Lacrimal Gland Adenocarcinoma Clinicopathologic Features and Outcomes Compared With Those of Lacrimal Gland Adenoid Cystic Carcinoma.

PURPOSE: Lacrimal gland (LG) adenocarcinomas (ACs) are rare, with limited data. We compared clinicopathologic features and local recurrence, distant metastasis, and survival rates between LG AC and LG adenoid cystic carcinoma (ACC). METHODS: The records of LG AC patients treated from 2008 to 2022 and LG ACC patients treated from 1998 to 2022 at the same center were retrospectively reviewed. RESULTS: The study included 20 patients with AC; 10 de-novo AC, 10 ex-pleomorphic AC; and 51 ACC patients. The median age at diagnosis was 61 years for de-novo AC, 54 years for ex-pleomorphic AC, and 45 years for ACC. All groups had male predominance. The initial T category was T2 in 50% (5/10) of de-novo ACs; 60% (6/10) of ex-pleomorphic ACs; and 59% (30/51) of ACCs. Perineural invasion was present in 33% (5/15) of ACs and 90% (45/50) of ACCs ( p < 0.001). Of the 20 AC patients, 14 had eye-sparing surgery; 4 had orbital exenteration; and 2 had unresectable disease. All AC patients received postoperative radiotherapy and 15 (75%) received concurrent chemotherapy. Fourteen AC patients were tested for human growth factor receptor 2 expression, and 10 (71%) were human growth factor receptor 2 positive; 5 received human growth factor receptor 2-targeted therapy. AC and ACC had similar 5-year recurrence rates (20% and 33%, respectively, p = 0.31) and metastasis rates (20% and 34%, respectively, p = 0.30). de-novo AC, ex-pleomorphic AC, and ACC had similar 5-year disease-specific survival rates (80%, 79%, and 81%, respectively, p > 0.99). CONCLUSIONS: LG AC and ACC have similar baseline clinicopathologic features, except that perineural invasion is more common in ACC, and similar recurrence, metastasis, and survival rates. Human growth factor receptor 2-targeted therapy may be appropriate in some patients with LG AC.

Post-traumatic canalicular fistula: description and review of literature - SALDO update study (SUP) - paper II.

PURPOSE: To discuss the clinical spectrum and management strategies in patients with post-traumatic canalicular fistula (PTCF). METHODS: Retrospective, interventional case series of consecutive patients diagnosed with PTCF over a 6-year study period between June 2016 and June 2022. The demographics, mode of injury, location, and communication of the canalicular fistula were noted. The outcomes of several management modalities including dacryocystorhinostomy, lacrimal gland therapies, and conservative approaches were assessed. RESULTS: Eleven cases with PTCF over the study period were included. The mean age at presentation was 23.5 years (range: 6-71 years), with male: female ratio of 8:3. The median time interval between trauma to presentation at the Dacryology clinic was 3 years (range: 1 week to 12 years). Seven had iatrogenic trauma and four had the canalicular fistula following primary trauma. Management modalities pursued include conservative approach for minimal symptoms, and dacryocystorhinostomy, dacryocystectomy, and lacrimal gland botulinum toxin injection. The mean follow-up period was 30 months (range: 3-months-6 years). CONCLUSION: PTCF is a complex lacrimal condition and the management of the PTCF needs a tailored approach guided by its nature and location and patient symptomatology.

[Cell technologies as a basis for the development of regenerative principles for the treatment of lacrimal gland diseases]. / Kletochnye tekhnologii kak osnova razrabotki regeneratornykh printsipov lecheniya zabolevanii sleznoi zhelezy.

The lacrimal gland (LG) is a tubuloacinar exocrine gland composed of acinar, ductal, and myoepithelial cells. Three-dimensional distribution of acinar lobules, ducts, and myoepithelial cells is necessary for the effective functioning of the organ. LG is the main organ of immune surveillance of the ocular surface system. The embryogenesis of the gland is regulated by the interaction of genetic mechanisms, internal epigenetic (enzyme systems, hormones) and exogenous factors. There is no doubt that there is a clear genetic program for the implementation of the complex process of embryonic development. The mechanisms regulating LG organogenesis initiate the work of a huge number of structural oncogenes, transcription and growth factors, etc. Studying the expression and selective activity of regulatory genes during organ development, their participation in the differentiation of different cell types is a current trend at the nexus of clinical genetics, molecular biology, embryology and immunocytochemistry. Due to its relatively simple structure and accessibility, human LG is a suitable object for potential application in regenerative medicine. Development of a universal protocol for obtaining functional differentiated secretory epithelium of LG capable of expressing tissue-specific markers is an urgent task. Determining the nature and origin of stem cells and progenitor cells will allow the isolation and multiplication of these cells in culture. After obtaining a functionally active culture of LG cells, it is possible to create a model of autoimmune diseases.

