Intravascular papillary endothelial hyperplasia of the maxillary sinus extending into the contralateral nasal cavity.

INTRODUCTION: Intravascular papillary endothelial hyperplasia (IPEH) predominantly occurs in the subcutaneous and dermal regions and rarely originates from the sinonasal mucosa. CASE PRESENTATION: We report on the case of a 58-year-old male patient who presented with progressive bilateral nasal obstruction, left-sided epiphora, and intermittent epistaxis. Computed tomography revealed a soft tissue opacity in the left maxillary sinus with intersinusoidal nasal wall demineralization, extending into the surrounding ethmoid cells and the right nasal cavity through a contralateral deviation of the nasal septum. Contrast-enhanced T1-weighted magnetic resonance imaging further confirmed these findings. The IPEH originating from the maxillary sinus extended into the contralateral nasal cavity, and it was successfully removed using an endoscopic endonasal approach, avoiding overly aggressive treatment. CONCLUSION: This case report highlights the diagnostic challenges of IPEH in the sinonasal region and the importance of considering IPEH as a differential diagnosis in patients presenting with nasal obstruction, epiphora, and intermittent epistaxis.

Primary Lacrimal Sac Tumors with Extension into Vicinity: Outcomes with Endoscopy-Assisted Modified Weber-Ferguson's Approach.

PURPOSE: To evaluate the outcomes of endoscopy-assisted modified Weber-Ferguson's approach in the management of primary lacrimal sac tumors with extension into the neighboring tissues. METHODS: A retrospective interventional study was performed on all patients with lacrimal sac tumors treated with the endoscopy-assisted modified Weber-Ferguson approach between January 2010 and June 2022 at the Shanghai Ninth People's Hospital, China. Data assessed include demographics, clinical presentations, imaging features, surgical techniques, histopathology, adjuvant modalities of management, complications, and outcomes. RESULTS: A total of 13 patients were included in the analysis. Epiphora and palpable mass lesion were the presenting complaint in 84.6% (11/13) of the patients. Nearly half of the patients (46.1%, 6/13) were misdiagnosed as lacrimal duct obstruction. All the lacrimal sac tumors in the present series showed uneven enhancement on T1-weighted MRI imaging. Postoperatively, 84.6% (11/13) patients recovered well with excellent esthetics and were disease-free after a mean follow-up of 58.6 months. Two patients who underwent additional exenteration developed recurrence and succumbed (at 41 and 96 months follow up) while they were on palliative chemoradiation. CONCLUSION: The endoscopic-assisted modified Weber-Fergusson surgical approach is effective in providing better visibility and accessibility to lacrimal sac tumors with extension into neighboring tissue.

Neoadjuvant Intra-arterial Cytoreductive Chemotherapy Improves Outcomes in Lacrimal Gland Adenoid Cystic Carcinoma.

BACKGROUND: Lacrimal gland adenoid cystic carcinoma (LGACC) has historically been associated with a poor prognosis even with localized disease, with a survival of 56% at 5 years. In 1988, we treated the first patient with neoadjuvant intra-arterial cytoreductive chemotherapy (IACC). Since then, we have used this protocol as the standard approach. We aim to analyze the outcomes of patients with LGACC treated with the protocol and compare them to a population-based cohort to assess if IACC can improve survival. METHODS: We prospectively assessed all non-metastatic patients with LGACC treated with IACC at a single institution between 1988 and 2021. For a comparison group, we identified all non-metastatic patients with LGACC treated with excision from the Surveillance, Epidemiology, and End Results (SEER) registry. We calculated disease-specific survival using the Kaplan-Meier and Cox proportional-hazards modeling methods. RESULTS: Thirty-five non-metastatic patients with LGACC treated with IACC were identified at a single institution, and 64 patients with non-metastatic LGACC treated with excision were identified in the SEER database. The 5- and 10-year disease-specific survival rates for patients treated with IACC were 84% (95%CI 71-97) and 76% (95%CI 60-92), respectively. While the 5- and 10-year disease-specific survival rates for the population-based cohort were 72% (95%CI 62-82) and 46% (95%CI 32-60). The survival analysis favored IACC, with a 60% lower risk of death (HR: 0.4; 95%CI 0.2-0.9). CONCLUSION: IACC improves disease-specific survival in comparison to a population-based cohort treated with excision. Additional patients treated with IACC at multiple institutions are required to provide further external validity.

A Pilot Study of Dynamic Magnetic Resonance Dacryocystography Imaging to Assess Functional Epiphora.

