scholarly journals Retinal Pigment Epithelium Adenoma and Adenocarcinoma: A Review

2020 ◽  
pp. 1-12
Author(s):  
Janani  Sreenivasan ◽  
Pukhraj Rishi ◽  
Kalpita Das ◽  
Subramanian Krishnakumar ◽  
Jyotirmay Biswas

Adenoma and adenocarcinoma of the retinal pigment epithelium (RPE) are rare intraocular tumours that are often misdiagnosed as posterior uveal melanoma or other simulating lesions. It is important to be able to differentiate these tumours from melanoma for 3 reasons. First, an inability to rule out melanoma often tilts the management towards enucleation. Second, management options like radiotherapy and local resection which work well for melanoma may not be easily applied to these tumours. Third, and most importantly, patients with melanoma need a lifetime follow-up to rule out metastases (metastatic dormancy) whereas RPE tumours hardly metastasize. An abruptly elevated, often deeply pigmented tumour, with a prominent retinal feeding artery and a draining vein causing exudation, should raise a suspicion of RPE tumours. RPE tumours have a remarkable local invasive potential but a low metastatic potential. Most RPE tumours require treatment due to local complications. Small, asymptomatic tumours can be generally observed. Enucleation is still the gold standard of treatment, although local resection has been reported in selected cases with good results. Here, we provide a comprehensive review of the demographic, clinical, and imaging features of true acquired neoplasms of the RPE, namely adenoma and adenocarcinoma, the ways to differentiate them from melanoma, their clinical course and prognosis, and Options for their management.

2016 ◽  
Vol 2016 ◽  
pp. 1-5 ◽  
Author(s):  
José L. Sánchez-Vicente ◽  
Miguel Contreras-Díaz ◽  
Trinidad Rueda ◽  
Enrique Rodríguez de la Rúa-Franch ◽  
Fredy E. Molina-Socola ◽  
...  

Purpose. To describe the case of spontaneous resolution of epiretinal membrane in a patient with Combined Hamartoma of the Retina and Retinal Pigment Epithelium (CHR-RPE), in the clinical context of Gorlin Syndrome (GS).Methods. Observational case report of a 12-year-old female patient is presented. The diagnosis of CHRRPE was made by OCT and fundus examination, which showed a mound of disorganized tissue originating from retina and retinal pigment epithelium. Epiretinal membrane (EM) was also detected. Genetic study was performed to confirm the diagnosis of GS.Results. The patient was observed for 39 months, showing spontaneous resolution of the traction caused by the EM and improvement in visual acuity (VA), which was 20/80 at initial presentation, rising to 20/40 after follow-up period.Conclusions. The presence of EM in CHR-REP is a cause of reduction of visual acuity. Management of this condition is controversial; however, we would like to highlight that spontaneous resolution of the traction caused by EM is possible, resulting in recovery of VA.


2020 ◽  
pp. 112067212094657
Author(s):  
Dario Pasquale Mucciolo ◽  
Vittoria Murro ◽  
Dario Giorgio ◽  
Andrea Sodi ◽  
Ilaria Passerini ◽  
...  

Purpose: To report a case of choroideremia characterized by peripheral retinoschisis with vascular abnormalities and vitreous hemorrhage. Observations: A 58-year-old man affected by advanced-stage choroideremia was diagnosed with peripheral retinoschisis in both eyes. Vitreous hemorrhage was present in the right eye with a peculiar clot-like lesion at the periphery. At the 1-year follow-up, the vitreous hemorrhage had reabsorbed and the vascular clot-like lesion in the periphery had almost completely disappeared. Conclusion and importance: We have reported fundoscopic and OCT features of peripheral-acquired retinoschisis with vascular abnormalities in a patient with choroideremia. OCT examination is extremely useful in clinical evaluation of the peripheral retinal alterations in these cases, where the absence of the retinal pigment epithelium and the choriocapillaris pose many diagnostic difficulties.


2003 ◽  
Vol 13 (5) ◽  
pp. 491-495
Author(s):  
M. Rinaldi ◽  
A. Villani ◽  
M. Borrelli ◽  
S. Russo ◽  
L. Cotticelli

Purpose To describe the clinical manifestations in a patient with Sjögren reticular dystrophy of the retinal pigment epithelium, and the evolution of the disease over a 20-year follow-up period. Case Report A 45-year-old woman with Sjögren reticular dystrophy of the retinal pigment epithelium was seen for the first time in 1983; the patient underwent 20 years of annual check-ups. Results Over the follow-up period, fundus photographs, computerized perimetry, electroretinogram, and electro-oculogram findings had either normal or slightly subnormal outcome. Conclusions The results confirm that this disease involves only the retinal pigment epithelium and should not be considered a central tapeto-retinal degeneration or late onset retinopathy.


