Imani M Williams, Vamsee K Neerukonda, Anna M Stagner
{"title":"The Histopathology of Two Eyes Enucleated after Continuous Transscleral and Micropulse Transscleral Cyclophotocoagulation for Refractory Secondary Glaucoma.","authors":"Imani M Williams, Vamsee K Neerukonda, Anna M Stagner","doi":"10.1159/000521739","DOIUrl":"https://doi.org/10.1159/000521739","url":null,"abstract":"<p><strong>Introduction: </strong>Cyclodestructive procedures, which target the nonpigmented epithelium of the ciliary body, have been utilized to treat recalcitrant glaucoma since the early 1930s. There are now various types of cyclophotocoagulation (CPC) available. The authors provide a retrospective description that details the histopathologic findings in 2 patients who underwent CPC for uncontrolled uveitic and neovascular glaucoma (NVG) with subsequent enucleation.</p><p><strong>Case presentations: </strong>Two enucleated globes from 2 patients with secondary refractory glaucoma underwent cilioablative therapy: one with uveitic glaucoma and a remote history of micropulse transscleral CPC (MP-TSCPC) and the other with NVG and a recent history of traditional continuous transscleral CPC (CW-TSCPC). The clinical histories are summarized, and light microscopy reviewed for degree of coagulative necrosis and inflammation of the ciliary body and surrounding structures, as well as the underlying pathology of the glaucoma.</p><p><strong>Conclusion: </strong>Both patients ultimately experienced pain and vision loss with either a recrudescence of elevated intraocular pressure or inflammatory hypotony and subsequently underwent enucleation of the affected eye. One globe was enucleated shortly after CW-TSCPC and found to have near full-thickness coagulative necrosis of the pigmented and nonpigmented ciliary epithelium and ciliary muscle as well as necrosis of adjacent nontarget tissues with fibrin in the anterior chamber. The second patient underwent enucleation many months after MP-TSCPC with partial healing fibrosis of the ciliary body and some remaining viable ciliary processes. The histopathologic findings post-CPC may vary based on the method used and evolve over time; additional study is needed.</p>","PeriodicalId":19434,"journal":{"name":"Ocular Oncology and Pathology","volume":"8 2","pages":"93-99"},"PeriodicalIF":1.0,"publicationDate":"2022-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9218618/pdf/oop-0008-0093.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9560888","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Hardeep Singh Mudhar, Sachin S Salvi, Daniel Pissaloux, Arnaud de La Fouchardiere
{"title":"Single Time Frame Overview of the Genetic Changes in Conjunctival Melanoma from Intraepithelial Disease to Invasive Melanoma: A Study of 4 Exenteration Specimens Illustrating the Potential Role of Cyclin D1.","authors":"Hardeep Singh Mudhar, Sachin S Salvi, Daniel Pissaloux, Arnaud de La Fouchardiere","doi":"10.1159/000520953","DOIUrl":"https://doi.org/10.1159/000520953","url":null,"abstract":"<p><strong>Introduction: </strong>Despite advances in the understanding of the molecular pathogenesis of cutaneous melanoma, relatively little is known about the genetic changes that occur in the progression of conjunctival melanocytic intraepithelial lesions to invasive conjunctival melanoma.</p><p><strong>Methods: </strong>We exposed 4 exenteration specimens that each contained varying grades of intraepithelial conjunctival melanocytic neoplasia and invasive neoplasia to a combination of various techniques, including array comparative genomic hybridization (aCGH), ribonucleic acid sequencing (RNA-seq), fluorescence in situ hybridization (FISH), and immunohistochemistry.</p><p><strong>Results: </strong>Three out of 4 of the invasive melanomas showed gains in 11q13 (CCND1 locus) by aCGH. FISH demonstrated CCND1 gain in invasive melanoma and in conjunctival melanocytic intraepithelial lesions (CMILs) of all grades (low-grade CMILs and in situ melanoma), and this was paralleled by increased expression of Cyclin D1 protein within the atypical melanocytes by immunohistochemistry, using a double-staining method with a red end point for Melan A cytoplasmic staining and a brown end point for nuclear Cyclin D1 expression. Higher grades of melanocytic intraepithelial lesions showed more cells expressing Cyclin D1 than lower grade melanocytic intraepithelial lesions. The Cyclin D1 protein expression was in the same location as the amplified CCND1 signal by FISH. One out of 3 of these cases also showed the amplification of the 12q13-15 locus corresponding to MDM2 and FISH confirmed gains in the conjunctival melanocytic intraepithelial neoplasia and invasive melanoma. The remaining fourth case showed a homozygous deletion of 9p21 (CDKN2A) by aCGH only, with immunohistochemistry showing clonal loss of p16 protein expression in the invasive and conjunctival melanocytic intraepithelial lesion. Two out of 4 of the invasive melanomas harboured classical driver mutations in NRAS and NF-1, respectively. None of the cases showed mutations in BRAF, KIT, and TERT mutations. RNA-seq data showed secondary mutations in ARAF, PLCB4, MET, EZH2, MAP2K2, CTNNB1, CIITA, NF2, TP53, and MEN1, some of which are implicated in the MAPK pathway.