma during pregnancy: a case report.

Adenoid cystic carcinoma (ACC) is the most common malignant neoplasm involving the lacrimal glands, with high rates of recurrence and metastasis. During the pregnancy, reports of recurrence of ACC of the salivary glands and trachea have previously been published, but no lacrimal gland ACC recurrence has been reported. We present a 35-year-old woman with lacrimal gland ACC who was initially treated by surgical resection and adjunctive radiotherapy, but her cancer recurred during pregnancy, with rapid progression to cavernous sinuses and brain. Estrogen and progesterone receptors have been detected on lacrimal glands and ACCs of salivary glands. Thus, hormonal changes during pregnancy might contribute to the recurrence of ACC. However, the inherent invasive and recurrent nature of ACC could also account for the regrowth in this patient and further molecular studies can provide more accurate explanations.

Clinical Findings and Management of Punctal Apposition Syndrome: Rare Cause of Epiphora.

ABSTRACT: The purpose of this study is to evaluate the diagnosis and treatment of punctal apposition syndrome. We retrospectively reviewed the medical records of 2 patients diagnosed with punctal apposition syndrome who underwent lateral tarsal strip procedure. The patients included 1 male and 1 female, with an average age of 76.5 years. Before surgery, both patients had epiphora symptoms with a Munk score of 4 in both eyes. in the primary gaze, it was noted that the upper and lower puncta were appose with each other in both eyes throughout the blinking cycle. On slit lamp examination, there were no keratoconjunctival diseases, and the shape of the puncta were normal. On fluorescein dye disappearance test, grade 3 findings were observed in both eyes of both patients. in dacryos-cintigraphy of the 2 patients, both eyes showed complete obstruction, However, no obstruction of the lacrimal duct system was found in probing and syringing tests. The patient's subjective symptoms improved after patching test. A lateral tarsal strip procedure was performed as a treatment and we obtain satisfactory results. Therefore, punctal apposition syndrome could be 1 of the causes of epiphora.

The forgotten lacrimal gland and lacrimal drainage apparatus: pictorial review of CT and MRI findings and differential diagnosis.

The lacrimal gland is a bilobed serous gland located in the superolateral aspect of the orbit. Lacrimal system pathologies can be broadly divided into pathologies of the lacrimal gland and those of the nasolacrimal drainage apparatus. These include distinct congenital, infectious, inflammatory, and benign, indeterminate, and malignant neoplastic lesions. Trauma and resultant fractures affecting lacrimal drainage apparatus is not part of this review; only non-traumatic diseases will be discussed. CT is the initial modality of choice because of its ability to delineate lacrimal system anatomy and demonstrate most lacrimal drainage system abnormalities and their extent. It also assesses bony architecture and characterizes any osseous changes. MRI is helpful in further characterizing these lesions and better assessing involvement of the surrounding soft tissue structures. In this pictorial review, we will review the anatomy of the lacrimal system, describe CT/MRI findings of the common and uncommon lacrimal system abnormalities and discuss relevance of imaging with regards to patient management.

Solitary fibrous tumour of lacrimal sac masquerading as lacrimal sac mucocele: a diagnostic and surgical dilemma.

Swellings in medial canthal area in eye most often involve the lacrimal sac, the commonest aetiology being chronic dacryocystitis. Very rarely, there is a deviation in the routine diagnosis when the pathology comes out to be a lacrimal sac tumour. We report the case of a young man who presented with a medial canthal mass not extending above the medial canthal ligament. The clinical presentation was typical for a lacrimal sac mucocele, however on histopathological evaluation was diagnosed as a rare case of solitary fibrous tumour of lacrimal sac. We recommend radiological investigation in medial canthal masses where there is negative regurgitation on pressure over lacrimal sac area to avoid misdiagnoses.

Response to Miles Kiernan Re: a rare case of mucoepidermoid carcinoma ex pleomorphic adenoma of the lacrimal gland.

Our goal is to contribute to the medical literature with our case of CEPA and hope to better help fellow clinicians diagnose and treat these rare tumors.

Persistent epiphora after endonasal ENT surgery: CT dacryography findings.

