[Severe Conjunctival and Corneal Epithelial Dysplasia: A Case Series]. / Schwere konjunktivale und korneale epitheliale Dysplasie: eine Fallserie.

BACKGROUND/OBJECTIVES: Ocular surface squamous neoplasia (OSSN) are among the most frequent non-pigmented malignancies of the ocular surface. They have a wide range of histological characteristics - ranging from mild epithelial dysplasia to invasive carcinoma of the squamous cells of the cornea. They may be restricted to the conjunctiva or also involve the cornea. As there are no leading symptoms in the early stages, diagnosis may be very delayed in patients who do not receive regular ophthalmological treatment. The present case series describes clinical and histological data on OSSN and includes clinical and histological data on OSSN, including possible clinical presentations, important risk factors, special histological and cytological features and therapeutic options. METHODS: Retrospective case series of patients with histologically confirmed severe epithelial dysplasia of the conjunctiva and cornea consistent with OSSN who presented to the Department of Ophthalmology in Basel University Hospital. The analysis covered demographic data, symptoms, diagnostic testing (photo documentation, brush biopsy), treatment and cytological and/or histological material and findings. RESULTS: We report on five patients aged between 41 and 92 years at the time of diagnosis. The histological findings in all patients included severe epithelial dysplasia, but with a heterogenous clinical presentation. In all cases, the lesion started in the conjunctiva, but traversed the limbus and extended to the cornea. The primary treatment was always surgical removal. In one patient, this had to be repeated several times due to recurrent metaplasia and was complemented by subsequent mitomycin C therapy. The clinical outcome ranged between total restitution of the original state to inevitable enucleation. CONCLUSION: The clinical presentation of OSSN is highly heterogenous, so that the initial diagnosis is difficult. There are no official guidelines for treatment, so that the treatment of choice varied between clinics. Regular ophthalmological follow-ups are recommended, even after complete surgical excision. Possible relevant concomitant diseases and risk factors must be identified before therapy.

[A giant malignant melanoma of the palpebral conjunctiva: a case report].

An 81-year-old female patient experienced a rapid increase in the volume of a rice-sized black mass on the left eye over a period of six months. The mass extended out of the eye and exhibited surface erosion with accompanying hemorrhage. Following a live tissue examination and histopathology after orbital exenteration under general anesthesia, the diagnosis of a giant malignant palpebral conjunctival melanoma of the spindle cell subtype was confirmed. The patient refused to undergo local radiation therapy or systemic chemotherapy and died from the disease six months later.

Epidemiological and histopathological aspects of ocular melanomas in Northeastern Romania.

Ocular melanoma is a rare but complex disease in current medical practice. Our retrospective study spans over a period of 28 years and analyzed uveal and conjunctival melanomas that were consecutively admitted, diagnosed, and treated in the 2nd Ophthalmology Clinic of Prof. Dr. Nicolae Oblu Emergency Clinical Hospital, Iasi, Romania. The patients were selected from the records of the Department of Pathology of our Hospital, being diagnosed by standard histopathological techniques. The aim of this study was to summarize the epidemiological and pathological aspects of uveal and conjunctival melanomas in Northeastern region of Romania. In our study, we did not notice a predilection of uveal and conjunctival melanoma to one particular gender. The most common histological subtypes of ocular melanomas were the heavily pigmented spindle cell subtype, followed by the epithelioid subtype. Our patients sought medical help in a timely manner, before the systemic invasion of the disease could develop.

Conjunctival myxoma: A case report and review of a rare tumor.