Microbial Reconstitution Improves Aging-Driven Lacrimal Gland Circadian Dysfunction.

Lacrimal glands are highly susceptible to aging and exhibit age-related structural and functional alterations. However, the mechanisms by which aging affects the lacrimal glands are not well-established. The current study explores the crosstalk between the aging process, gut microbiota, and circadian rhythm in age-associated lacrimal gland dysfunction. C57BL/6J mice were divided into young, old, and fecal microbiota transplant (FMT)-treated old groups. The gut bacterial community diversity was analyzed by 16S rRNA sequencing. Exorbital lacrimal glands (ELGs) were collected at 3-hour intervals over a 24-hour circadian cycle, and total RNA was subjected to high-throughput sequencing. Rhythmic transcriptional data were analyzed using the Jonckheere-Terpstra-Kendall algorithm and bioinformatics analysis technology. Immunostaining was used to identify lymphocytic infiltration, lipid deposition, and nerve innervation in the ELGs. Compared with young mice, old mice underwent a significant gut microbial community shift. The rhythmically transcriptomic profile was significantly reprogrammed over a 24-hour cycle in the old ELG group. Intervention with serial FMT from young donors for 1 month rejuvinated the gut microbial community of the old mice. Most alterations in rhythmic transcriptomic profiling were improved. Furthermore, chronic inflammation, lipid deposition, and aberrant neural response of the aging lacrimal glands were significantly reduced. Thus, the study shows that reconstitution of age-associated gut dysbiosis with FMTs from young donors improves aging-driven lacrimal gland circadian dysfunction.

New strategies for the management of ocular surface disease in glaucoma patients.

PURPOSE OF REVIEW: Glaucoma patients commonly suffer from ocular surface disease (OSD). As treatment strategies, medications and devices for the treatment of OSD as well as glaucoma surgical approaches evolve rapidly, it is important to consider their application to these patients. RECENT FINDINGS: OSD in glaucoma patients may lead to reduced reliability of diagnostic tests, decreased medication compliance, poor surgical outcomes, and overall decreased quality of life. Chronic use of topical glaucoma medications has been linked to the development of limbal stem cell deficiency, and the role of preservatives in OSD continues to be demonstrated. Preservative free glaucoma medications as well as new anti-inflammatory agents for the treatment of OSD are now available. Omega-3 fatty acid supplementation and punctal plugs have been shown to benefit glaucoma patients with OSD. Drop burden may be reduced through the use of the new sustained-release delivery systems, selective laser trabeculoplasty, and minimally invasive glaucoma surgery. SUMMARY: There are multiple emerging strategies for managing OSD that may be applied to patients with glaucoma. With continued research and clinical experiences, we hope to better understand the multifaceted relationship between glaucoma and OSD and develop evidence-based algorithms for the management of these complex patients.

Dacryops with extensive lacrimal and maxillary bone defects in four dogs.

OBJECTIVE: To describe and discuss ventromedial orbital lacrimal gland or duct cysts (dacryops) in dogs with extensive bone defects based on their symptoms, results of diagnostic imaging and histopathological examination, and therapy and discuss their potential origin based on the morphology. ANIMALS STUDIED: Four dogs of different breeds, age, and sex were presented with a unilateral round, slow growing, indolent, and non-tender process ventromedial to the nasal canthus of the eye. PROCEDURES: Transverse computed tomography showed a low-density, non-contrast-enhancing cystic process ventromedial to the globe with extensive defects in the lacrimal and maxillary bones in all cases. The cystic character of the structure was confirmed by the aspiration of the brownish fluid without cellular and microbiological contents. For treatment, the cystic fluid was aspirated, and the sclerosing agent polidocanol was injected in three cases. Cystorhinostomy (nasal marsupialisation) was performed in one case as the first choice and in another case following failure of sclerotherapy. Histopathological examination of the cyst walls was performed in two cases and confirmed the diagnosis of dacryops. RESULTS: Follow-up between 2 and 18 months showed no recurrence and very good to excellent cosmetic results. CONCLUSIONS: Aberrant lacrimal gland or duct tissue with secondary development of dacryops should be included in the differential diagnoses of ventromedial orbital cysts. Large cysts near the lacrimal drainage system with extensive bone defects in dogs should be treated by nasal marsupialization. Treatments such as evacuation of the cyst and inducing sclerosis (sclerotherapy) should be reserved for exceptional cases.

