Pseudoretinoblastoma: Spectrum of Pathology and Frequency in Different Age Groups. Analysis of 14 years of Experience

2021 ◽  
pp. 9-14
Author(s):  
A.A. Yarovoy ◽  
◽  
V.A. Yarovaya ◽  
D.P. Volodin ◽  
A.V. Kotelnikova ◽  
...  
Keyword(s):  
Retinopathy Of Prematurity ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Age Groups ◽  
Morning Glory ◽  
Radiology Department ◽  
Federal State ◽  
Choroidal Hemangioma ◽  
Coats Disease ◽  
Vitreous Opacities

Purpose. To evaluate the spectrum and the occurrence frequency of lesions simulating retinoblastoma in different age groups. Material and methods. A retrospective study of 608 patients (871 eyes) with suspected retinoblastoma was performed in the Ocular Oncology and Radiology Department of the Fyodorov Eye Microsurgery Federal State Institution in the period from 2007 to 2020. The mean patient age at presentation was 21 months (from 1 to 209 months). In 475 patients (78.1%) (715 eyes), the diagnosis of retinoblastoma was confirmed after a comprehensive assessment of the eye. 133 patients (156 eyes) (21.9%) had symptoms mimicking retinoblastoma-pseudoretinoblastomas. All patients with pseudoretinoblastomas were divided into different groups based on the simulating condition and age: from 0 to 1 year, from 1 to 2 years, from 2 to 5 years, older than 5 years. Results. There were 25 conditions simulating retinoblastoma. The most common conditions were Coats' disease (n=17; 12.8%), combined hamartoma of retina and retinal pigment epithelium (n=12. 9%), retinal detachment (n=11; 8.3%), vitreous opacities caused by intrauterine uveitis (n=11; 8.3%), choroidal hemangioma (n=11; 8.3%), vasoproliferative tumor (n=10; 7.5%), retinopathy of prematurity (n=7; 5.3%), morning glory syndrome (n=7; 5.3%). Most patients had unilateral lesions (n=116; 87.2%). Pseudoretinoblastomas differed based on age at presentation, because children 1 year of age or younger were most likely to have vitreous opacities caused by intrauterine uveitis (n=9; 21.4%) and retinopathy of prematurity (n=7; 16.7%); children aged from 1 to 2 years – Coats' disease (n=4; 20%) and choroidal hemangioma (n=3; 15%), from 2 to 5 years – Coats' disease (n=8; 21.6%), older than 5 years – Coats' disease (n=5; 14.7%) and vasoproliferative tumors (n=7; 20.6%). Conclusion. Timely referral of children with suspected retinoblastoma in specialized ocular oncology centers helps to avoid unnecessary treatment for retinoblastoma, enucleation and improve the vital prognosis.

scholarly journals A Case of Coats' Disease with Spontaneous Retinal Reattachment after Total Detachment

2015 ◽  
Vol 6 (2) ◽  
pp. 200-203 ◽  
Author(s):  
Ryosuke Ochi ◽  
Bunpei Sato ◽  
Masashi Mimura ◽  
Seita Morishita ◽  
Masanori Fukumoto ◽  
...  
Keyword(s):  
Retinal Detachment ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Light Perception ◽  
Functional Effect ◽  
Computed Tomography Imaging ◽  
Tomography Imaging ◽  
Retinal Blood Vessels ◽  
Retinal Reattachment ◽  
Coats Disease

Purpose: To report a case of Coats' disease in which spontaneous reattachment occurred after total retinal detachment. Patient and Methods: A young boy (patient age: 4 years and 11 months) presented with leukocoria in the left eye. Slit-lamp examination revealed total retinal detachment with abnormal retinal blood vessels and subretinal exudation just behind the lens. Computed tomography imaging showed no solid mass lesion in the intraocular space. Secondary total retinal detachment as a complication of Coats' disease was diagnosed. No light perception was detected, so we determined that surgical treatment was not indicated. Results: Four months after the initial diagnosis, the retina showed complete reattachment with a large amount of subretinal hard exudate. Visual acuity remained unchanged, with no light perception. Conclusions: We speculate that the spontaneous retinal reattachment in the present case was caused by the decreased permeability of the abnormal retinal vessels and the good functional effect of the retinal pigment epithelium.

High-resolution scanning electron microscopy (HRSEM) of mitochondria from various rat tissues

1988 ◽  
Vol 46 ◽  
pp. 14-15
Author(s):  
P.J. Lea ◽  
M.J. Hollenberg
Keyword(s):  
Electron Microscopy ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Rat Hepatocytes ◽  
Three Dimensions ◽  
Objective Lens ◽  
Rat Tissues ◽  
Thin Sections ◽  
Reconstruction Methods ◽  
Scanning Electron

Our current understanding of mitochondrial ultrastructure has been derived primarily from thin sections using transmission electron microscopy (TEM). This information has been extrapolated into three dimensions by artist's impressions (1) or serial sectioning techniques in combination with computer processing (2). The resolution of serial reconstruction methods is limited by section thickness whereas artist's impressions have obvious disadvantages.In contrast, the new techniques of HRSEM used in this study (3) offer the opportunity to view simultaneously both the internal and external structure of mitochondria directly in three dimensions and in detail.The tridimensional ultrastructure of mitochondria from rat hepatocytes, retinal (retinal pigment epithelium), renal (proximal convoluted tubule) and adrenal cortex cells were studied by HRSEM. The specimens were prepared by aldehyde-osmium fixation in combination with freeze cleavage followed by partial extraction of cytosol with a weak solution of osmium tetroxide (4). The specimens were examined with a Hitachi S-570 scanning electron microscope, resolution better than 30 nm, where the secondary electron detector is located in the column directly above the specimen inserted within the objective lens.