RATIONALE: Conjunctival myxoma is a rare benign tumor, which can mimic more common conjunctival lesions such as a cyst, lymphangioma, amelanotic nevus, neurofibroma, amelanotic melanoma, or lipoma. We describe a patient with the conjunctival myxoma, who was initially misdiagnosed as a conjunctival cyst. This case report includes intraoperative photographs and various immunohistochemical staining images. PATIENTS CONCERNS: A 55-year-old woman presented with a painless mass in the superotemporal conjunctiva of the left eye, which she had noticed 1 month ago. The patient had no previous history of trauma or eye surgery. Slit-lamp examination revealed a well-circumscribed, freely movable, pinkish, semi-translucent mass on the temporal bulbar conjunctiva, suggestive of a conjunctival cyst. DIAGNOSES: Histopathological analysis showed stellate- and spindle-shaped cells within the loose myxoid stroma, confirming a diagnosis of conjunctival myxoma. INTERVENTIONS: The conjunctival lesion was completely excised under local anesthesia. OUTCOMES: After 4 months of follow-up, the patient remained in good health without recurrence of the conjunctival lesion and no evidence of any systemic abnormality. LESSONS: Myxoma is an extremely uncommon benign tumor derived from primitive mesenchyme. Considering the rarity of the tumor and its similarity to other conjunctival tumors, diagnosis can be challenging. Ophthalmologists should consider myxoma as a possible differential diagnosis when encountering conjunctival lesions. Surgical excision is essential to confirm the diagnosis and careful systemic evaluation is required to prevent potentially life-threatening underlying systemic conditions.

[Evaluation of the accuracy of clinical diagnosis of conjunctival neoplasms : Comparison with histological findings after excision]. / Untersuchung der Genauigkeit klinischer Diagnosen von Neoplasien der Konjunktiva : Vergleich mit den histologischen Befunden nach Exzision.

BACKGROUND: Neoplasms of the conjunctiva include many different entities with a broad variety of clinical presentations. This can make a precise clinical diagnosis difficult. R0 resection is the gold standard treatment for most malignant conjunctival neoplasms, but not every benign lesions must treated by excision. In clinical practice it is important to make an accurate clinical diagnosis to enable the best possible management of conjunctival neoplasms. OBJECTIVE: The aim of this study was to determine the accuracy of clinical diagnosis of neoplasms of the conjunctiva. MATERIALS AND METHODS: Within a retrospective design, the data from all patients with excision of a conjunctival lesion between 2011 and 2020 in the Department of Ophthalmology of the UKSH Campus Kiel were extracted and analyzed. The specificity, sensitivity, and positive and negative predictive value for the preoperative clinical rating of dignity and diagnosis were evaluated based on the histological diagnostic findings. RESULTS: Of 220 included cases, 75% were benign and 25% malignant. The most frequent neoplasm of the conjunctiva was benign conjunctival nevus. The sensitivity for clinical prediction of a benign lesion was 0.86 (95% confidence interval [CI] 0.59-0.92), the specificity 0.95 (CI 0.85-0.99), and the positive predictive value 0.98 (CI 0.94-1.0). The sensitivity for clinical prediction of malign dignity was 0.95 (CI 0.85-0.99), the specificity 0.88 (CI 0.83-0.93), and the positive predictive value 0.73 (CI 0.61-0.83). CONCLUSION: The derived values for clinical diagnosis of conjunctival neoplasms can be rated as good. However, in clinical practice, untypical lesions can be hard to diagnose correctly, and the clinical diagnosis should be carefully reviewed; if in doubt, excision should be preferred.

Incidence of ocular surface squamous neoplasia in pterygium specimens.

OBJECTIVE: Pterygium and ocular surface squamous neoplasia (OSSN) have been recognized as likely related conditions and share similar risk factors such as ultraviolet radiation and chronic inflammation. The purpose of this study is to review the incidence of OSSN in pathology specimens sent as pterygium at a single tertiary centre between 2010 and 2022. METHODS: This is a retrospective chart review of patients operated on for pterygium between 2010 and 2022 at the University of Montreal Health Centre. Data collected include baseline demographics, results of pathology specimen, and clinical information for cases diagnosed as OSSN on pathology. RESULTS: A total of 1559 patients were operated on for a clinical diagnosis of pterygium between 2010 and 2022, of which 854 patients (55%) were male. A total of 1142 specimens had available pathology reports, and most of the specimens were consistent with pterygium on pathology (1105 of 1142; 97%). There was an unexpected finding of 3 cases of OSSN (3 of 1142; 0.3%). Other diagnosis besides pterygium were seen in 3% of specimens (34 of 1142), including nevus (n = 12), spheroidal degeneration (n = 3), pyogenic granuloma (n = 3), and lymphangiectasia (n = 2). The 3 cases of OSSN included an 81-year-old male of French-Canadian background, a 52-year-old male of South Asian background, and a 59-year-old female of French-Canadian background. The pathology was diagnosed as conjunctival intraepithelial neoplasia (CIN) grade 3, CIN grade 2, and CIN grade 2, respectively. CONCLUSION: The finding of OSSN in pterygium is rare in our population but can be clinically difficult to distinguish. It is important to send all pterygium specimens for pathology.