OBJECTIVE: To evaluate dynamic magnetic resonance dacryocystography (MRDCG) in eyes with functional epiphora. METHODS: We included prospective eyes with epiphora if no alternative cause was found on clinical examination, were patent on syringing, had no obstruction or stenosis on DCG, and had an abnormal DSG. MRDCG was performed to qualitatively assess for block or patency and quantitatively measure tear transit time. We compared measurements to asymptomatic fellow eyes and to historical reference values from asymptomatic eyes. RESULTS: We included 26 symptomatic eyes of 19 patients (median age 63 years). There was a block on MRDCG in 18 (69%) eyes and patency in 8 (31%) eyes. The block occurred at the sac-nasolacrimal duct (NLD) junction in 9 (50%), proximal NLD in 5 (28%), mid-NLD in 1 (5.6%), and distal NLD in 1 (5.6%) eye(s). No contrast was observed in the lacrimal system in two eyes. For eyes patent on MRDCG, median times to the sac, NLD, inferior meatus, first 25%, and first 50% of the fundus-to-nose distance (FND) were 22, 54, 118, 34, and 84 s, respectively. Times to the sac, NLD, and to fill the first 25% and 50% of the FND were significantly longer than historical values from asymptomatic lacrimal systems (p = 0.017, 0.050, 0.035, 0.017, respectively). CONCLUSION: MRDCG shows a high rate of block in functional epiphora. However, DSG and MRDCG results may not always correlate. The improved temporal resolution of this emerging modality may be advantageous in the critical first 2 min of tear transit.

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.

Metastatic HER2 lacrimal/salivary gland duct adenocarcinoma.

In this report, we present a case of a patient with a 30-year history of orbital asymmetry who presented with metastatic human epidermal growth factor receptor 2 (HER2) positive lacrimal/salivary gland ductal adenocarcinoma. The patient was treated with chemoradiotherapy and trastuzumab. Tumours of lacrimal gland origin are rare, and unfortunately can frequently present in late stage. There are no current guidelines on the optimal treatment of metastatic lacrimal gland tumours, in particular those with HER2 amplified malignancy. This case highlights a unique presentation of a rare disease, and the potential for targeted therapy.

Neutrophil Extracellular Traps Drive Dacryolithiasis.

Mucopeptide concretions, previously called dacryoliths, are macroscopic stones that commonly obstruct the lacrimal sac. The mechanism behind dacryolithiasis remains unclear; however, the involvement of various immune cells, including neutrophils, has been confirmed. These findings remain limited, and no information on neutrophil extracellular traps (NETs), essentially involved in the pathogenesis of other lithiases, is available yet. Here, we employ microcomputed tomography, magnetic resonance tomography, histochemistry, mass spectrometry, and enzyme activity analyses to investigate the role of neutrophils and NETs in dacryolithiasis. We classify mucopeptide concretions into three types, with respect to the quantity of cellular and acellular material, polysaccharides, and mucosubstances. We propose the role of neutrophils and NETs within the existing model of gradual formation and growth of mucopeptide concretions, with neutrophils contributing to the initial stages of dacryolithiasis, as they localized on the inner (older) parts of the tissue. As NETs localized on the outer (newer) parts of the tissue, we link their role to the late stages of dacryolithiasis, presumably maintaining the proinflammatory environment and preventing efficient clearance. An abundance of IgG on the surface indicates the involvement of the adaptive immune system later as well. These findings bring new perspectives on dacryolithiasis, in which the innate and adaptive immune system are essentially involved.

A case report: An unusual presentation of adenoid cystic carcinoma of the lacrimal gland.

RATIONALE: Adenoid cystic carcinoma (ACC) is the most common malignant epithelial tumor of the lacrimal gland with the highest malignant degree. ACC of the lacrimal gland is characterized by symptoms of <1 years duration. We present a 38-year-old male patient who complained of an enlarging mass in the left lacrimal fossa for almost 10 years previous to the diagnosis of ACC. PATIENT CONCERNS: A 38-year-old male patient visited our ophthalmology clinic with a chief complaint of a mass in his left upper lid, which had enlarged significantly over the previous months. DIAGNOSES: Magnetic resonance imaging with intravenous Gadobutrol showed moderate and homogenous mass enhancement. Bone destruction is found. The periosteum is not eroded. The magnetic resonance imaging finding was supportive for malignancy. Histopathological examination of the specimen revealed solid tumor showing a cribriform pattern mixed small amount of basaloid cell proliferation. Therefore, the final diagnose was Adenoid cystic carcinoma of the lacrimal gland. INTERVENTIONS: The treatment included en bloc resection of the mass and adjacent bone and radiotherapy. OUTCOMES: In 1 year follow-up after operation, there is no recurrence. Visual acuity is 30/30. The left eye shows limitation on abduction. LESSONS: The present case demonstrates an unusual progression of ACC of the Lacrimal Gland.

