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Ciliary Body Benign Medulloepithelioma

MedGen UID:
736681
•Concept ID:
C1562687
•
Neoplastic Process
Synonyms: Benign Ciliary Body Medulloepithelioma; Benign Intraocular Medulloepithelioma; Benign intraocular medulloepithelioma; Benign medulloepithelioma of ciliary body
SNOMED CT: Benign medulloepithelioma of ciliary body (417647002); Benign intraocular medulloepithelioma (1230023005)

Definition

A rare, unilateral, benign embryonal neoplasm typically presenting as a ciliary body mass during childhood. It arises from primitive medullary epithelium. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCiliary Body Benign Medulloepithelioma

Professional guidelines

PubMed

Intraocular medulloepithelioma - A review of clinical features, DICER 1 mutation, and management.
Tadepalli SH, Shields CL, Shields JA, Honavar SG
Indian J Ophthalmol 2019 Jun;67(6):755-762. doi: 10.4103/ijo.IJO_845_19. PMID: 31124483Free PMC Article

Recent clinical studies

Etiology

DICER1-associated metastatic abdominopelvic primitive neuroectodermal tumor with an EWSR1 rearrangement in a 16-yr-old female.
Pancaldi A, Peng L, Rhee DS, Dunn E, Forcucci JA, Belchis D, Pratilas CA
Cold Spring Harb Mol Case Stud 2020 Oct;6(5) Epub 2020 Oct 7 doi: 10.1101/mcs.a005603. PMID: 33028642Free PMC Article
Intraocular medulloepithelioma - A review of clinical features, DICER 1 mutation, and management.
Tadepalli SH, Shields CL, Shields JA, Honavar SG
Indian J Ophthalmol 2019 Jun;67(6):755-762. doi: 10.4103/ijo.IJO_845_19. PMID: 31124483Free PMC Article
Ciliary body medulloepithelioma: analysis of 41 cases.
Kaliki S, Shields CL, Eagle RC Jr, Vemuganti GK, Almeida A, Manjandavida FP, Mulay K, Honavar SG, Shields JA
Ophthalmology 2013 Dec;120(12):2552-2559. Epub 2013 Jun 21 doi: 10.1016/j.ophtha.2013.05.015. PMID: 23796765
Medulloepithelioma of the ciliary body: the delay in diagnosis and frequent initial mismanagement.
Alkatan H, Al-Amry M, Al-Hussain H, Al-Dhibi H, Al-Mesfer S
Can J Ophthalmol 2011 Oct;46(5):431-8. Epub 2011 Aug 4 doi: 10.1016/j.jcjo.2011.07.007. PMID: 21995988
Congenital neoplasms of the nonpigmented ciliary epithelium (medulloepithelioma).
Shields JA, Eagle RC Jr, Shields CL, Potter PD
Ophthalmology 1996 Dec;103(12):1998-2006. doi: 10.1016/s0161-6420(96)30394-1. PMID: 9003333

Diagnosis

Intraocular medulloepithelioma - A review of clinical features, DICER 1 mutation, and management.
Tadepalli SH, Shields CL, Shields JA, Honavar SG
Indian J Ophthalmol 2019 Jun;67(6):755-762. doi: 10.4103/ijo.IJO_845_19. PMID: 31124483Free PMC Article
Ciliary body medulloepithelioma: analysis of 41 cases.
Kaliki S, Shields CL, Eagle RC Jr, Vemuganti GK, Almeida A, Manjandavida FP, Mulay K, Honavar SG, Shields JA
Ophthalmology 2013 Dec;120(12):2552-2559. Epub 2013 Jun 21 doi: 10.1016/j.ophtha.2013.05.015. PMID: 23796765
Medulloepithelioma of the ciliary body: the delay in diagnosis and frequent initial mismanagement.
Alkatan H, Al-Amry M, Al-Hussain H, Al-Dhibi H, Al-Mesfer S
Can J Ophthalmol 2011 Oct;46(5):431-8. Epub 2011 Aug 4 doi: 10.1016/j.jcjo.2011.07.007. PMID: 21995988
Ciliary body medulloepithelioma: four cases associated with pleuropulmonary blastoma--a report from the International Pleuropulmonary Blastoma Registry.
Priest JR, Williams GM, Manera R, Jenkinson H, Bründler MA, Davis S, Murray TG, Galliani CA, Dehner LP
Br J Ophthalmol 2011 Jul;95(7):1001-5. Epub 2010 Dec 13 doi: 10.1136/bjo.2010.189779. PMID: 21156700
Congenital intraocular teratoma.
Kivelä T, Merenmies L, Ilveskoski I, Tarkkanen A
Ophthalmology 1993 May;100(5):782-91. doi: 10.1016/s0161-6420(93)31575-7. PMID: 8493024