Primary Malignant Epithelial Tumors of the Lacrimal Drainage System: A Major Review.

Purpose: To perform a literature review on the primary malignant epithelial tumors (PMET) involving the lacrimal drainage system (LDS).Methods: The authors performed a PubMed search of all articles published in English on primary malignant epithelial tumors of the lacrimal sac and the nasolacrimal duct. Data analyzed include prevalence, demographics, clinical presentation, histopathological subtypes, management modalities, outcomes, and recurrence rates.Results: Malignant epithelial tumors of the lacrimal sac are commonly diagnosed in the fifth to seventh decades of life (mean age: 58 years) and have no gender predilection. Typical clinical presentation includes epiphora (84%) and lacrimal fossa mass lesion (78%). Pain (9%) and blood-stained epiphora (4%) were inconsistent findings. Among the PMET, squamous cell carcinomas (SCCs) were the most common (61%) followed by transitional cell carcinomas (TCC; 15%), and mucoepidermoid carcinoma (MEC; 7%). Complete surgical excision followed by radiotherapy (59%) was the preferred modality of management, and only 18% required orbital exenteration. Of the 331 tumors with available treatment details, 85% survived at a mean follow-up of 33 months. The overall recurrence rate following treatment was 20% (65/331). MECs of the lacrimal sac had the best while adenoid cystic carcinoma had the worst prognosis.Conclusion: Blood-stained epiphora was not the classical presentation for primary epithelial malignancies of the LDS. No validated staging system exists for lacrimal drainage tumors. There is a need for consistent and detailed reporting in the literature, more so on the histological subtypes and their management.

[Chinese expert consensus on the diagnosis and treatment of epithelial tumors of the lacrimal gland (2021)].

Orbital tumors frequently occur in the regions where the lacrimal gland is located. Lacrimal gland tumors account for about 10% of orbital space-occupying lesions. Among them, 20% to 50% were epithelial tumors, with benign tumors accounting for 55% and malignant tumors accounting for 45%. Epithelial lacrimal gland tumors usually have a special location with relatively distinctive manifestations, and the treatment principle is different from other orbital space-occupying lesions. Improper treatment often leads to recurrence and even malignant transformation, which compromise the prognosis and life quality of patients. In view of this, the Oculoplastic and Orbital Disease Group of Chinese Ophthalmological Society has put forward the expert consensus on the diagnosis and treatment of epithelial lacrimal gland tumors after serious discussion, in order to improve the level of diagnosis and treatment of this disease in China. (Chin J Ophthalmol, 2021, 57: 658-662).

Malignant Mixed Tumor (Carcinoma Ex Pleomorphic Adenoma) of the Lacrimal Gland.

PURPOSE: To review the clinical presentation, treatment, and prognosis of patients with malignant mixed tumor (carcinoma ex pleomorphic adenoma) of the lacrimal gland. METHODS: Clinical records and radiographic images were reviewed for patients with malignant mixed tumor of the lacrimal gland treated at the center during 2008-2019. RESULTS: The study included 9 patients (6 men, 3 women) aged 17-66 years (median age, 56 years). Six had primary malignant mixed tumor with no history of orbital lesions, and 3 had previously been diagnosed with pleomorphic adenoma. Tumor, Node, Metastasis classification per the eighth edition of the American Joint Committee on Cancer (AJCC) Cancer Staging Manual were T1aN0M0 in 2 patients, T2aN0M0 in 3 patients, T4bN0M0 in 2 patients, and T4cN0M0 in 2 patients. Two patients underwent orbital exenteration, 6 patients underwent eye-sparing surgery, and 1 patient had an unresectable tumor because of cavernous sinus extension. All patients received radiotherapy (intensity-modulated radiotherapy in 3 and proton therapy in 6). All patients received chemotherapy, 8 concurrently with radiotherapy and 1 after radiotherapy. The median follow-up time was 70 months. At last contact, 6 patients were alive without evidence of disease; 2 had died of disease, 1 of distant metastasis, and the other of cavernous sinus invasion. CONCLUSIONS: The findings suggest that de novo malignant mixed tumor of the lacrimal gland is more common than disease that results from transformation after incomplete resection of lacrimal gland pleomorphic adenoma. Most cases can be treated with eye-sparing surgery and radiation unless skull base extension is present.

