U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Adenoma of the Retinal Pigment Epithelium

MedGen UID:
1761376
•Concept ID:
C5420846
•
Neoplastic Process

Definition

A benign neoplasm that arises from the retinal pigment epithelium. It consists of cords and tubules of variably pigmented proliferating retinal pigment epithelium cells, separated by fibrous stroma. Malignant transformation is rare. (WHO 2018) [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAdenoma of the Retinal Pigment Epithelium

Professional guidelines

PubMed

Genotype-phenotype correlation in 99 familial adenomatous polyposis patients: A prospective prevention protocol.
de Oliveira JC, Viana DV, Zanardo C, Santos EMM, de Paula AE, Palmero EI, Rossi BM
Cancer Med 2019 May;8(5):2114-2122. Epub 2019 Mar 21 doi: 10.1002/cam4.2098. PMID: 30897307Free PMC Article
Multiple approach to the exploration of genotype-phenotype correlations in familial adenomatous polyposis.
Bertario L, Russo A, Sala P, Varesco L, Giarola M, Mondini P, Pierotti M, Spinelli P, Radice P; Hereditary Colorectal Tumor Registry
J Clin Oncol 2003 May 1;21(9):1698-707. doi: 10.1200/JCO.2003.09.118. PMID: 12721244
The clinical [corrected] background of familial adenomatous polyposis. History, epidemiology, diagnosis and treatment.
Kartheuser A, Detry R, West S, Coppens JP, Gribomont AC, Hoang P, Melange M, Vanheuverzwyn R, Verellen-Dumoulin C, Burn J
Acta Gastroenterol Belg 1995 Mar-Apr;58(2):252-66. PMID: 7571988

Recent clinical studies

Etiology

Adenoma and Adenocarcinoma of the Retinal Pigment Epithelium: A Review of 51 Consecutive Patients.
Williams BK Jr, Di Nicola M, Acaba-Berrocal LA, Milman T, Mashayekhi A, Lucio-Alvarez JA, Eagle RC Jr, Shields JA, Shields CL
Ophthalmol Retina 2020 Aug;4(8):829-839. Epub 2020 Mar 18 doi: 10.1016/j.oret.2020.03.008. PMID: 32417354
Segmented retinal layer analysis of chiasmal compressive optic neuropathy in pituitary adenoma patients.
Moon JS, Shin SY
Graefes Arch Clin Exp Ophthalmol 2020 Feb;258(2):419-425. Epub 2019 Dec 18 doi: 10.1007/s00417-019-04560-3. PMID: 31853626
Tumors and Related Lesions of the Pigmented Epithelium.
Shields JA, Shields CL
Asia Pac J Ophthalmol (Phila) 2017 Mar-Apr;6(2):215-223. doi: 10.22608/APO.201705. PMID: 28399346
Pigmented and depigmented lesions of the ocular fundus.
Traboulsi EI
Curr Opin Ophthalmol 2012 Sep;23(5):337-43. doi: 10.1097/ICU.0b013e32835622b0. PMID: 22847029
Familial adenomatous polyposis.
Half E, Bercovich D, Rozen P
Orphanet J Rare Dis 2009 Oct 12;4:22. doi: 10.1186/1750-1172-4-22. PMID: 19822006Free PMC Article

Diagnosis

Analysis of clinical features of RPE adenoma.
Zhou N, Xu X, Wei W
Graefes Arch Clin Exp Ophthalmol 2020 Dec;258(12):2831-2840. Epub 2020 Jun 13 doi: 10.1007/s00417-020-04784-8. PMID: 32535672
Tumors and Related Lesions of the Pigmented Epithelium.
Shields JA, Shields CL
Asia Pac J Ophthalmol (Phila) 2017 Mar-Apr;6(2):215-223. doi: 10.22608/APO.201705. PMID: 28399346
Pigmented and depigmented lesions of the ocular fundus.
Traboulsi EI
Curr Opin Ophthalmol 2012 Sep;23(5):337-43. doi: 10.1097/ICU.0b013e32835622b0. PMID: 22847029
Familial adenomatous polyposis.
Half E, Bercovich D, Rozen P
Orphanet J Rare Dis 2009 Oct 12;4:22. doi: 10.1186/1750-1172-4-22. PMID: 19822006Free PMC Article
Familial adenomatous polyposis.
Galiatsatos P, Foulkes WD
Am J Gastroenterol 2006 Feb;101(2):385-98. doi: 10.1111/j.1572-0241.2006.00375.x. PMID: 16454848

