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

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVRetinocytoma

Disease
- Neoplasm
  - Neoplasm by Special Category
    - Benign neoplasm
      - Benign Eye Neoplasm
        Benign neoplasm of retina
        Retinocytoma

Professional guidelines

PubMed

Beyond retinocytomas: clinical benefit of topotecan in the management of other intra-cranial tumors especially glioblastomas.

Kapoor S
Chin Med J (Engl) 2013;126(9):1635. PMID: 23652042

See all (1)

Recent clinical studies

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

See all (1)

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: 25528954 Free 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: 25499675 Free 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: 23154492 Free 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

See all (9)

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.

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: 19204237 Free 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

See all (13)