Lymphoproliferative tumors involving the lacrimal drainage system: a major review.

PURPOSE: To provide a literature review on lymphoproliferative lesions involving the lacrimal drainage system. METHODS: The authors performed a pubmed search of all articles published in English on lymphoma/leukemia of lacrimal sac and the nasolacrimal duct. Data analyzed include prevalence, demographics, clinical presentations, treatment outcomes of primary versus secondary lacrimal involvement, and recurrence rates. RESULTS: Lymphoma/leukemia of lacrimal sac presented at a mean age of 55 years. The majority of the tumors (63%) were primary involvement of lacrimal sac, bilateral involvement being more common in secondary than primary lacrimal sac lymphoma. Epiphora (96%, 98/102), swelling in the lacrimal sac region (75%, 77/102), and acute dacryocystitis (31%) were the most frequent presenting features. Thirty-six percent of the lesions were diagnosed incidentally while performing a dacryocystorhinostomy (DCR). Among primary sac lymphomas, diffuse large B-cell lymphoma (DLBCL) (43%) was the most common sub-type followed by MALToma (24%), unclassified B-cell lymphoma (21%), lymphoid hyperplasia (5%) and 3% each small lymphocytic lymphoma (SLL) and NK/T cell lymphoma. Management usually involves chemotherapy and/ or radiotherapy with or without surgical resection. Successful outcomes in terms of local disease control could be achieved in all the cases; however, 15% died of the systemic disease after a mean 18 months from the time of sac involvement. Aggressive lymphomas like NK/T-cell have the worst prognosis. CONCLUSION: Lymphoproliferative involvement of lacrimal sac has a high incidence of acute dacryocystitis with a good response to chemotherapy. Epiphora in patients with leukemia/lymphoma should arouse suspicion of a lacrimal drainage involvement.

Poorly differentiated primary adenocarcinoma of the lacrimal sac and the nasolacrimal duct.

Primary adenocarcinoma of the lacrimal drainage system is a rare malignancy. Poorly differentiated subtypes of this tumor demonstrate aggressive behaviors, and metastasis is not uncommon. We present a patient with poorly differentiated adenocarcinoma of the lacrimal sac and nasolacrimal duct with an orbital extension. The case was managed by a combined external and endoscopic approach excision followed by adjuvant radiotherapy.

[Disorders of the Lacrimal Apparatus, Part 2: Basics of Therapy]. / Erkrankungen des Tränenapparats, Teil 2: Grundlagen der Therapie.

The tearing eye (epiphora) is deemed to be the leading symptome of efferent tear duct stenosis. Nevertheless, epiphora might be caused by ocular surface pathologies or even intraocular diseases. A distinguished anamnesis and sufficient clinical examination is most meaningful for the differential diagnostic distinction. Therapy is based on pathologic changes. In case of ocular surface disease, a suitable tear substitution and antiinflammatory approach is mandatory. Multifarious triggers have to be considered as well. Addressing tear film distribution, suitable oculoplastic surgery is required. Efferent tear duct stenosis necessitates surgical treatment as the level of suffering is often very high and acute exacerbations may develop. Besides recanalization, anastomosing techniques represent the available therapeutic principles. Possible surgical approaches offer a great variety and many influencing factors navigate therapy decisions. This part of the review comments on the most important therapeutic approaches for lacrimal apparatus diseases.

[Clinical and pathological analysis of 64 patients with primary neoplasms of the lacrimal drainage system].

