Decreased Macular Choriocapillaris Perfusion Correlates with Contrast Sensitivity Function in Dry Age-related Macular Degeneration.

PURPOSE: To investigate the relationships between contrast sensitivity (CS), choriocapillaris perfusion and other structural optical coherence tomography (OCT) biomarkers in dry age-related macular degeneration (AMD). DESIGN: Cross-sectional, observational study. PARTICIPANTS: One hundred AMD eyes (22 early, 52 intermediate and 26 late) from 74 patients and 45 control eyes from 37 age-similar subjects. METHODS: All participants had visual acuity (VA) assessment, quantitative contrast sensitivity function (qCSF) testing, macular OCT, and 6x6-mm swept-source OCT angiography (OCTA) scans on the same day. OCT volumes were analyzed for subretinal drusenoid deposits and hyporeflective drusen cores, and to measure thickness of the outer nuclear layer (ONL). OCTA scans were utilized to calculate drusen volume, inner choroid flow deficit percentage (IC-FD%), and to measure the area of choroidal hypertransmission defects (HTD). IC-FD% was measured from a 16 µm-thick choriocapillaris slab after compensation and binarization with Phansalkar's method. Generalized linear mixed-effects models were used to evaluate the associations between functional and structural variables. MAIN OUTCOME MEASURES: To explore the associations between qCSF-measured CS, ICFD% and various AMD imaging biomarkers. RESULTS: AMD exhibited significantly reduced qCSF metrics eyes across all stages compared to controls. Univariate analysis revealed significant associations between various imaging biomarkers, reduced qCSF metrics and VA in both groups. Multivariate analysis confirmed that higher IC-FD% in the central 5 mm was significantly associated with decreases in all qCSF metrics in AMD eyes (ß= -0.74 to -0.25, all p<0.05), but not with VA (p>0.05). ONL thickness in the central 3 mm correlated with both VA (ß= 2.85, p<0.001) and several qCSF metrics (ß= 0.01-0.90, all p<0.05), especially in AMD eyes. Further, larger HTD areas were associated with decreased VA (ß=-0.89, p<0.001) and reduced CS at low-intermediate frequencies across AMD stages (ß= -0.30 to -0.29, p<0.001). CONCLUSIONS: The significant association between IC-FD% in the central 5 mm and qCSF-measured CS reinforces the hypothesis that decreased macular choriocapillaris perfusion contributes to visual function changes in AMD, which are more pronounced in CS than in VA.

A systematic review of undifferentiated pleomorphic sarcoma of the chest wall.

BACKGROUND: Undifferentiated pleomorphic sarcoma (UPS) accounts for approximately 15% of all soft-tissue sarcoma (STS) cases and have a 5-year survival prognosis of around 60%. Due to its complexity, tumors are often identified by clinical and pathological exclusion. UPS is commonly found in the extremities, so finding them in the trunk and chest wall is rare. The primary objectives of this systematic review are: (I) identifying patient characteristics with lesion; (II) compiling patient outcomes following surgery; (III) identifying best therapy modalities; (IV) characterizing reported lesion histology; (V) assessing current surgical recommendations for resection; (VI) classifying lesions and their association with radiation. METHODS: The PRISMA framework was utilized to identify case reports and records providing information on UPS in the chest wall. Case reports and articles were screened for relevance, full-text accessibility, and if they contained the terms ("undifferentiated pleomorphic sarcoma", "breast", "chest wall", or "trunk") in their title or abstract. The PubMed database was the primary database, and the search criteria was "(undifferentiated pleomorphic sarcoma) AND ((breast) OR (trunk) OR (chest) OR (chest wall))" from 01/01/2003 to 05/21/2023. Given that these were case reports, bias risk and heterogeneity was not assessed due to its difficulty. Information from case reports were compiled into a table and a Chi-squared test was performed, but no meta-analysis was completed. RESULTS: Of 433 studies, 24 case reports and 22 records were selected to inform on UPS in the chest wall. The 24 case reports yielded 32 cases providing information on patient outcomes, tumor characteristics, and treatment. A meta-analysis was not performed, but literature was summarized to inform on treating the condition. Case reports were compiled into a table providing information on patient age, gender, tumor location, treatment modalities, margin distance, and other factors. CONCLUSIONS: Treatment of UPS involving the chest is extremely complex. Unlike typical UPS, it is more often found in women than in men, which is corroborated by the results of this study. This study also notes no difference in recurrence or metastasis between patient who were treated and those who were not treated with other therapies.

Identification of high-risk germline variants for the development of pancreatic cancer: Common characteristics and potential guidance to screening guidelines.

Pancreatic cancer (PC) is a product of a variety of environmental and genetic factors. Recent work has highlighted the influence of hereditary syndromes on pancreatic cancer incidence. The purpose of this review is to identify the high-risk syndromes, common variants, and risks associated with PC. The study also elucidates common characteristics of patients with these mutations, which is used to recommend potential changes to current screening protocols for greater screening efficacy. We analyzed 8 syndromes and their respective variants: Hereditary Breast and Ovarian Cancer (BRCA1/2), Familial Atypical Multiple Mole Melanoma Syndrome (CDKN2A), Peutz-Jeghers Syndrome (STK11), Lynch Syndrome (PMS2, MLH1, MSH2, MSH6, EPCAM), Ataxia Telangiectasia (ATM), Li-Fraumeni Syndrome (TP53), Fanconi Anemia (PALB2), and Hereditary Pancreatitis (PRSS1, SPINK1, CFTR). Of 587 studies evaluated, 79 studies fit into our inclusion criteria. Information from each study was analyzed to draw conclusions on these variants as well as their association with pancreatic cancer. Information from this review is intended to improve precision medicine and improve criteria for screening.