OBJECTIVES: To analyze postoperative CT dacryography features in patients with persistent epiphora after endonasal surgery. METHODS: We conducted a retrospective study of 76 patients with a history of persistent epiphora after endonasal ENT surgery who underwent CT dacryography between January 2014 and February 2020. Volume acquisition of sub-millimeter sections allowed 2D and 3D reconstructions with virtual endoscopy of the nasosinusal cavities and the lacrimal canal. RESULTS: The postsurgical appearance of the nasosinusal cavities revealed a middle meatal antrostomy in 37% of cases, less frequently an ethmoidectomy or an inferior meatal antrostomy, sometimes completed by a middle or inferior turbinectomy. In thirty-five patients (46%), the lacrimal canal was distant from the endonasal ENT procedure. Epiphora was related to mucosal hypertrophy, constricting all or part of the lacrimal canal. Thirty-three patients (43%) showed changes in the lacrimal canal at the surgical site. In the inferior meatus, the nasolacrimal orifice was sometimes involved in the inferior turbinectomy or meatotomy, but most of the time, in the middle meatus, resection of the uncinate process prior to ethmoidectomy or middle meatotomy was associated with a lesion of the contiguous lacrimal canal. CONCLUSION: As a rare cause of persistent tearing, involvement of the nasolacrimal duct at the edge of the endonasal ENT surgery highlights the importance of intraoperative localization of the nasolacrimal duct before resection of the uncinate process or the inferior turbinate, ideally predicted by preoperative CT imaging.

An Algorithm Recommendation to Detect Specific Pathology of the Lacrimal Sac.

ABSTRACT: We aimed to recommend an efficient algorithm to detect the presence of specific pathologies of the lacrimal sac (LS). The charts of 296 patients who had undergone LS biopsy were reviewed retrospectively. The age, gender, history, examination and radiological findings, sac appearance, and pathology results of the patients were recorded. The power of our data to predict the presence of potential specific pathology was identified by regression analysis. After the causality evaluation of the statistical results, an algorithm was created to differentiate specific LS pathologies from chronic dacryocystitis. Our algorithm was mainly formed by deciding on radiological examination and biopsy according to the risk scoring created by the examination findings. A specific LS pathology was observed in 11 (3.7%) patients. When we applied the recommended algorithm to cases with a suspicion of specific pathology and/or found to have a specific pathology, 36.4% of the patients would not require radiological examination and 29.6% of them would not require a biopsy. On the other hand, 80% of the frozen biopsies had been conducted on cases without a specific pathology. Managing the cases according to our algorithm would mean that frozen biopsy would be recommended in only 1 (12.5%) case without a specific pathology. Besides, the detection of cases with a specific pathology would not be prevented and the necessary diagnostic procedures would continue to be performed. Specific LS pathologies can be detected adequately, whereas decreasing unnecessary examinations and procedures in cases without a specific pathology by using our algorithm.

Primary apocrine adenocarcinoma of the lacrimal sac: an extremely rare variant of lacrimal sac neoplasm.

A 52-year-old male presented with right eye prominence of 1-year duration. Right eye examination revealed proptosis and limitation of extraocular movements. Magnetic resonance imaging (MRI) of the orbit showed soft tissue mass in the inferomedial orbit with medial rectus involvement and involving lacrimal sac. The patient underwent an incisional biopsy. Histopathology confirmed a diagnosis of primary orbital adenocarcinoma. Positron emission tomography computed tomography (PET CT) did not show the involvement of any other organ. Right orbital exenteration was done for disease control. On histopathology the mass was found to be arising from the lacrimal sac and was reported as apocrine adenocarcinoma. The diagnosis was further confirmed by immunohistochemistry with the tumor staining positive for gross cystic disease fluid protein 15 (GCDFP 15). The patient received adjuvant radiotherapy and is alive without any recurrence at 1 year of follow up. Apocrine adenocarcinomas are rare malignant skin adnexal tumors. Involvement of lacrimal sac is extremely rare with only one case reported in the literature till date. Immunohistochemistry utilizing GCDFP 15 stain can confirm the diagnosis.

Cutaneous pleomorphic adenoma of the periocular region - a case series.

Mixed tumour of the skin is a rare entity also known as chondroid syringoma and pleomorphic adenoma. These usually present as slow-growing skin nodules with a smooth surface, clear boundaries, and no ulceration. Case series exist describing pleomorphic adenomas in the periocular region including the lids and orbit, separate to the more familiar lacrimal gland pleomorphic adenoma. These may arise from accessory or ectopic lacrimal gland tissue but in the eyelids are more likely to arise from sweat glands in the skin. Histopathological analysis of these lesions is important to identify complete excision, minimising recurrences and in identifying rare but potential malignant transformation. We describe the clinical features and outcomes in three cases of pleomorphic adenoma with two at the medial canthus (including one recurrence) and one in the brow region.