Objective: To analyze the pathological classification and age distribution of primary neoplasms of the lacrimal drainage system. Methods: Retrospective case series study. A total of 64 patients (65 eyes) were diagnosed with primary neoplasms of the lacrimal drainage system and received surgery at Tianjin Eye Hospital from January 2006 to December 2016. All the clinical data of the patients were analyzed, including gender, diseased eye, age, clinical manifestations, composition of benign and malignant masses, and prognosis. The histopathological composition and age distribution of patients with primary lacrimal mass, lacrimal duct mass and lacrimal sac mass were analyzed according to the different diseased sites. Results: Twenty-three patients (24 eyes) were male, and 41 patients (41 eyes) were female. The right eye was involved in 36 patients, the left eye in 27 patients, and both eyes in one patient. The age at diagnosis ranged from 12 to 78 years old [mean, (46±4) years]. The course of disease was (13.1±4.2) months, ranging from 1 month to 7 years. The chief complaint was tear discharge in 43 patients, tumor in 19 patients, and abscess discharge in 2 patients. There were 29 patients with angular displacement and 21 patients with swelling pain. There were 51 patients with benign lesions, 4 with borderline lesions, and 9 with malignantlesions. These neoplasms consisted of primary peripunctal neoplasms in 17 patients, primary canalicular neoplasms in 2 patients, and primary lacrimal sac neoplasms in 45 patients. All primary peripunctal neoplasms (17 cases) were benign, among which nevi (10 cases) occupied the first place. All primary canalicular neoplasms (2 cases) were benign, there were 1 case of epidermoid cysts and 1 case of degenerative disease. Among the primary benign lacrimal sac masses (32 cases), mucous epithelial cysts (9 cases), dermoid cysts (6 cases), and epidermoid cysts (6 cases) occupied the first three places. Among the primary borderlin lacrimal sac masses (4 cases), there were 2 cases of giant cell tumor of soft tissue, 1 case of solitary fibrous tumor, and 1 case of inflammatory myofibroblastic tumor. The primary malignant mass of lacrimal sac (9 cases) was dominated by squamous cell carcinoma (3 cases). In terms of age distribution, the patients with primary peripunctal mass were mainly in the group of 40-59 years old (14 cases). The primary benign mass of lacrimal sac mainly occurred in the group of less than 40 years old (15 cases) and the group of 40-59 years old (11 cases). The patients with primary lacrimal sac borderline and malignant masses were all in the groups of over 40 years old. A total of 49 patients were followed up for 27 months to 16 years. The average follow-up time was (57.2±3.8) months. Lacrimal sac transitional cell carcinoma relapsed 7 months after surgery in one patient, and lacrimal sac melanoma relapsed 1 year after surgery in one patient. The patients did not relapse in 24 months and 38 months after surgery respectively. There were no recurrence of other cases. Conclusions: Primary peripunctal neoplasms are mostly characterized with benign lesions, among which nevi are most common. Mucous epithelial cysts, epidermoid cysts, and dermoid cysts are the major benign lacrimal sac neoplasms. Squamous cell carcinomas are the most common malignant lacrimal sac neoplasms. The malignant tumor of lacrimal sac often occurs in the middle-aged and elderly patients. (Chin J Ophthalmol, 2020, 56: 364-369).

[Retrospective investigation of spontaneous bloody tears: a report of 27 cases].