</p><p><strong>Conclusion: </strong>CMILs harbour amplifications of CCND1 (3 cases), MDM2 (1 case), and loss of CDKN2A (1 case), which are also present when the lesion progresses to invasive melanoma, implicating these amplifications in the early pathogenesis of CMILs. This study represents the first attempt to capture the mutational landscape of all stages of conjunctival melanoma in a single tissue excision.</p>","PeriodicalId":19434,"journal":{"name":"Ocular Oncology and Pathology","volume":"8 1","pages":"52-63"},"PeriodicalIF":1.0,"publicationDate":"2022-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8914236/pdf/oop-0008-0052.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10596008","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ahmad Abdel-Aty, Wendy L Linderman, Ninani Kombo, John Sinard, Renelle Pointdujour-Lim
{"title":"Necrotic Uveal Melanoma Mimics Orbital Cellulitis: A Review.","authors":"Ahmad Abdel-Aty, Wendy L Linderman, Ninani Kombo, John Sinard, Renelle Pointdujour-Lim","doi":"10.1159/000515558","DOIUrl":"https://doi.org/10.1159/000515558","url":null,"abstract":"<p><strong>Background: </strong>Uveal melanoma is the most common primary intraocular malignancy in adults, often resulting in painless vision loss. We report a case of necrotic uveal melanoma presenting with orbital inflammation mimicking orbital cellulitis and present a comprehensive review of the literature and tabulation of reported cases.</p><p><strong>Summary: </strong>Our review found 44 published reports of spontaneously necrotic uveal melanoma involving 55 patients. Of these reports, 26 patients (47%) presented with orbital cellulitis. Presenting symptoms of necrotic uveal melanoma with orbital cellulitis included proptosis (82.8%), pain (80.7%), vision loss (61.5%), and restricted extraocular movements (46.2%).</p><p><strong>Key messages: </strong>Uveal melanoma can rarely mimic orbital cellulitis. Autoinfarction and tumor necrosis causes secondary orbital inflammation. Intraocular malignancy must remain in the differential for patients with orbital inflammation and vision loss.</p>","PeriodicalId":19434,"journal":{"name":"Ocular Oncology and Pathology","volume":"8 1","pages":"1-8"},"PeriodicalIF":1.0,"publicationDate":"2022-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8914239/pdf/oop-0008-0001.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10595541","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Front & Back Matter","authors":"Arun D. Singh, H. Grossniklaus","doi":"10.1159/000522614","DOIUrl":"https://doi.org/10.1159/000522614","url":null,"abstract":"","PeriodicalId":19434,"journal":{"name":"Ocular Oncology and Pathology","volume":"21 1","pages":""},"PeriodicalIF":1.0,"publicationDate":"2022-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"78510321","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Sabrina Schlüter, Norbert Bornfeld, Elbrus Valiyev, Dirk Flühs, Martin Stuschke, Nikolaos E Bechrakis, Tobias Kiefer, Petra Ketteler, Sophia Göricke, Eva M Biewald
{"title":"Combination of Brachytherapy and Intravitreal Chemotherapy in the Treatment of Retinoblastoma with Vitreous Seeding.","authors":"Sabrina Schlüter, Norbert Bornfeld, Elbrus Valiyev, Dirk Flühs, Martin Stuschke, Nikolaos E Bechrakis, Tobias Kiefer, Petra Ketteler, Sophia Göricke, Eva M Biewald","doi":"10.1159/000520952","DOIUrl":"https://doi.org/10.1159/000520952","url":null,"abstract":"<p><strong>Purpose: </strong>The aim of this study was to report the efficacy of combined intravitreal chemotherapy (IVC) and ruthenium-106 brachytherapy in retinoblastoma, either as first-line or second-line treatment, following systemic chemoreduction or intra-arterial chemotherapy.</p><p><strong>Methods: </strong>Retrospective data of 18 eyes from 18 patients treated with IVC and brachytherapy from August 2014 to December 2019 were collected.