Adenoid cystic carcinoma of the palpebral lobe of the lacrimal gland - case report and literature review.

Epithelial tumors of the lacrimal gland are rare and usually develop in the orbital lobe. We report the exceedingly rare occurrence of a primary adenoid cystic carcinoma in the palpebral lobe of the lacrimal gland. A 26-year-old female was referred for evaluation of a gradually enlarging mass in the lateral upper eyelid, previously diagnosed as a chalazion. Computed tomography revealed a heterogeneous round lesion anterior to the orbital rim. Excisional biopsy was compatible with an adenoid cystic carcinoma. After excluding distant metastasis, and as the patient refused adjuvant radiotherapy, a second surgical procedure, with wide local excision, was indicated. Follow-up showed no recurrence. This case highlights the importance of performing a thorough clinical examination when diagnosing any lateral upper eyelid mass. A high index of suspicion for malignant tumors of the lacrimal gland should always be maintained, and a complete excision with histological analysis should be preferred whenever possible.

Lacrimal gland pleomorphic adenoma with extensive necrosis.

Lacrimal gland pleomorphic adenomas (LGPA) are benign mixed tumors. Diagnosis is based on clinical and radiological findings which usually prompts complete excision of the lesion to minimise recurrence and a cumulative risk of malignant transformation. Necrosis in pleomorphic adenoma has been rarely reported in salivary gland PA, either spontaneously or due to iatrogenic interventions. Necrosis is suggestive of a malignant process and makes interpretation of histology specimens difficult. A 23 year old woman, while awaiting biopsy for a mass in the left lacrimal gland, which had been symptomatic for only several months, presented with acute pain and swelling of the left lateral lid. An incisional biopsy showed an inflamed lacrimal gland with focal necrosis and atypia of adjacent cytology and gland architecture. Subsequent excisional biopsy confirmed an LGPA with some inflammation but no necrosis. Necrosis may occur as an atypical presentation in LGPA.

A case of pleomorphic adenoma of the lacrimal gland invading the lower orbit.

Pleomorphic adenoma is a benign tumor that originates from the lacrimal gland and typically develops in the upper orbit. There is a risk of postoperative recurrence due to capsule damage by biopsy or incomplete tumor resection. Here, we report a case of primary lacrimal gland pleomorphic adenoma that extended to the lower orbit. A 76-year-old man visited a nearby clinic with swelling of the right eyelid, and was referred to our department because of decreased vision in the right eye and marked chemosis. At the initial presentation, external eye findings showed swelling of the right eyelid, and elastic hard masses were palpable beneath the skin of the upper and lower eyelids. Magnetic resonance imaging revealed a multinodular tumor in the upper and lower orbits, measuring about 2 cm in the long axis. Excision was attempted by a transcutaneous approach from the upper eyelid, and the tumor was totally removed as a mass without damage to the capsule. The orbital mass was histologically diagnosed as pleomorphic adenoma of the lacrimal gland. We encountered a rare morphological variation of pleomorphic adenoma of the lacrimal gland that extended to the lower orbit. When diagnosing large tumors extending to the upper and lower orbits, total tumor resection should be attempted without biopsy due to the possibility of pleomorphic adenoma.

Chronic unilateral tearing as a sign of lacrimal sac squamous cell carcinoma.

Tearing is a very common symptom in ophthalmic practice; however, this issue is commonly overlooked. The authors describe the case of a patient with chronic unilateral epiphora that had been neglected for 8 y; lacrimal system workup was performed for this patient only because of dacryocystitis evolvement. Following the diagnosis of nasolacrimal duct blockage, dacryocystorhinostomy was indicated. Intraoperative abnormal mucosa was subjected to biopsy, and a diagnosis of squamous cell carcinoma in the lacrimal sac was established. Extended tumor excision was performed for the patient and adjuvant radiotherapy was administered, without any recurrence till the 2-year follow-up. The present report highlights the relevance of a detailed evaluation for determining the underlying causes of tearing, especially in chronic unilateral presentation. The consideration of potential malignancy in such cases could prevent delayed diagnosis of uncommon but potentially life-threatening malignancies of the lacrimal drainage system.