Objective: To summarize clinical experience on the clinical feature, etiology and treatment of patients with spontaneous bloody tears as the initial symptom. Methods: Retrospective series of case studies. The clinical data and follow-up data of 27 cases of bloody tears as the first symptom in Lacrimal Center of Ophthalmology, the Third Medical Center of Chinese PLA General Hospital from June 2015 to December 2018 were reviewed. The clinical feature, specific cause, diagnosis, treatment and prognosis of these cases were evaluated. Results: A total of 27 cases were collected in this study. The patients were 10 males (37.0%) and 17 females (63.0%), including 21 adults (≥ 18 years old, 77.8%) and 6 minors (<18 years old, 22.2%). There were 22 monocular cases (81.5%) and 5 binocular cases (18.5%). Five cases (18.5%) were bleeding from the eye and other parts of the body, and 22 cases (81.5%) were bleeding only from the eye. There were 19 cases (70.4%) with hematic epiphora and secretions from the punctum, 3 cases (11.1%) with blood-stained tears, and 7 cases (25.9%) with blood-like tears. With regard to etiology, 6 cases (22.2%) were combined with systemic lesions, one of which was granulomatosis with polyangiitis and five of which (<18 years old) were idiopathic bloody tears. Twenty-one cases (77.8%) were local lesions, including 18 cases only involving the lacrimal system, 2 cases only involving the ocular surface, and 1 case involving both the lacrimal system and the ocular surface. Among the 21 cases with local lesions, 5 cases were induced by foreign body, 6 cases were induced by simple inflammation, and 10 cases were induced by tumor including 1 case with conjunctival benign tumor and 9 cases with tumor of the lacrimal system (5 with malignant tumor and 4 with benign tumor). Patients with idiopathic bloody tears received psychological and medical treatment, and interictal discharge was lengthened. One case of granulomatosis with polyangiitis was treated by trans-nasal endoscopic dacryocystorhinostomy. With the recurrence of granulomatosis and polyangiitis, bloody tears recurred after surgery. One patient with conjunctival hemangioma was untreated. Lesions in the lacrimal duct system were removed and dacryocystorhinostomy was performed. In this study, 2 patients (1 with small cell neuroendocrine carcinoma and 1 with adenoid cystic adenocarcinoma) died and the other had a good prognosis. Conclusions: Among the cases of bloody tears, adults and local lesions are more common. Most of the lesions are located in the lacrimal system and are tumors. The main treatment is to remove the lesions, and if necessary, to expand the resection and reconstruct the lacrimal duct. Idiopathic bloody tears occur in minors, who are gave psychotherapy and necessary medical treatment. (Chin J Ophthalmol, 2020, 56: 53-58).

Multimodal therapy in the management of lacrimal gland adenoid cystic carcinoma.

BACKGROUND: To evaluate the prognosis of Chinese patients with lacrimal gland adenoid cystic carcinoma treated with eye-sparing surgery and adjuvant multimodal therapy. METHODS: The study included 24 consecutive patients with lacrimal gland adenoid cystic carcinoma treated at the Ninth People's Hospital of Shanghai from May 2008 to September 2017. All patients underwent eye-sparing surgical tumor resection and 20 (83.3%) of the 24 patients in the cohort received postoperative RT. Eight (41.7%) patients in the cohort received chemotherapy. Each patient's medical records were reviewed. RESULTS: The study included 13 male and 11 female patients. The median follow-up time after surgery was 33.5 months. Fifteen (62.5%) patients experienced local recurrence. The 1-, 3-, and 5-year recurrence rates were 27.9, 60.0, and 80.0%, respectively. Eleven (45.8%) patients developed metastasis. The 1-, 3-, and 5-year metastasis rates were 8.7, 48.5, and 66.9%, respectively. Eight (33.3%) patients died of lacrimal gland adenoid cystic carcinoma, with a median survival duration of 34.0 months. The 1-, 3-, and 5-year tumor-related mortality was 4.5, 28.1, and 58.0%, respectively. More advanced T stage (≥ T3a) was a risk factor for local recurrence (hazard ratio [HR]: 5.374, P = 0.02), distant metastasis (HR: 8.585, P < 0.01), and tumor-related survival (HR: 9.654, P < 0.01). CONCLUSIONS: Eye-sparing tumor resection protocol followed by adjuvant therapy seems to be associated with high rates of local recurrence, metastases and death. In addition, greater attention should be paid to patients with lacrimal gland adenoid cystic carcinoma with ≥ T3a tumors.

A Staged Procedure in the Treatment of Primary Lacrimal Sac Epithelial Malignancy: A Retrospective Cases Analysis.

PURPOSE: To determine the effect of a staged procedure in the treatment of primary lacrimal sac epithelial malignancy. METHODS: This is a retrospective case series of 18 consecutive patients with primary lacrimal sac epithelial malignancy treated at an orbital tumor referral center between 2002 and 2017. Study was conducted in compliance with the Declaration of Helsinki. All patients underwent biopsy of the mass to confirm the diagnosis pathologically. Chemotherapy concurrent with radiotherapy was delivered to the patients to reduce and concrete the tumor prior to surgery. En bloc resection of the lacrimal sac malignancy and nasolacrimal duct was followed. RESULTS: Eleven patients were male and 7 patients were female. The median follow-up time was 72.2 months. Nine patients had squamous cell carcinoma, 7 poorly differentiated carcinoma, 1 transitional cell carcinoma, and 1 adenoid cystic carcinoma. After chemotherapy and radiotherapy, the tumor volume was reduced significantly (p < 0.0001). En bloc resection of the lacrimal sac malignancy was performed in all patients with concurrent partial ethmoidectomy in 8 patients and medial maxillectomy in 5 patients. One patient (5.6%) suffered from adenoid cystic carcinoma died of metastatic disease. Two patients (11.1%) with local recurrence received reoperation, and 1 patient (5.6%) with pulmonary metastasis received gamma knife radiosurgery. These patients are alive with no evidence of tumor. Other patients are alive without evidence of disease at last follow up. No patient had new onset of lymph node enlargement during and after the treatment. CONCLUSIONS: The staged procedure is a promising method for the treatment of primary lacrimal sac epithelial malignancy with no postoperative lymph node metastasis.