Ultrastructural Changes of Smoooth Endoplasmic Reticulum in the Retinal Pigment Epithelium by Choline Chloride

1972 ◽  
Vol 30 ◽  
pp. 58-59
Author(s):  
Kazushige Hirosawa ◽  
Eichi Yamada
Keyword(s):  
Endoplasmic Reticulum ◽  
Epithelial Cell ◽  
Smooth Endoplasmic Reticulum ◽  
Pigment Epithelium ◽  
Choline Chloride ◽  
Retinal Pigment ◽  
Ultrastructural Changes ◽  
Lower Vertebrate ◽  
Visual Cells ◽  
Pigment Epithelial

The pigment epithelium is located between the choriocapillary and the visual cells. The pigment epithelial cell is characterized by a large amount of the smooth endoplasmic reticulum (SER) in its cytoplasm. In addition, the pigment epithelial cell of some lower vertebrate has myeloid body as a specialized form of the SER. Generally, SER is supposed to work in the lipid metabolism. However, the functions of abundant SER and myeloid body in the pigment epithelial cell are still in question. This paper reports an attempt, to depict the functions of these organelles in the frog retina by administering one of phospholipid precursors.

High-voltage electron microscopy of subretinal scar formation

1992 ◽  
Vol 50 (1) ◽  
pp. 486-487
Author(s):  
G.E. Korte ◽  
M. Marko ◽  
G. Hageman
Keyword(s):  
Electron Microscopy ◽  
Monoclonal Antibody ◽  
Retinal Pigment Epithelium ◽  
Glial Cells ◽  
High Voltage ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Sodium Iodate ◽  
High Voltage Electron Microscopy ◽  
Voltage Electron

Sodium iodate iv. damages the retinal pigment epithelium (RPE) in rabbits. Where RPE does not regenerate (e.g., 1,2) Muller glial cells (MC) forma subretinal scar that replaces RPE. The MC response was studied by HVEM in 3D computer reconstructions of serial thick sections, made using the STEREC0N program (3), and the HVEM at the NYS Dept. of Health in Albany, NY. Tissue was processed for HVEM or immunofluorescence localization of a monoclonal antibody recognizing MG microvilli (4).

The photoreceptor cytoskeleton visualized

1992 ◽  
Vol 50 (1) ◽  
pp. 480-481
Author(s):  
Beth Burnside
Keyword(s):  
Outer Segment ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Cell Polarization ◽  
Synaptic Proteins ◽  
Cell Functions ◽  
Vertebrate Photoreceptor ◽  
Numerous Cell ◽  
Turn Over

The vertebrate photoreceptor provides a drammatic example of cell polarization. Specialized to carry out phototransduction at its distal end and to synapse with retinal interneurons at its proximal end, this long slender cell has a uniquely polarized morphology which is reflected in a similarly polarized cytoskeleton. Membranes bearing photopigment are localized in the outer segment, a modified sensory cilium. Sodium pumps which maintain the dark current critical to photosensory transduction are anchored along the inner segment plasma membrane between the outer segment and the nucleus.Proximal to the nucleus is a slender axon terminating in specialized invaginating synapses with other neurons of the retina. Though photoreceptor diameter is only 3-8u, its length from the tip of the outer segment to the synapse may be as great as 200μ. This peculiar linear cell morphology poses special logistical problems and has evoked interesting solutions for numerous cell functions. For example, the outer segment membranes turn over by means of a unique mechanism in which new disks are continuously added at the proximal base of the outer segment, while effete disks are discarded at the tip and phagocytosed by the retinal pigment epithelium. Outer segment proteins are synthesized in the Golgi near the nucleus and must be transported north through the inner segment to their sites of assembly into the outer segment, while synaptic proteins must be transported south through the axon to the synapse.The role of the cytoskeleton in photoreceptor motile processes is being intensely investigated in several laboratories.

Major Hereditary Gastrointestinal Cancer Syndromes: a Narrative Review

2017 ◽  
Vol 26 (2) ◽  
pp. 157-163 ◽  
Author(s):  
Lakshmi Manogna Chintalacheruvu ◽  
Trudy Shaw ◽  
Avanija Buddam ◽  
Osama Diab ◽  
Thamer Kassim ◽  
...  
Keyword(s):  
Gastric Cancer ◽  
Genetic Testing ◽  
Gastrointestinal Cancer ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Clinical Manifestations ◽  
Micro Rna ◽  
Diffuse Gastric Cancer ◽  
Adenomatous Polyposis ◽  
Cancer Syndromes

Gastrointestinal cancer is one of the major causes of death worldwide. Hereditary gastrointestinal cancer syndromes constitute about 5-10% of all cancers. About 20-25% of undiagnosed cases have a possible hereditary component, which is not yet established. In the last few decades, the advance in genomics has led to the discovery of multiple cancer predisposition genes in gastrointestinal cancer. Physicians should be aware of these syndromes to identify high-risk patients and offer genetic testing to prevent cancer death. In this review, we describe clinical manifestations, genetic testing and its challenges, diagnosis and management of the major hereditary gastrointestinal cancer syndromes.Key words:  −  −  −  − .Abbreviations: ACG: American College of Gastroenterology; AFAP: attenuated FAP; APC: adenomatous polyposis coli; CDH1: E-cadherin; CHRPE: congenital hypertrophy of the retinal pigment epithelium; CRC: colorectal cancer; FAMMM: Familial atypical multiple mole melanoma; FAP: Familial adenomatous polyposis; GC: gastric cancer; HDGC: Hereditary diffuse gastric cancer; IHC: immunohistochemical; IPAA: ileal pouch–anal anastomosis; IRA: ileorectal anastomosis; MSI: microsatellite instability; MMR: mismatch repair; miRNA: micro RNA.

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