Blood-stained tears-a red flag for malignancy?

BACKGROUND: Blood-stained tears can indicate occult malignancy of the lacrimal drainage apparatus. This study reviews data on patients presenting with blood in their tears and the underlying cause for this rare symptom. METHODS: Patients presenting with blood in their tears, identified over a 20-year period, were retrospectively collected from a single tertiary ophthalmic hospital's database and analysed. RESULTS: 51 patients were identified, the majority female (58%) with a mean age of 55 years. Most cases were unilateral (96%) with blood originating from the nasolacrimal drainage system in 53%. The most common diagnosis for blood-stained tears was a lacrimal sac mucocele (n = 16) followed by a conjunctival vascular lesion (n = 4). Three patients had systemic haematological disorders. The rate of malignancy was 8% (n = 4), with 2 patients having lacrimal sac transitional cell carcinomas, one with a lacrimal sac plasmacytoma and the other with chronic lymphocytic leukaemia and bilateral orbital infiltration (with bilateral bloody tears). One patient had a lacrimal sac inverted papilloma, a premalignant lesion. Four patients had benign papillomas (of the lacrimal sac, conjunctiva and caruncle). CONCLUSION: Haemolacria was a red flag for malignancy in 8% of patients (and tumours in 18% of patients). A thorough clinical examination including lid eversion identified a conjunctival, caruncle, eyelid or canalicular cause in 27% of cases.

Clinical Analysis of Primary Malignant Lacrimal Sac Tumors: A Case Series Study With a Comparison to the Previously Published Literature.

PURPOSE: To report management of a series of patients with primary malignant lacrimal sac tumors and to compare these results to the previously published literature. MATERIALS AND METHODS: A total of 27 patients with pathologically confirmed primary malignant lacrimal sac lesions were enrolled into this study. Pathological classifications, clinical characteristics, various treatment modalities and follow-up time, including tumor recurrence, were documented. The outcome measures included overall survival, progression-free survival, and median survival time. RESULTS: Among 27 eligible cases, 33.33% (9/27) of the tumor was non-Hodgkin B-cell lymphoma, and 33.33% (9/27) was squamous cell carcinoma; both were the most common tumor in this series, followed by adenocarcinoma 18.52% (5/27), then melanoma 7.41% (2/27). Treatment modalities included surgery, radiotherapy, and/or chemotherapy, the overall survival rate of 27 patients was 70.37%, with a median follow-up of 45 months (range: 7 mo-16 y), 8 patients had died from metastatic disease, but 13 patients remained without evidence of recurrent tumor. The 5-year overall survival and progression-free survival for all cases were 73.33% and 66.67%, respectively. The median survival time for 5 deceased patients with interstitial brachytherapy was 98 months, and 5-year survival rate was 60%. CONCLUSIONS: In this series, among primary malignant lacrimal sac tumors, the proportion of lymphoma had increased when compared with the previously published literature, and multidisciplinary therapy may lead to a good prognosis in the majority of patients with the tumors and patients may benefit more from interstitial brachytherapy than external beam radiotherapy.

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.

Recurrent lacrimal gland pleomorphic adenoma.

Pleomorphic adenoma is the most common tumour of the lacrimal gland. Correct preoperative diagnosis is essential as the tumour should be removed en bloc. Incisional biopsy is contraindicated due to risk of recurrence and subsequent risk of malignancy. We identified 13 patients who were diagnosed with lacrimal gland pleomorphic adenoma in our institution over a 31-year period from 1990 to 2021. Of these, we describe four patients, three males and one female, who underwent incisional biopsy. One of these patients had a number of recurrences and required an orbital exenteration. The other two were treated with radiation to the orbit and experienced severe dry eye post-radiotherapy. All three patients are currently disease-free. The fourth patient underwent an incisional biopsy of his lacrimal gland pleomorphic adenoma (LGPA) within the last 6 months and has been referred for radiotherapy. The reasons for a lack of preoperative diagnosis of LGPA included clinical uncertainty and broad radiologic differential. None of the patients who had an en bloc resection experienced recurrence. We recommend that a fine needle aspirate (FNA) biopsy in lieu of a tissue biopsy should be performed where LGPA is included in the clinical and radiological differential diagnosis. If incisional biopsy is required, the suture track should be marked so that the entire biopsy track is removed with the specimen.