Prognosis

Intraocular medulloepithelioma - A review of clinical features, DICER 1 mutation, and management.
Tadepalli SH, Shields CL, Shields JA, Honavar SG
Indian J Ophthalmol 2019 Jun;67(6):755-762. doi: 10.4103/ijo.IJO_845_19. PMID: 31124483Free PMC Article
Medulloepithelioma of the ciliary body: the delay in diagnosis and frequent initial mismanagement.
Alkatan H, Al-Amry M, Al-Hussain H, Al-Dhibi H, Al-Mesfer S
Can J Ophthalmol 2011 Oct;46(5):431-8. Epub 2011 Aug 4 doi: 10.1016/j.jcjo.2011.07.007. PMID: 21995988
Differential diagnosis of anterior chamber cysts with ultrasound biomicroscopy: ciliary body medulloepithelioma.
Zhou M, Xu G, Bojanowski CM, Song Y, Chen R, Sun X, Wang W, Chan CC
Acta Ophthalmol Scand 2006 Feb;84(1):137-9. doi: 10.1111/j.1600-0420.2005.00542.x. PMID: 16445454Free PMC Article
Congenital neoplasms of the nonpigmented ciliary epithelium (medulloepithelioma).
Shields JA, Eagle RC Jr, Shields CL, Potter PD
Ophthalmology 1996 Dec;103(12):1998-2006. doi: 10.1016/s0161-6420(96)30394-1. PMID: 9003333
Concurrent benign teratoid medulloepithelioma and pineoblastoma.
Mamalis N, Font RL, Anderson CW, Monson MC, Williams AT
Ophthalmic Surg 1992 Jun;23(6):403-8. PMID: 1513537

Clinical prediction guides

Unusual case of intraocular medulloepithelioma in an adult male.
Rehman O, Narang S, Nayyar S, Aggarwal P
Rom J Ophthalmol 2021 Jul-Sep;65(3):296-299. doi: 10.22336/rjo.2021.61. PMID: 35036657Free PMC Article
Recurrent lacrimal gland choristoma of the ciliary body in an infant mimicking a medulloepithelioma.
Tauziède-Espariat A, Roche O, Dufier JL, Putterman M
Eur J Ophthalmol 2016 Nov 4;26(6):e145-e148. doi: 10.5301/ejo.5000798. PMID: 27173894
Ciliary body medulloepithelioma: four cases associated with pleuropulmonary blastoma--a report from the International Pleuropulmonary Blastoma Registry.
Priest JR, Williams GM, Manera R, Jenkinson H, Bründler MA, Davis S, Murray TG, Galliani CA, Dehner LP
Br J Ophthalmol 2011 Jul;95(7):1001-5. Epub 2010 Dec 13 doi: 10.1136/bjo.2010.189779. PMID: 21156700
Differential diagnosis of anterior chamber cysts with ultrasound biomicroscopy: ciliary body medulloepithelioma.
Zhou M, Xu G, Bojanowski CM, Song Y, Chen R, Sun X, Wang W, Chan CC
Acta Ophthalmol Scand 2006 Feb;84(1):137-9. doi: 10.1111/j.1600-0420.2005.00542.x. PMID: 16445454Free PMC Article
Medulloepithelioma diagnosed by ultrasound and vitreous aspirate. Electron microscopic observations.
Orellana J, Moura RA, Font RL, Boniuk M, Murphy D
Ophthalmology 1983 Dec;90(12):1531-9. doi: 10.1016/s0161-6420(83)34352-9. PMID: 6677853

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