Unusual conjunctival melanocytic proliferations. Report of five cases and review of the literature.

Indeterminate melanocytic proliferations of the conjunctiva have both benign and malignant features that previously made these lesions nearly impossible to categorize in existing classification schemes. With the evolution of immunohistochemistry and molecular genetics, however, subclassifications have emerged that allow for a more tailored diagnosis and management. These conjunctival melanocytic proliferations include deep penetrating nevus, granular cell nevus, and nevoid melanoma. There remains a small subset of conjunctival melanocytic proliferations that defy precise characterization as nevi, primary acquired melanosis, or melanomas despite currently available ancillary diagnostic modalities and remain indeterminate. We highlight these unusual types of nevi and melanomas, with an update on their morphologic, immunohistochemical, and molecular genetic characteristics.

Comparison of non-invasive diagnostic modalities for ocular surface squamous neoplasia at a tertiary hospital, South Africa.

AIMS: The aim of the study is to assess non-invasive diagnostic modalities for ocular surface squamous neoplasia (OSSN) when compared to histology. METHODS: A prospective case-control study was conducted of patients presenting with conjunctival masses at a tertiary eye hospital in Johannesburg, South Africa. Patients completed an interview and had three non-invasive diagnostic tests: optical coherence tomography, impression cytology and methylene blue stain. A biopsy with histology was performed as the gold standard to confirm the diagnosis. RESULTS: One hundred and eighty-two conjunctival masses of 175 patients were evaluated. There were 135 lesions identified as OSSN on biopsy and 47 lesions were benign on histology. Optical coherence tomography had a sensitivity and specificity of 87.2% (95% CI: 80.0-92.5) and 75.6% (95% CI: 60.5-87.1), respectively, when an epithelial thickness cutoff of 140 um was used. Shadowing was found in 46% of cases due to leukoplakia or increased thickness of the mass. Cytology had a sensitivity of 72.4% (95% CI: 62.5-81.0) and a specificity of 74.3% (95% CI: 56.7-87.5). Twenty-seven per cent of cytology specimens were excluded from analysis due to inadequate cellularity. Methylene blue had a high sensitivity of 91.9% (95% CI: 85.9-95.9), but low specificity of 55.3% (95% CI: 40.1-69.8). CONCLUSION: Optical coherence tomography had a high sensitivity and specificity as a non-invasive test and liquid-based cytology performed well but had a lower sensitivity and specificity than with optical coherence tomography. Methylene blue performed well as a screening test, with a high sensitivity but low specificity.

Black Henna Deposits Masquerading a Melanoma of the Plica Semilunaris.

PURPOSE: The aim of this study was to describe a rare case of conjunctival black henna accumulation that was clinically misdiagnosed as melanoma. METHODS: This retrospective case report reviewed the clinical findings and histopathologic features of a pigmented plica semilunaris lesion excised from a 63-year-old woman. The patient had a history of regular use of black henna in her childhood. The conjunctival pigmentation was present since her early teenage years but started to grow only recently. RESULTS: There was a darkly pigmented mass mainly covering the plica semilunaris of the right eye with additional irregularly scattered pigmentation in the medial bulbar conjunctiva. Excisional biopsy of the plical tumor revealed nodular pigment deposits with lymphocytic infiltration. There were no neoplastic cells. The specimen stained negative for pan-keratin, CD68, and CD34. Melanin bleaching was also negative. CONCLUSIONS: Long-term exposure to black henna as a hair dye may lead to subconjunctival accumulation and mimic melanoma.

