Clinicopathologic Correlation of Kaposi Sarcoma Involving the Ocular Adnexa: Immunophenotyping of Diagnostic and Therapeutic Targets

Abstract

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.

A rare case of bilateral conjunctival Kaposi's sarcoma in a HIV-negative patient

“… 12 Moreover, CD31, platelet-derived growth factor receptor alpha (PDGFR-A) and vascular endothelial growth factor receptor-1 (VEGF) are considered helpful diagnostic adjuncts for ocular adnexal KS. 13 Our patient was HCV positive, while blood tests revealed no other viral infections, including HIV and HHV-8, strengthening the evidence that ocular KS may occur in HIV-negative patients. 14 There are only few reports in the updated literature on adnexal KS in HIV-negative patients.…”

Platelet Derived Growth Factor-AA Correlates With Muscle Function Tests and Quantitative Muscle Magnetic Resonance in Dystrophinopathies

“…These mediators promote the activation of myofibroblasts which liberate extracellular matrix proteins leading to structural changes. Therefore, it is not surprising to find that the growth factors that we have studied here are also implicated in other diseases such as atherosclerosis, liver and pulmonary fibrosis, cirrhosis, myocardial infarction and neoplasms (52)(53)(54)(55)(56)(57)(58). However, the primary site of injury, the cells that differentiate into myofibroblasts and the organization of the immune response differs among the different types of disorders (52).…”

Unusual Localization of AIDS-Related Kaposi’s Sarcoma in a Heterosexual Male during the COVID-19 Pandemic: A Case Report

“…The clinical manifestations of ocular KS may not be typical, with the appearance of red eyes or conjunctival hemorrhages. The suggestive appearance of KS lesions is a macule, plaque or nodule, in bright red, purple or brown color, highly vascularized, and usually with telangiectatic vessels [ 20 , 21 , 22 ].…”