Long-Term Survival and Factors Associated with Increased Mortality in Patients with Ocular Adnexal Lymphomas.

Orbital and ocular adnexal lymphoma (OAL) affects the orbit and the surrounding structures and can arise as several subtypes with variable prognoses. We performed an observational study on the relationship between OAL subtype, diagnostic features, and prognosis to offer valuable insights into imaging techniques, such as Positron Emission Tomography with 2-deoxy-2-[fluorine-18] fluoro-D-glucose integrated with Computed Tomography (18F-FDG PET-CT), in predicting outcomes. With this aim, we retrospectively reviewed 99 patients with OALs, recording demographics, cancer subtype, location and treatment, 18FDG avidity, and bone marrow positivity. We divided patients into Group 1 (those presenting with extranodal marginal zone lymphoma-EMZL) and Group 2, including all other subtypes. The primary outcome was long-term cancer-specific survival (CSS) based on key predictors, performed through Kaplan-Meier curves and the log-rank test, with a p < 0.05 significance threshold. The mean patient age was 67 years (57-75.5). The most frequent histopathologic subtypes were EMZL lymphoma in 69 patients (69.7%), small lymphocytic lymphoma (11.1%) and diffuse-large B-cell lymphoma (10.1%). Patients of Group 1 showed a better prognosis (CSS = 80%) compared to those of Group 2 (CSS = 60%) (p = 0.01). In patients with high-grade lymphoma, the occurrence of 18FDG avidity (p = 0.003) and bone marrow positivity (p = 0.005) were related to a worse prognosis. In our group, EMZL was the most prominent subtype of OALs and exhibited the best prognosis, low 18FDG avidity, and bone marrow negativity. By observing specific patterns in radiological findings, it is possible to increase our understanding of disease progression, treatment response, and the overall prognosis in OAL patients.

Targeting shared pathways in tauopathies and age-related macular degeneration: implications for novel therapies.

The intricate parallels in structure and function between the human retina and the central nervous system designate the retina as a prospective avenue for understanding brain-related processes. This review extensively explores the shared physiopathological mechanisms connecting age-related macular degeneration (AMD) and proteinopathies, with a specific focus on tauopathies. The pivotal involvement of oxidative stress and cellular senescence emerges as key drivers of pathogenesis in both conditions. Uncovering these shared elements not only has the potential to enhance our understanding of intricate neurodegenerative diseases but also sets the stage for pioneering therapeutic approaches in AMD.

Primary Orbital Respiratory Epithelial Cyst: A Systematic Review and Two Case Reports.

A primary orbital respiratory cyst is a congenital choristoma that presents in the orbit and with different signs and symptoms depending on the location, which might also change the surgical approach. The aim of this report is to describe 2 new cases of primary respiratory epithelial cysts and to review the literature on presentation, management, and risk factors with different surgical approaches and complications. Two cases presenting with gradually increased proptosis had a confirmed diagnosis of a respiratory epithelial cyst. CT and MRI imaging revealed a thin-walled intraconal cystic lesion. Complete surgical removal was not possible and an incisional biopsy of the wall was performed. Rarely, orbital respiratory epithelium cysts are primary lesions. The high risk of breaking the capsule during surgery and the presence of residual epithelial cells within the orbit may cause cyst's recurrence. Indeed, we recommend careful attention during dissection maneuvers and a long-term follow-up.

Outcomes and risk factors of surgical management of thyroid eye disease-related diplopia.