2021 ◽  
pp. 112067212110024
Author(s):  
Chunyan Lei ◽  
Rui Hua ◽  
Jianan Duan ◽  
Meixia Zhang

Purpose: To present retinal pigment epithelium (RPE) aperture related to an avascular pigment epithelium detachment (PED) secondary to acute central serous chorioretinopathy (CSC). Methods: Case report. Results: A 47-year-old man diagnosed as acute CSC presented with RPE aperture in the superonasal area of the macula in his left eye during follow-up. At 2-week follow-up, his decimal best-corrected visual acuity (BCVA) was improved from 0.08 to 0.6 and subretinal fluid was partially absorbed. However, the near-infrared reflectance demonstrated a round mild hyperreflective lesion on the superonasal area of the macula. On spectral-domain optical coherence tomography (SD-OCT), RPE band of the round lesion discontinued but RPE fractured edges without shrinkage and curling. Fundus autofluorescence (FAF) showed RPE aperture appeared as round hypoautofluorescence and hyperautofluorescence outlined its borderline. OCT angiography demonstrated that no evidence of neovascularization within the sub-RPE space. En Face OCT confirmed that the RPE aperture developed at the edge of the PED lesion. At 8-month follow-up, his decimal BCVA was improved to 1.0 and SD-OCT demonstrated spontaneous resolution of subretinal fluid and restoration of RPE structure, with complete flattening of PED. However, FAF revealed hypoautofluorescence mingled with slight hyperautofluorescence within the lesion. Conclusions: To the best of our knowledge, this is the first report of an RPE aperture secondary to acute CSC. Our case indicated another novel possible pathological mechanism that in the relatively healthy RPE, increased hydrostatic pressure simply itself could contribute to RPE aperture.


1980 ◽  
Vol 64 (6) ◽  
pp. 412-416 ◽  
Author(s):  
M L Klein ◽  
H Obertynski ◽  
A Patz ◽  
S L Fine ◽  
M Kini

2019 ◽  
Vol 5 (5) ◽  
pp. 369-378 ◽  
Author(s):  
Stephanie J. Weiss ◽  
Christina Stathopoulos ◽  
Carol L. Shields

Purpose: Choroidal nevus can cause overlying chronic retinal pigment epithelium (RPE) degenerative features, but frank retinal invasion is exquisitely rare. Procedures: This is a retrospective review of 8 cases of choroidal nevus with retinal invasion with evaluation of clinical and imaging features. Results: At the time of diagnosis of choroidal nevus with retinal invasion, mean patient age was 65 years. Mean tumor basal diameter was 7 mm, and mean thickness was 2.3 mm. Retinal invasion was ophthalmoscopically visible in all eyes. Related features included drusen (n = 4/8) and RPE fibrous metaplasia (n = 2/8). Overlying lipofuscin, subretinal fluid, RPE detachment, and retinal edema were absent. On B-scan ultrasonography, the lesion was dome-shaped (n = 7/7) and echo-dense (n = 6/7). Optical coherence tomography demonstrated outer retinal invasion (n = 8/8) with additional inner retinal invasion (n = 3/8). The tissue was hypoautofluorescent at the site of invasion (n = 6/7). Over a mean follow-up of 40 months, tumor enlargement was detected in 2 eyes and managed with observation (< 1 mm enlargement) or plaque radiotherapy (5 mm enlargement). Nevus hypoautofluorescence was correlated with nevus stability (p = 0.035). Conclusion: Retinal invasion of the choroidal nevus is rare. In this series of 8 cases, only 1 demonstrated transformation to melanoma over a mean interval of 40 months. Long-term monitoring of such lesions is warranted.


2020 ◽  
Vol 13 (9) ◽  
pp. e235731
Author(s):  
Padmaja Kumari Rani ◽  
Aniruddh Soni

A middle-aged man presented with exudative retinal detachment in the left eye. He was previously diagnosed as a case of large pigment epithelial detachment in the same eye, for which he was asked to follow-up closely, citing the risk of an retinal pigment epithelium (RPE) rip. Multimodal imaging confirmed the presence of a large RPE rip with exudative retinal detachment. He is a known retrovirus patient on anti-retroviral therapy with stable CD4 counts. He was diagnosed as a possible case of bullous variant of central serous chorioretinopathy and underwent external subretinal fluid drainage. We highlight the multimodal imaging findings of RPE rip with exudative retinal detachment and its impact on the patients visual acuity.


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