Uveal Exposure Following Globe Rupture Simulating Conjunctival Melanoma.

This case report describes a diagnosis of uveal prolapse masquerading as a conjunctival melanoma after globe rupture in a woman aged 89 years.

Machine Learning Model with Texture Analysis for Automatic Classification of Histopathological Images of Ocular Adnexal Mucosa-associated Lymphoid Tissue Lymphoma of Two Different Origins.

PURPOSE: The purpose of this study was to develop artificial intelligence algorithms that can distinguish between orbital and conjunctival mucosa-associated lymphoid tissue (MALT) lymphomas in pathological images. METHODS: Tissue blocks with residual MALT lymphoma and data from histological and flow cytometric studies and molecular genetic analyses such as gene rearrangement were procured for 129 patients treated between April 2008 and April 2020. We collected pathological hematoxylin and eosin-stained (HE) images of lymphoma from these patients and cropped 10 different image patches at a resolution of 2048 × 2048 from pathological images from each patient. A total of 990 images from 99 patients were used to create and evaluate machine-learning models. Each image patch of three different magnification rates at ×4, ×20, and ×40 underwent texture analysis to extract features, and then seven different machine-learning algorithms were applied to the results to create models. Cross-validation on a patient-by-patient basis was used to create and evaluate models, and then 300 images from the remaining 30 cases were used to evaluate the average accuracy rate. RESULTS: Ten-fold cross-validation using the support vector machine with linear kernel algorithm was identified as the best algorithm for discriminating between conjunctival mucosa-associated lymphoid tissue and orbital MALT lymphomas, with an average accuracy rate under cross-validation of 85%. There were ×20 magnification HE images that were more accurate in distinguishing orbital and conjunctival MALT lymphomas among ×4, ×20, and ×40. CONCLUSION: Artificial intelligence algorithms can successfully distinguish HE images between orbital and conjunctival MALT lymphomas.

ATRX Loss in the Development and Prognosis of Conjunctival Melanoma.

Metastatic disease is linked to TERT promoter mutations in conjunctival melanomas (CM). Both TERT promoter and ATRX mutations are associated with faulty telomere maintenance. This study aimed to determine the prognostic value of ATRX loss in conjunctival melanocytic lesions. Eighty-six conjunctival melanocytic lesions from the Rotterdam Ocular Melanoma Study group were collected. ATRX status and TERT promoter status were determined using immunohistochemical staining and molecular diagnostics, respectively. None of the nevi (n = 16) and primary acquired melanosis (PAM) without atypia (n = 6) showed ATRX loss. ATRX loss was found in 2/5 PAM with atypia without CM and in 8/59 CM. No cases with a TERT promoter mutation (n = 26) showed ATRX loss. Eight/eleven metastatic CM harbored a TERT promoter mutation, two other metastatic CM showed ATRX loss and one metastatic case showed no TERT promoter/ATRX alterations. In conclusion ATRX loss and TERT promoter mutations are only found in (pre)malignant conjunctival melanocytic lesions, with most metastatic cases harboring one of these alterations, suggesting that both alterations are associated with adverse behavior. Similar to TERT promoter mutations, ATRX loss may be used as a diagnostic tool in determining whether a conjunctival melanocytic lesion is prone to having an adverse course.

Concordancia clínica y patológica en tumores conjuntivales: Análisis de 195 casos / Clinic-pathological agreement in the diagnosis of conjunctival tumours: Analysis of 195 cases