Therapy

ATYPICAL CONGENITAL HYPERTROPHY OF THE RETINAL PIGMENT EPITHELIUM COMPLICATED BY PRESUMED RETINAL PIGMENT EPITHELIAL ADENOMA AND EXUDATIVE MACULOPATHY.
Mehta N, Gal-Or O, Barbazetto I, Modi Y, Shields CL, Freund KB
Retin Cases Brief Rep 2021 May 1;15(3):330-334. doi: 10.1097/ICB.0000000000000800. PMID: 30063581
Diagnostic and Therapeutic Challenges.
Kolomeyer AM, Brucker AJ, Boldt HC
Retina 2020 Oct;40(10):2061-2063. doi: 10.1097/IAE.0000000000002652. PMID: 31584556
Germline mutations of the APC gene in patients with familial adenomatous polyposis-associated thyroid carcinoma: results from a European cooperative study.
Cetta F, Montalto G, Gori M, Curia MC, Cama A, Olschwang S
J Clin Endocrinol Metab 2000 Jan;85(1):286-92. doi: 10.1210/jcem.85.1.6254. PMID: 10634400
Adenocarcinoma of the retinal pigment epithelium: a diagnostic and therapeutic challenge.
Finger PT, McCormick SA, Davidian M, Walsh JB
Graefes Arch Clin Exp Ophthalmol 1996 Aug;234 Suppl 1:S22-7. doi: 10.1007/BF02343044. PMID: 8871146
Familial polyposis coli: the spectrum of ocular and other extracolonic manifestations.
Heinemann MH, Baker RH, Miller HH, DeCosse JJ
Graefes Arch Clin Exp Ophthalmol 1991;229(3):213-8. doi: 10.1007/BF00167870. PMID: 1651277

Prognosis

Adenoma and Adenocarcinoma of the Retinal Pigment Epithelium: A Review of 51 Consecutive Patients.
Williams BK Jr, Di Nicola M, Acaba-Berrocal LA, Milman T, Mashayekhi A, Lucio-Alvarez JA, Eagle RC Jr, Shields JA, Shields CL
Ophthalmol Retina 2020 Aug;4(8):829-839. Epub 2020 Mar 18 doi: 10.1016/j.oret.2020.03.008. PMID: 32417354
Segmented retinal layer analysis of chiasmal compressive optic neuropathy in pituitary adenoma patients.
Moon JS, Shin SY
Graefes Arch Clin Exp Ophthalmol 2020 Feb;258(2):419-425. Epub 2019 Dec 18 doi: 10.1007/s00417-019-04560-3. PMID: 31853626
Pigmented and depigmented lesions of the ocular fundus.
Traboulsi EI
Curr Opin Ophthalmol 2012 Sep;23(5):337-43. doi: 10.1097/ICU.0b013e32835622b0. PMID: 22847029
Familial adenomatous polyposis.
Galiatsatos P, Foulkes WD
Am J Gastroenterol 2006 Feb;101(2):385-98. doi: 10.1111/j.1572-0241.2006.00375.x. PMID: 16454848
Congenital hypertrophy of the retinal pigment epithelium and familial adenomatous polyposis.
McKay DL
Aust N Z J Ophthalmol 1993 Feb;21(1):3-6. doi: 10.1111/j.1442-9071.1993.tb00122.x. PMID: 8388223

Clinical prediction guides

Adenoma and Adenocarcinoma of the Retinal Pigment Epithelium: A Review of 51 Consecutive Patients.
Williams BK Jr, Di Nicola M, Acaba-Berrocal LA, Milman T, Mashayekhi A, Lucio-Alvarez JA, Eagle RC Jr, Shields JA, Shields CL
Ophthalmol Retina 2020 Aug;4(8):829-839. Epub 2020 Mar 18 doi: 10.1016/j.oret.2020.03.008. PMID: 32417354
Segmented retinal layer analysis of chiasmal compressive optic neuropathy in pituitary adenoma patients.
Moon JS, Shin SY
Graefes Arch Clin Exp Ophthalmol 2020 Feb;258(2):419-425. Epub 2019 Dec 18 doi: 10.1007/s00417-019-04560-3. PMID: 31853626
Familial adenomatous polyposis.
Galiatsatos P, Foulkes WD
Am J Gastroenterol 2006 Feb;101(2):385-98. doi: 10.1111/j.1572-0241.2006.00375.x. PMID: 16454848
Fundus lesions of adenomatous polyposis.
Tiret A, Parc C
Curr Opin Ophthalmol 1999 Jun;10(3):168-72. doi: 10.1097/00055735-199906000-00003. PMID: 10537774
Congenital hypertrophy of the retinal pigment epithelium and familial adenomatous polyposis.
McKay DL
Aust N Z J Ophthalmol 1993 Feb;21(1):3-6. doi: 10.1111/j.1442-9071.1993.tb00122.x. PMID: 8388223

Supplemental Content

Table of contents

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...