</p><p><strong>Results: </strong>The method described was our first-line therapy in 6 patients, whereas it was used as second-line treatment after chemoreduction in the remaining 12 patients. The eyes showed the following classification at initial presentation: 2 group B eyes, 3 group C eyes, and 13 group D eyes. The mean follow-up was 19.5 months (range 2-53 months). The mean patient age at brachytherapy was 34.0 months (range 15-83 months). The median prescribed dose at the tumour base and apex was 574.5 ± 306.7 Gy and 88.5 ± 12.2 Gy, respectively. The ocular retention rate was 66.7%. Six eyes had to be enucleated due to uncontrollable subretinal and recurrent vitreous seeding, tumour relapse, recurrence of a solid tumour elsewhere in the eye, and persistent vitreous bleeding with loss of tumour control. The mean number of intravitreal injections of melphalan was 5.0. Two patients received a simultaneous injection of topotecan for insufficient therapeutic response. With regard to radiogenic complications, we could observe temporary retinal and vitreous bleeding (27.8%), serous retinal detachment (44.4%), and radiogenic maculopathy and retinopathy (11.1%). None of the children showed metastatic disease during follow-up.</p><p><strong>Conclusion: </strong>Ruthenium-106 plaque therapy in combination with IVC is an effective local therapy with good tumour control rates even in advanced eyes. Overall, the analysed therapeutic approach shows an acceptable side-effect profile, especially when considering that external-beam radiation therapy and systemic polychemotherapy or at least the number of cycles needed, with their increased incidence of adverse events, can thus be avoided.</p>","PeriodicalId":19434,"journal":{"name":"Ocular Oncology and Pathology","volume":"8 1","pages":"64-70"},"PeriodicalIF":1.0,"publicationDate":"2022-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8914268/pdf/oop-0008-0064.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10596007","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Sepideh Siadati, Ashley A Campbell, Timothy McCulley, Charles G Eberhart
{"title":"Clinicopathological Features of 19 Eyelid Pilomatrixomas.","authors":"Sepideh Siadati, Ashley A Campbell, Timothy McCulley, Charles G Eberhart","doi":"10.1159/000520219","DOIUrl":"https://doi.org/10.1159/000520219","url":null,"abstract":"<p><strong>Introduction: </strong>Pilomatrixoma is a relatively rare, benign tumor arising from the hair root matrix. It is found frequently on the head and neck, with most involving the eyebrow in the periocular region. In contrast, eyelid pilomatrixoma is less common, and often clinically misdiagnosed. Here, we present clinical and histological data from 19 pilomatrixomas arising in the eyelid.</p><p><strong>Methods: </strong>The study represents a retrospective study of eyelid pilomatrixoma diagnosed at our institution since 1981. All slides were reviewed, and demographic as well as clinical data were obtained.</p><p><strong>Results: </strong>Patient ages ranged from 2 to 63 years (mean 24 years), including 12 (63%) females and 7 (37%) males. Eight (42%) and 4 (21%) cases arose in the first and second decades of life, respectively. Upper eyelid involvement was found in 14 (74%) of cases. Microscopically, the tumors were characterized by basaloid and shadow cells accompanied by calcification and foreign body giant cells.</p><p><strong>Conclusions: </strong>Eyelid pilomatrixoma is rarely suspected clinically, and can be mistaken for cyst, chalazion, sebaceous carcinoma, and other tumors. Physicians should consider the possibility of pilomatrixoma in the eyelid area, especially in children or young female patients. Complete excision is curative, and diagnosis can generally be established by histopathological examination.</p>","PeriodicalId":19434,"journal":{"name":"Ocular Oncology and Pathology","volume":"8 1","pages":"30-34"},"PeriodicalIF":1.0,"publicationDate":"2022-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8914276/pdf/oop-0008-0030.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10596005","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Alessandro Marchese, Maria Vittoria Cicinelli, Francesco Bandello, Giulio Modorati, Elisabetta Miserocchi