INI1/SMARCB1-Deficient Carcinoma (Rhabdoid Tumor) of the Lacrimal Gland.

Integrase interactor 1 (INI1) is a tumor suppressor gene that is ubiquitously expressed in all nucleated cells. The loss of INI1 protein activity was first demonstrated in aggressive pediatric tumors, including atypical teratoid/rhabdoid (AT/RT) tumor of the central nervous system and malignant rhabdoid tumor of the kidney. Subsequently, INI1 deficiency was discovered in other pediatric and some adult neoplasms. The spectrum of INI1-negative tumors includes a wide variety of neoplasms that occur over a wide age range, are variably aggressive, and have a variable rhabdoid component on histopathologic evaluation. In this report, the authors describe a 27-year-old gravid woman with INI1-deficient carcinoma of the lacrimal gland, previously not described in this location.

Mantle Cell Lymphoma of the Lacrimal Gland.

The authors present 2 female patients of mantle cell lymphoma (MCL) involving the lacrimal gland. The patients include a 75-year-old female and a 59-year-old female. Both patients presented with palpable upper eyelid masses. The first patient was previously diagnosed with gastric and ileocecal MCLs and underwent chemotherapy with remission. The second patient had distant lesions in the muscular limbs that were confirmed to be MCL after the diagnosis of lacrimal gland MCL. Both patients underwent chemotherapy with complete remission.

[Treatment Options for Adenoid Cystic Lacrimal Gland Carcinoma - A Case Series and Literature Review]. / Therapieoptionen bei adenoidzystischem Tränendrüsenkarzinom ­ Fallserie und aktueller Literaturüberblick.

Adenoid cystic carcinoma of the lacrimal gland (ACC) is a rare malignant orbital tumour with a poor overall prognosis. Current therapeutic approaches like radical and local surgery, adjuvant radiation and neoadjuvant intra-arterial chemotherapy are controversial. We present three cases of patients with ACC with different therapy concepts and discuss these with current recommendations from the literature.

Clinical analysis of 90 cases of malignant lacrimal sac tumor.

PURPOSE: To investigate the pathology, clinical manifestations, and potential risk factors associated with the prognosis of malignant lacrimal sac tumors. In addition, the treatment outcomes and complications were also evaluated. METHODS: Ninety cases of malignant lacrimal sac tumors were retrospectively analyzed at our hospital. Pathological classifications, clinical manifestations, risk factors, and follow-up time were documented. The outcomes and complications were evaluated and compared among the various treatment modalities. RESULTS: The median follow-up time was 50 months (range, 3-258 months). The 5-year overall survival (OS) and progression-free survival (PFS) for all cases were 85.7 and 77.9%, respectively. The 5-year OS and PFS for 69 cases of squamous cell carcinoma were 87.6 and 76.3%, and which were 80.4 and 72.4% for 21 cases of non-squamous cell carcinoma, respectively. There was no difference of 5-year OS and PFS between squamous cell carcinoma and non-squamous cell carcinoma (p = 0.350 and p = 0.946). Positive lymph node status was associated with worse OS (p < 0.001) and PFS (p = 0.020). For the 23.3% of cases (21/90) treated with the definitive radiotherapy, the outcomes were equivalent to that of surgery combined with radiotherapy, with the incidence of treatment-related visual acuity complication not being significant. The addition of chemotherapy to the treatment course had a marginal and non-significant improvement in OS and distant metastasis-free survival. CONCLUSIONS: Lymph node status was found to be a key factor for prognosis. Advanced tumors could benefit from multimodality treatment, with radiotherapy playing an important role. However, the role of chemotherapy requires further investigation.