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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/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
Histopathologic grading of anaplasia in retinoblastoma.
Mendoza PR, Specht CS, Hubbard GB, Wells JR, Lynn MJ, Zhang Q, Kong J, Grossniklaus HE
Am J Ophthalmol 2015 Apr;159(4):764-76. Epub 2014 Dec 19 doi: 10.1016/j.ajo.2014.12.014. PMID: 25528954Free PMC Article
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
Observations on 17 patients with retinocytoma.
Singh AD, Santos CM, Shields CL, Shields JA, Eagle RC Jr
Arch Ophthalmol 2000 Feb;118(2):199-205. doi: 10.1001/archopht.118.2.199. PMID: 10676785

Diagnosis

Unilateral retinocytoma associated with a variant in the RB1 gene.
Wu S, Zou X, Sun Z, Zhu T, Wei X, Sui R
Mol Genet Genomic Med 2020 Apr;8(4):e1156. Epub 2020 Jan 29 doi: 10.1002/mgg3.1156. PMID: 31997559Free PMC Article
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
Observations on 17 patients with retinocytoma.
Singh AD, Santos CM, Shields CL, Shields JA, Eagle RC Jr
Arch Ophthalmol 2000 Feb;118(2):199-205. doi: 10.1001/archopht.118.2.199. PMID: 10676785
Retinoma. Case studies.
Balmer A, Munier F, Gailloud C
Ophthalmic Paediatr Genet 1991 Sep;12(3):131-7. doi: 10.3109/13816819109029394. PMID: 1754160

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
Histopathologic grading of anaplasia in retinoblastoma.
Mendoza PR, Specht CS, Hubbard GB, Wells JR, Lynn MJ, Zhang Q, Kong J, Grossniklaus HE
Am J Ophthalmol 2015 Apr;159(4):764-76. Epub 2014 Dec 19 doi: 10.1016/j.ajo.2014.12.014. PMID: 25528954Free PMC Article
p16(INK4a) expression in retinoblastoma: a marker of differentiation grade.
Liu Y, Zhong X, Wan S, Zhang W, Lin J, Zhang P, Li Y
Diagn Pathol 2014 Dec 11;9:180. doi: 10.1186/s13000-014-0180-1. PMID: 25499675Free PMC Article
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
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

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
Histopathologic grading of anaplasia in retinoblastoma.
Mendoza PR, Specht CS, Hubbard GB, Wells JR, Lynn MJ, Zhang Q, Kong J, Grossniklaus HE
Am J Ophthalmol 2015 Apr;159(4):764-76. Epub 2014 Dec 19 doi: 10.1016/j.ajo.2014.12.014. PMID: 25528954Free PMC Article
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
Expression of alpha-crystallin in retinoblastoma.
Kase S, Parikh JG, Rao NA
Arch Ophthalmol 2009 Feb;127(2):187-92. doi: 10.1001/archophthalmol.2008.580. PMID: 19204237Free PMC Article
Observations on 17 patients with retinocytoma.
Singh AD, Santos CM, Shields CL, Shields JA, Eagle RC Jr
Arch Ophthalmol 2000 Feb;118(2):199-205. doi: 10.1001/archopht.118.2.199. PMID: 10676785

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