Propósito Evaluar la concordancia entre el diagnóstico clínico y patológico en tumores conjuntivales en una unidad especializada en oncología ocular. Métodos Estudio retrospectivo de pacientes consecutivos con tumores conjuntivales diagnosticados en la Unidad de Oncología Ocular del Hospital Universitario de Valladolid desde 1992 hasta 2017. Los tumores se clasificaron según su origen (epitelial, melanocítico, linfoide y otros) y grado de malignidad (benigno, premaligno, maligno). Se realizó biopsia en los casos de lesiones sintomáticas o en crecimiento. Como indicador de concordancia entre el diagnóstico clínico y el patológico se utilizo el estadístico kappa (κ) de Cohen. Resultados Cuatrocientos sesenta y dos pacientes fueron atendidos de manera consecutiva, requiriendo biopsia en 195 (42,2%). La concordancia con el diagnóstico anatomopatológico fue satisfactoria en 154 (79%) casos. El análisis según el grado de malignidad mostró la menor tasa de concordancia en las lesiones benignas (n = 83; 91,6%) y premalignas (n = 62; 90,3%), con una concordancia total en las lesiones malignas (n = 50; 100%); el valor κ fue de 0,90. Los mayores índices de concordancia se encontraron en las lesiones epiteliales, melanocíticas y de partes blandas, con valores κ de 1, 0,8 y 1 respectivamente. El peor índice de concordancia se observó en lesiones linfoides, con un valor κ de 0,3. Conclusiones La mayoría de los tumores conjuntivales fueron correctamente identificados clínicamente. Las lesiones benignas y malignas mostraron la mayor tasa de precisión; sin embargo, las lesiones premalignas pueden ocultar enfermedad microinvasiva que puede pasar desapercibida en el examen clínico. La biopsia es esencial para un diagnóstico y un tratamiento precisos (AU)

Purpose The present study aims to assess the agreement between clinical and pathological diagnosis in conjunctival tumours in a specialist ocular oncology unit. Methods A retrospective study of consecutive patients with conjunctival tumours diagnosed at the Ocular Oncology Unit of the University Hospital of Valladolid was performed from 1992 to 2017. Tumours were classified according to their origin (epithelial, melanocytic, lymphoid, and others) and degree of malignancy (benign, premalignant, and malignant). A biopsy was performed in cases of symptomatic or growing lesions. Cohen's kappa (κ) statistics was used as an indicator of agreement between clinical and pathological diagnosis. Results Of 462 consecutive patients, a biopsy was required in 195 (42.2%). The agreement with the pathological diagnosis was successful in 154 (79%) cases. Analysis according to the grade of malignancy showed the lowest rate of agreement among benign (n = 83; 91.6%) and premalignant (n = 62; 90.3%) lesions, with a total agreement in malignant lesions (n = 50; 100%); the Cohen's kappa coefficient (κ) was 0.90. The highest rates of concordance were found in epithelial, melanocytic and soft tissue lesions with κ values of 1, 0.8 and 1, respectively. The worst rate of concordance was found in lymphoid lesions with a κ value of 0.3. Conclusions Most of the conjunctival tumours were correctly identified clinically; benign and malignant lesions showed the highest rate of accuracy; however, premalignant tumours can hide micro-invasive diseases that can go unnoticed on clinical examination. The biopsy is essential for accurate diagnosis and treatment (AU)

[Conjunctival tumors in children : Histopathological diagnoses in 262 cases]. / Bindehauttumoren bei Kindern : Histopathologische Diagnosen in 262 Fällen.

BACKGROUND: The spectrum of conjunctival tumors in children and adolescents significantly differs from that of adults. OBJECTIVE: The aim of this study was to show the distribution of histopathological diagnoses of pediatric conjunctival tumors. MATERIAL AND METHODS: Retrospective case series including all conjunctival tumors of children and adolescents under 18 years of age examined histologically over a period of 25 years (1990-2015) at the Eye Center in Freiburg, Germany. RESULTS: Among the 262 conjunctival specimens, the most common diagnoses were conjunctival nevi (148, [56%]), most often with junctional activity (106/148, [72%]), pyogenic granulomas (26, [10%]), and dermolipomas (18, [7%]). In one case a malignant tumor (conjunctival melanoma) was detected (1, < 1%). CONCLUSION: The results suggest that pediatric conjunctival tumors are benign in most cases. Most of them were of melanocytic origin, with a junctional conjunctival nevus being the most common diagnosis.