OBJECTIVES: To evaluate the outcomes of strabismus surgery in patients with thyroid eye disease (TED) and to assess the influence of preceding treatments such as steroid administration, orbital decompression and radiotherapy. METHODS: Retrospective study on a cohort of patients with TED undergoing strabismus surgery. Data collection included clinical features, smoking status, preceding steroid therapy, orbital radiation therapy and orbital decompression. Qualitative evaluation, assessing diplopia and quality of life and quantitative evaluation, assessing the eye misalignment were analysed 1 year after the last squint surgical procedure. RESULTS: Seventy-nine patients were identified. Ninety-five surgical procedures were performed on 92 eyes. A significant overall improvement of ocular motility was registered (p < 0.001, paired t-test). Forty-five (57%) patients had excellent outcomes, twenty-three (29%) had good outcomes and eleven (14%) had poor outcomes. The average number of muscles operated was significantly greater in patients who underwent orbital bone decompression (1.58 ± 0.63; p = 0.0082; 95% CI 1.413-10.214). Quantitative and qualitative outcomes were not associated with the preceding therapy: steroid (p = 0.75), radiotherapy (p = 0.95) or orbital bone decompression (p = 0.25). The success rate was no different between adjustable and fixed sutures (p = 0.8). CONCLUSION: Strabismus surgery in TED patients resulted in a high success rate in reducing diplopia and improving ocular motility. The success rate was not negatively associated with previous therapies. Indeed, resolution of diplopia required more squint procedures in patients who have previously undergone to orbital decompression.

Primary cutaneous anaplastic large-cell lymphoma of the eyelid: report of two cases and review of the literature.

PURPOSE: Two new cases of primary cutaneous CD30+ anaplastic large-cell lymphoma (cALCL) of the eyelid are reported; these are analysed alongside existing cases to identify challenges relating to the diagnosis and management of such rare lesions. MATERIAL AND METHODS: A review of existing literature on the PubMed database is conducted using the keywords: 'eyelid lymphoid proliferations', 'lymphoma of the eyelid', and 'primary cutaneous CD30+, ALK-anaplastic large-cell lymphoma of the eyelid'. Two new cases of cALCL are reported. Cases where patients present solely with a nodular periocular lesion are analysed for recurrence and survival rate. RESULTS: Two new patients with a painless ulcerated nodule on the upper eyelid receive a confirmed diagnosis of cALCL after undergoing an excisional biopsy. The first, elderly patient has spontaneous remission; the second patient, with a concomitant chronic infection of hepatitis C virus (HCV), presents a more diffuse disease at the onset and requires radiotherapy. Together with 13 patients a primary cALCL identified from 11 previous studies, this constitutes a cohort of 15 patients. Of these, 10 present with an exclusively nodular lesion of the eyelid and four experience disease recurrence; no deaths from cALCL are reported. CONCLUSION: Differential diagnosis between primary cALCL and lymphomatoid papulosis is essential and requires careful consideration of clinical and pathologic features. Radiologic staging examination is crucial in order to exclude systemic ALCL, particularly for patients with comorbidity. Though cALCL has the pathological features of a malignant lesion, the prognosis seems favourable for patients; a relatively high percentage even experience spontaneous resolution.

Optical Coherence Tomography Angiography Findings After Intravitreal Ranibizumab in Patients With Coats Disease.

The aim of this retrospective study was to describe the vascular features in eyes with Coats disease, using optical coherence tomography angiography (OCTA), at baseline and after 3 monthly intravitreal injections of ranibizumab. Fifteen eyes of 15 consecutive patients affected by Coats' disease were recruited in this study. All patients underwent the best-corrected visual acuity (BCVA) evaluation, fundus examination, fluorescein angiography (FA), indocyanine green angiography (ICGA), multicolor imaging, structural Spectral Domain (SD)-OCT and OCTA at baseline and 1 month after the third monthly ranibizumab injection (loading phase). Fifteen patients completed the study, of whom nine were males and six females. Mean age was 20.4 ± 2 years. BCVA was 0.46 ± 0.11 logMar and 0.47 ± 0.12 logMar at baseline and after treatment, respectively (p = 0.164). SD-OCT revealed no significant decrease in central macular thickness (486.33 µm ± 93.37 at baseline vs. 483.4 µm ± 80.97 after treatment; p = 0.915). The subretinal exudates persisted in macular region after intravitreal injections. OCTA showed a general vascular rarefaction in superficial capillary plexus (SCP), deep capillary plexus (DCP), and choriocapillary (CC) that did not change after loading phase. This study showed no functional and vascular improvement following 3 monthly ranibizumab injections. OCTA, non-invasive technique, could be useful during follow up of these patients and provide a better understand of pathogenesis of this disorder.