Purpose To disclose, using an ex vivo study, the histopathological mechanism behind the in vivo thickening of the Endothelium/Descemet’s membrane complex (En/DM) observed in rejected corneal grafts (RCGs). Methods Descemet’s membrane (DM), endothelium (En), and retrocorneal membranes (RCM) make up the total En/DM thickness. These layers are non-differentiable by HD-OCT; therefore, the source of the thickening is unclear from an in vivo perspective. A retrospective ex vivo study (from September 2015 to December 2015) was conducted to measure the thicknesses of DM, En, and RCM in 54 corneal specimens (31 RCGs and 23 controls) using light microscopy. Controls were globes with posterior melanoma without corneal involvement. Results There were 54 corneas examined ex vivo with mean age 58.1 ± 12.2 in controls and 51.7 ± 27.9 years in rejected corneal grafts. The ex vivo study uncovered the histopathological mechanism of En/DM thickening to be secondary to significant thickening (p<0.001) of DM (6.5 ± 2.4 μm) in rejected corneal grafts compared to controls (3.9 ± 1.5 μm). Conclusions Our ex vivo study shows Descemet’s membrane is responsible for the thickening of the En/DM in RCGs observed in vivo by HD-OCT and not the endothelium or retrocorneal membrane.
Purpose: To describe the clinicopathologic characteristics and the expression of diagnostic/treatment targets in ocular adnexal Kaposi Sarcoma. Methods: We conducted a clinical-pathologic retrospective case series. Immunohistochemical staining for cluster of differentiation 31 (CD31), human herpesvirus-8 (HHV8), platelet-derived growth factor receptor alpha (PDGFR-A), vascular endothelial growth factor receptor-1 (VEGF), tyrosine-protein kinase Kit (c-Kit), and programmed cell death protein 1 (PD-1) were performed. Percentage of positive tumor cells was recorded for PD-1; staining intensity and distribution (H-score) were determined for the remaining stains. A Friedman non-parametric ANOVA analysis evaluated the staining. Results: The study cohort included 13 patients (age 25 to 95 years; mean 46): 7 lesions were in the eyelid, 5 in the conjunctiva, and 1 in the cornea. Nine of 11 lesions (82%) were in human immunodeficiency syndrome-positive patients (human immunodeficiency syndrome status was unknown in 2 cases). Staging included 6 plaques and 7 nodules. The mean H-scores of CD31, HHV8, c-Kit, VEGF, and PDGF-A were 8.00, 8.23, 2.77, 11.54, and 10.31, respectively. Mean PD-1 staining was 6.46%. The Friedman non-parametric ANOVA analysis showed VEGF, PDGF-A, CD31, and HHV8 differed significantly, and all differed significantly from c-Kit. Programmed cell death protein 1 staining was not significant with any clinical variable. Conclusions: Cluster of differentiation 31 and HHV8 are helpful diagnostic adjuncts for ocular adnexal Kaposi Sarcoma. Platelet-derived growth factor receptor alpha and VEGF are promising treatment targets. Programmed cell death protein 1/PD-L1 and c-Kit are targets that are useful in several tumors; their roles in ocular adnexal Kaposi Sarcoma warrant further studies.
Unilateral neuroretinitis can present as a late-onset manifestation of CHIKV. Three patients with diagnosis of CHIKV developed unilateral neuroretinitis weeks after the acute viral syndrome, suggesting a possible immunological etiology for this manifestation. To our knowledge, this is the first case series of CHIKV leading to neuroretinitis in the Western World.
: Atypical spindle cell and pleomorphic lipomatous tumor (ASCPLT) is a rare lipomatous neoplasm that was recently introduced into the World Health Organization Classification of Soft Tissue and Bone tumors as a distinct entity. ASCPLT has potential for local recurrence but does not metastasize. This biologic behavior separates ASCPLT from its morphologic mimics. Ocular adnexal ASCPLT has not been previously reported. Described herein are two patients with ASCPLT. The subcutaneous orbital rim lesion featured markedly pleomorphic spindle and multinucleated cells. The eyelid lesion was dominated by atypical spindle cells in a background of mature adipocytes. Both neoplasms demonstrated infiltrative margins, rare mitotic figures, immunoreactivity for CD34 and loss of Rb1, and the absence of MDM2 amplification by fluorescence in situ hybridization. Recognition of ASCPLT in the differential of ocular adnexal neoplasms may lead to a re-evaluation of morphologically similar tumors, which may have varied biologic behavior and warrant a different management approach.
Fibromas are benign, well-circumscribed tumors that are characterized as spindle-cell lesions with interlacing fibrous stroma. Here, we describe the clinical presentation, management, and outcome of a patient with an orbital fibroma. To our knowledge, this is the second case report of a biopsy-proven subperiosteal orbital fibroma to date.
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