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Retinocytoma

MedGen UID:
91105
•Concept ID:
C0346396
•
Neoplastic Process
Synonyms: Adenoma of retinal pigment epithelium; Retinoma
SNOMED CT: Retinocytoma (128913004); Retinocytoma (416864007); Adenoma of retinal pigment epithelium (255027009); Retinoma (255027009)

Definition

A rare, unilateral or bilateral benign neoplasm that arises from the retina. There is an increased risk of malignant transformation to retinoblastoma; therefore, patients with a diagnosis of retinocytoma should be closely observed. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRetinocytoma

Recent clinical studies

Etiology

RETINOCYTOMA WITH VITREOUS SEEDING: NEW INSIGHTS FROM ENHANCED DEPTH IMAGING OPTICAL COHERENCE TOMOGRAPHY AND HIGH-RESOLUTION POSTERIOR SEGMENT ULTRASONOGRAPHY.
Garoon RB, Medina CA, Scelfo C, Harbour JW
Retin Cases Brief Rep 2021 Jan 1;15(1):68-70. doi: 10.1097/ICB.0000000000000732. PMID: 29470300
Effect of topotecan on retinocytoma cell apoptosis and expression of Livin and PTEN.
Zhang M, Shan BE, Yuan NF, Liu W
Chin Med J (Engl) 2013 Jan;126(2):340-4. PMID: 23324287
The pathology of ocular cancer.
Eagle RC Jr
Eye (Lond) 2013 Feb;27(2):128-36. Epub 2012 Nov 16 doi: 10.1038/eye.2012.237. PMID: 23154492Free PMC Article
Retinocytoma shows lack of response to chemoreduction.
Chung J, Turaka K, Shields CL
J Pediatr Ophthalmol Strabismus 2010 Dec 22;47 Online:e1-3. doi: 10.3928/01913913-20101217-03. PMID: 21175116
Retinocytoma associated with calcified vitreous deposits.
Hadjistilianou T, De Francesco S, Martone G, Malandrini A
Eur J Ophthalmol 2006 Mar-Apr;16(2):349-51. doi: 10.1177/112067210601600227. PMID: 16703560

Diagnosis

Multimodal Imaging of a Retinocytoma.
Li X, Ju Y, Huang X
Ophthalmol Retina 2024 Jan;8(1):61. Epub 2023 Nov 10 doi: 10.1016/j.oret.2023.09.024. PMID: 37952138
Adult onset retinoblastoma.
Sengupta S, Pan U, Khetan V
Indian J Ophthalmol 2016 Jul;64(7):485-91. doi: 10.4103/0301-4738.190099. PMID: 27609158Free PMC Article
Photopsia revealing a retinocytoma.
Santos C, Ramalho M, Coutinho I, Teixeira S
BMJ Case Rep 2015 Aug 4;2015 doi: 10.1136/bcr-2015-211018. PMID: 26243747Free PMC Article
Retinocytoma shows lack of response to chemoreduction.
Chung J, Turaka K, Shields CL
J Pediatr Ophthalmol Strabismus 2010 Dec 22;47 Online:e1-3. doi: 10.3928/01913913-20101217-03. PMID: 21175116
What's your diagnosis? Retinocytoma.
Wong IB, Farzavandi S
J Pediatr Ophthalmol Strabismus 2010 Sep-Oct;47(5):268, 287. doi: 10.3928/01913913-20100916-02. PMID: 20839739

Therapy

Retinocytoma shows lack of response to chemoreduction.
Chung J, Turaka K, Shields CL
J Pediatr Ophthalmol Strabismus 2010 Dec 22;47 Online:e1-3. doi: 10.3928/01913913-20101217-03. PMID: 21175116

Prognosis

Retinocytoma/retinoma: comparative analysis of clinical features in 78 tumors and rate of transformation into retinoblastoma over 20 years.
Shields CL, Srinivasan A, Lucio-Alvarez JA, Shields JA
J AAPOS 2021 Jun;25(3):147.e1-147.e8. Epub 2021 May 26 doi: 10.1016/j.jaapos.2020.11.024. PMID: 34051357
Bilateral retinoblastoma in early infancy.
Saiju R, Duwal S
Nepal J Ophthalmol 2013 Jan-Jun;5(1):124-8. doi: 10.3126/nepjoph.v5i1.7840. PMID: 23584660
The pathology of ocular cancer.
Eagle RC Jr
Eye (Lond) 2013 Feb;27(2):128-36. Epub 2012 Nov 16 doi: 10.1038/eye.2012.237. PMID: 23154492Free PMC Article
Retinocytoma associated with calcified vitreous deposits.
Hadjistilianou T, De Francesco S, Martone G, Malandrini A
Eur J Ophthalmol 2006 Mar-Apr;16(2):349-51. doi: 10.1177/112067210601600227. PMID: 16703560
Histopathologic verification of Verhoeff's 1918 irradiation cure of retinoblastoma.
Marcus DM, Craft JL, Albert DM
Ophthalmology 1990 Feb;97(2):221-4. doi: 10.1016/s0161-6420(90)32602-7. PMID: 2326011

Clinical prediction guides

Retinocytoma/retinoma: comparative analysis of clinical features in 78 tumors and rate of transformation into retinoblastoma over 20 years.
Shields CL, Srinivasan A, Lucio-Alvarez JA, Shields JA
J AAPOS 2021 Jun;25(3):147.e1-147.e8. Epub 2021 May 26 doi: 10.1016/j.jaapos.2020.11.024. PMID: 34051357
RETINOCYTOMA WITH VITREOUS SEEDING: NEW INSIGHTS FROM ENHANCED DEPTH IMAGING OPTICAL COHERENCE TOMOGRAPHY AND HIGH-RESOLUTION POSTERIOR SEGMENT ULTRASONOGRAPHY.
Garoon RB, Medina CA, Scelfo C, Harbour JW
Retin Cases Brief Rep 2021 Jan 1;15(1):68-70. doi: 10.1097/ICB.0000000000000732. PMID: 29470300
Effect of topotecan on retinocytoma cell apoptosis and expression of Livin and PTEN.
Zhang M, Shan BE, Yuan NF, Liu W
Chin Med J (Engl) 2013 Jan;126(2):340-4. PMID: 23324287
Phenotypic variability of retinocytomas: preregression and postregression growth patterns.
Abouzeid H, Balmer A, Moulin AP, Mataftsi A, Zografos L, Munier FL
Br J Ophthalmol 2012 Jun;96(6):884-9. Epub 2012 Feb 10 doi: 10.1136/bjophthalmol-2011-300896. PMID: 22328814
Identification and significance of multinucleate tumor cells in retinoblastoma.
Howard MA, Dryja TP, Walton DS, Albert DM
Arch Ophthalmol 1989 Jul;107(7):1025-30. doi: 10.1001/archopht.1989.01070020087037. PMID: 2751457

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