{"title":"Hemorrhagic Mass-Like Presentation of Vitreoretinal Lymphoma.","authors":"Alessandro Marchese, Maria Vittoria Cicinelli, Francesco Bandello, Giulio Modorati, Elisabetta Miserocchi","doi":"10.1159/000519300","DOIUrl":"https://doi.org/10.1159/000519300","url":null,"abstract":"<p><strong>Purpose: </strong>The aim of this study was to report the presentation, treatment, and outcomes of vitreoretinal lymphoma (VRL) associated with hemorrhagic mass-like lesions (HMLs) in the retina.</p><p><strong>Methods: </strong>This study was a retrospective analysis of patients with HMLs associated with VRL seen at a single tertiary referral center. For each patient, the clinical charts, the fundus imaging, and the treatment outcomes were reviewed.</p><p><strong>Results: </strong>Three eyes of 2 patients had VRL with HMLs. In all study eyes, HMLs were preceded by an area of retinitis-like retinal infiltration and evolved into elevated hemorrhagic masses. Two eyes had multiple relapses with HMLs. All HMLs regressed with treatment and were replaced by extensive chorioretinal atrophy.</p><p><strong>Conclusion: </strong>VRL can present with HMLs. HMLs seem to correspond to massive intraretinal infiltration by VRL, mimicking a solid mass. Despite response to therapy, HMLs are associated with poor anatomical and functional outcomes.</p>","PeriodicalId":19434,"journal":{"name":"Ocular Oncology and Pathology","volume":"8 1","pages":"9-15"},"PeriodicalIF":1.0,"publicationDate":"2022-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8914238/pdf/oop-0008-0009.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10596009","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Emily C Zabor, Vishal Raval, Shiming Luo, David E Pelayes, Arun D Singh
{"title":"A Prediction Model to Discriminate Small Choroidal Melanoma from Choroidal Nevus.","authors":"Emily C Zabor, Vishal Raval, Shiming Luo, David E Pelayes, Arun D Singh","doi":"10.1159/000521541","DOIUrl":"https://doi.org/10.1159/000521541","url":null,"abstract":"<p><strong>Objective: </strong>This study aimed to develop a validated machine learning model to diagnose small choroidal melanoma.</p><p><strong>Design: </strong>This is a cohort study.</p><p><strong>Subjects participants and/or controls: </strong>The training data included 123 patients diagnosed as small choroidal melanocytic tumor (5.0-16.0 mm in largest basal diameter and 1.0 mm-2.5 mm in height; Collaborative Ocular Melanoma Study criteria). Those diagnosed as melanoma (<i>n</i> = 61) had either documented growth or pathologic confirmation. Sixty-two patients with stable lesions classified as choroidal nevus were used as negative controls. The external validation dataset included 240 patients managed at a different tertiary clinic, also with small choroidal melanocytic tumor, observed for malignant growth.</p><p><strong>Methods: </strong>In the training data, lasso logistic regression was used to select variables for inclusion in the final model for the association with melanoma versus choroidal nevus. Internal and external validation was performed to assess model performance.</p><p><strong>Main outcome measures: </strong>The main outcome measure is the predicted probability of small choroidal melanoma.</p><p><strong>Results: </strong>Distance to optic disc ≥3 mm and drusen were associated with decreased odds of melanoma, whereas male versus female sex, increased height, subretinal fluid, and orange pigment were associated with increased odds of choroidal melanoma. The area under the receiver operating characteristic \"discrimination value\" for this model was 0.880. The top four variables that were most frequently selected for inclusion in the model on internal validation, implying their importance as predictors of melanoma, were subretinal fluid, height, distance to optic disc, and orange pigment. When tested against the validation data, the prediction model could distinguish between choroidal nevus and melanoma with a high discrimination of 0.861. The final prediction model was converted into an online calculator to generate predicted probability of melanoma.</p><p><strong>Conclusions: </strong>To minimize diagnostic uncertainty, a machine learning-based diagnostic prediction calculator can be readily applied for decision-making and counseling patients with small choroidal melanoma.</p>","PeriodicalId":19434,"journal":{"name":"Ocular Oncology and Pathology","volume":"8 1","pages":"71-78"},"PeriodicalIF":1.0,"publicationDate":"2022-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8914269/pdf/oop-0008-0071.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10589294","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Andrew W Stacey, Vaidehi S Dedania, Miguel Materin, Hakan Demirci
