Background:The histological diagnosis of neurogenic tumors remains a challenge, which may be indicated particularly by the fact that new entities appeared in the new edition of the World Health organization (WHO) classification. Purpose: To review the histomorphologic and immunohistochemic features of rare variants of neurogenic ocular (retinal) tumors in adults. Material and Methods: Six rare ocular tumors were selected for the study from all clinical material submitted for pathohistological examination from 2017 to 2020 based on the presence of morphological evidence of neurogenic differentiation. Results:The study sample of six rare neurogenic retinal tumors in adults was conventionally divided into three types: (1) retinal tumors immunohistochemically similar to cellular ependymoma, but histologically similar to retinoblastoma;(2) tumors showing no histological pattern characteristic for dictyoma, but the immunohistochemical features of neuroepithelial differentiation; and (3) tumors showing histological patterns similar to medulloepithelioma, but the immunohistochemical features of glial markers. Conclusion: Obviously, when dividing these tumors into histogenetic groups, not only the histological structure and immunohistochemical profile, but also tumor location and typical patient age should be taken into account.

This is an initiative study. Cherubism as a form of fibrous dysplasia of jaw bones is a congenital hereditary abnormality of osseous tissue maturation. Although the disease requires mostly maxillofacial surgery and orthodontic care, patients also have to be consulted by an ophthalmologist. The purpose of this study was to share our experience in the diagnostic assessment and treatment of cherubism, a rare form of fibrous dysplasia that is commonly misdiagnosed, which results in inadequate treatment and may lead to serious ocular complications. We present our experience of diagnostic assessment and treatment of four patients with cherubism for one to twenty years. The diagnosis of cherubism became evident after histological study of the material excised during surgery, because postoperative histology showed that changes were not cancerous (adamantinoma), but dysplastic. In addition, new foci developed or old foci recurred some time after surgery. Consequently, true jaw tumors must be correctly differentiated from dental cysts and from dysplastic bone lesions. Patients were followed up once every 6 months. Over the period of follow-up, ocular involvement was observed in the woman who had been diagnosed in childhood with congenital mixed astigmatism. Given our observations, one may make a conclusion that cherubism, a rare form of fibrous dysplasia, is a hereditary or familial disorder, and regresses after completion of puberty and puberty-related changes in hormonal milieu. Timely orthodontic care (and, if required, maxillofacial surgery care) will facilitate preventing the progression of the disease and its ophthalmological consequences. Special attention of ophthalmologists should be paid to cherubism patients with maxillary lesions.