Stereotactic radiosurgery for recurrent pleomorphic adenoma of the lacrimal gland: a case report.

Lacrimal gland pleomorphic adenomas (LGPAs) are common, benign, and intraorbital tumours that cause exophthalmos, ptosis, and visual disturbances. The curative treatment for LGPAs is gross total resection, and radiotherapy is considered adjunctive for recurrence or an alternative for inoperable LGPAs. Stereotactic radiosurgery (SRS) can be used for precise delivery of high radiation doses to the tumour, crucial in the treatment of intra-and extracranial neoplasms. Here, we present a 95-year-old woman who had a rapidly growing, recurrent LGPA and was successfully treated with SRS. The tumour was controlled without any adverse events over 21 months following SRS. SRS is a potential alternative treatment for recurrent LGPA.

Clinico-radiologic characteristics of lacrimal sac area swellings misdiagnosed as dacryocystocele or mucocele.

PURPOSE: Several non-lacrimal lesions can present with lacrimal sac area swelling mimicking a dacryocystocele or mucocele with a possibility of misdiagnosis. This study investigates the clinic-radiologic characteristics of the mimicking conditions compared to true lacrimal sac distension. DESIGN: Retrospective, comparative, interventional case series. PARTICIPANTS: Patients referred by primary care ophthalmologists between January 2015 and October 2021 with a misdiagnosis of dacryocystocele or lacrimal sac mucocele (n = 39) and an age-matched group of proven true lacrimal sac swelling (TLS group, n = 44). METHODS: Data collected included demographics, presenting features, investigations, management, histopathology, and outcomes. Both groups were statistically compared for several clinical and radiological variables. RESULTS: Final diagnoses in the mimicking group were skin/subcutaneous swellings (14/39, 35.9%), vascular malformations (10/39, 25.6%), inferomedial anterior orbital cysts (7/39, 17.9%), sino-orbital masses (5/39, 12.8%). Female gender (<0.001), epiphora (p = 0.001), and discharge (p < 0.001) were significantly more frequent in the TLS group. The mimicking group was more likely to be associated with a longer swelling duration (p < 0.001), a swelling extending beyond the lacrimal sac area (p <0.001), orbital signs (p <0.001), and periorbital abnormality. Non-patent lacrimal irrigation (p < 0.001) was significantly more frequent in the TLS group. On imaging, all swellings in the mimicking group were separate from the lacrimal pathway and 94.9% (37/39) extended beyond the lacrimal sac fossa. CONCLUSION: Various cutaneous, subcutaneous, vascular, inferomedial orbital cystic, and sinonasal pathologies can present with lacrimal sac area swelling and mimic a TLS. A high index of suspicion, a thorough clinical evaluation and proper imaging are essential to avoid a misdiagnosis.

Lacrimal gland biopsies-results from a tertiary centre in the UK.

PURPOSE: To report the histopathological results of lacrimal gland biopsies over a 21-year period in a tertiary referral centre in the United Kingdom. To the best of our knowledge, this represents the largest series to be published in the United Kingdom. METHODS: A retrospective observational review was carried out for patients who underwent lacrimal gland biopsies in a tertiary referral centre at the University Hospitals of Leicester, United Kingdom between the years of 2000 and 2021. RESULTS: Lacrimal gland biopsies were performed on 248 patients during the specified 21-year period. They comprised 157 (63.3%) females and 91 (36.7%) males. The mean age at presentation was 50.8 years (range 15-94 years). The majority of patients were Caucasian (69.4%, n = 172) followed by Asians (25.0%, n = 62), African/Afro-Caribbean (4.8%, n = 12) and other ethnicities (0.8%, n = 2). The most common histopathological diagnosis was chronic inflammation dacryoadenitis (69.0%, n = 171) followed by lymphomas (15.3%, n = 38). CONCLUSION: Our study shows that chronic inflammation accounts for the majority of histopathological diagnosis followed by lymphoproliferative disorders.

Cutaneous eyelid melanoma with lacrimal sac metastasis: the potential role of lacrimal fluid as a metastatic pathway.

Here we present a patient with cutaneous eyelid melanoma patient with lacrimal sac metastasis. Clinicopathological findings in this case support the theory that lacrimal fluid can be a metastatic pathway for tumour cells. Dermatologists should be aware of the possibility that cutaneous eyelid melanoma may involve the nasolacrimal system and should examine it during the perioperative period and in postoperative follow-up.