{"title":"Improved Prognostic Precision in Uveal Melanoma through a Combined Score of Clinical Stage and Molecular Prognostication.","authors":"Andrew W Stacey, Vaidehi S Dedania, Miguel Materin, Hakan Demirci","doi":"10.1159/000520218","DOIUrl":"https://doi.org/10.1159/000520218","url":null,"abstract":"<p><strong>Introduction: </strong>Prognosis of uveal melanoma (UM) is assessed using clinical staging or molecular testing. Two modalities often used for prognostication are the American Joint Committee on Cancer (AJCC) staging and a tumor gene expression profile (GEP), the outcomes of which are often discordant. This article discusses a total risk score created to combine the discordant information from both sources.</p><p><strong>Methods: </strong>A retrospective case series was conducted of all patients presenting with UM over 6 years to 2 referral centers. Each tumor was classified using the AJCC and the GEP. A total risk score was calculated for each patient using results from both AJCC and GEP. Kaplan-Meier analysis of metastasis-free survival was used to compare groups.</p><p><strong>Results: </strong>A total of 294 patients were included in the study. Kaplan-Meier estimates showed significant curve separation between individual AJCC and GEP risk groups. The combined total risk score provided an accurate estimate of prognosis that incorporated results from both AJCC and GEP.</p><p><strong>Conclusions: </strong>Clinical staging and molecular prognostication of UM can be discordant. There is important information provided by each system that is not provided by the other. The total risk score provides a simple method to combine information from both AJCC stage and the GEP class in order to provide patients and care teams with a more complete understanding of metastatic risk.</p>","PeriodicalId":19434,"journal":{"name":"Ocular Oncology and Pathology","volume":"8 1","pages":"35-41"},"PeriodicalIF":1.0,"publicationDate":"2022-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8914271/pdf/oop-0008-0035.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10589295","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Lacrimal Sac Tumors: A Case Series.","authors":"Syeed Mehbub Ul Kadir, Riffat Rashid, Sadia Sultana, Murtuza Nuruddin, Mst Sayedatun Nessa, Mukti Rani Mitra, Golam Haider","doi":"10.1159/000520086","DOIUrl":"https://doi.org/10.1159/000520086","url":null,"abstract":"<p><strong>Objective: </strong>The aim of the study was to describe the clinical presentation, management strategies, and outcomes in a case series of primary lacrimal sac tumors.</p><p><strong>Methods: </strong>This retrospective study was conducted in Sheikh Fajilatunnessa Mujib Eye Hospital and Training Institute, Bangladesh, from July 1 to December 31, 2020, and included all patients who were evaluated, treated, and followed up for at least 6 months from January 2013 to October 2020. One patient developed a recurrence of the adenocarcinoma of the lacrimal sac after 1 year of primary treatment. Patients' demographic data were analyzed and reviewed from published articles on lacrimal sac tumors. We assessed patients clinically, followed by radiological evaluation. We also analyzed the biopsy technique, treatment modality, and recurrence. An oncologist reviewed all patients to prepare a plan for adjuvant treatment.</p><p><strong>Results: </strong>Ten patients with lacrimal sac tumors were included in this study. Swelling in the medial canthal region was the most common presenting feature (100%), followed by epiphora (60%) and pain (30%). Open biopsy was preferred over fine-needle aspiration biopsy. Incisional biopsy or complete excisional biopsy was performed for all suspected malignancies. Malignant tumors were found in 7 (70%) cases, and benign tumors in 3 (30%) cases. Non-Hodgkin's lymphoma (NHL) (40%) was the most common malignant lacrimal sac tumor. Mucosa-associated lymphoid tissue lymphoma was 75%, and diffuse large B-cell lymphoma was 25% among the cases of NHL. Patients with epithelial malignancy were treated with external beam radiation therapy, while NHL patients were treated with chemotherapy (CHOP regimen). Recurrence was noted in 1 case (10%) of epithelial malignancy after 1 year of treatment.</p><p><strong>Conclusion: </strong>Successful management of lacrimal sac tumors requires a high index of suspicion, as these are fatal tumors, often misdiagnosed as dacryocystitis. Nonepithelial malignancies are more predominant than epithelial malignancies, and hematolymphoid tumors are most frequent.</p>","PeriodicalId":19434,"journal":{"name":"Ocular Oncology and Pathology","volume":"8 1","pages":"42-51"},"PeriodicalIF":1.0,"publicationDate":"2022-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8914267/pdf/oop-0008-0042.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10589296","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}