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Intraocular medulloepithelioma

MedGen UID:
408140
Concept ID:
C1883694
Neoplastic Process
Synonyms: Ciliary Body Medulloepithelioma; Intraocular Medulloepithelioma; intraocular medulloepithelioma; Orbital medulloepithelioma; orbital medulloepithelioma
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0017050
Orphanet: ORPHA268139

Definition

Intraocular medulloepithelioma is a rare eye tumor characterized by a white, gray or yellow-colored cystic mass that arises from the primitive neuroectodermal, nonpigmented epithelium of the ciliary body, or occasionally from the optic nerve, optic disc, retina or iris. Typically it has a benign clinical course with good prognosis and generally presents with childhood onset of poor vision and pain, glaucoma, and/or cataract. Leukocoria, exotropia, exophthalmos, strabismus, epiphora, change in eye color, hyphema, and raised intraocular pressure are also remarkable manifestations. [from ORDO]

Professional guidelines

PubMed

Yi X, Meng F, Bi Y, He L, Qian J, Xue K
Br J Ophthalmol 2024 Mar 20;108(4):566-570. doi: 10.1136/bjo-2022-322449. PMID: 36997291
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
Slade I, Bacchelli C, Davies H, Murray A, Abbaszadeh F, Hanks S, Barfoot R, Burke A, Chisholm J, Hewitt M, Jenkinson H, King D, Morland B, Pizer B, Prescott K, Saggar A, Side L, Traunecker H, Vaidya S, Ward P, Futreal PA, Vujanic G, Nicholson AG, Sebire N, Turnbull C, Priest JR, Pritchard-Jones K, Houlston R, Stiller C, Stratton MR, Douglas J, Rahman N
J Med Genet 2011 Apr;48(4):273-8. Epub 2011 Jan 25 doi: 10.1136/jmg.2010.083790. PMID: 21266384

Recent clinical studies

Etiology

Imam U, Hameed M, Anis I, Bhutto I, Ahmed SH, Rizvi A, Ahmad Z
J Pak Med Assoc 2023 Dec;73(12):2469-2472. doi: 10.47391/JPMA.8253. PMID: 38083935
Nambirajan A, Gurung N, Suri V, Sarkar C, Kumar A, Singh M, Sharma MC
Childs Nerv Syst 2021 Apr;37(4):1067-1075. Epub 2020 Nov 24 doi: 10.1007/s00381-020-04973-0. PMID: 33236184
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
Galluzzi P, Casseri T, Cerase A, Guglielmucci D, Toti P, Hadjistilianou T
Neuroradiology 2018 Nov;60(11):1213-1222. Epub 2018 Sep 19 doi: 10.1007/s00234-018-2094-1. PMID: 30232518
Avedschmidt SE, Stagner AM, Eagle RC Jr, Harocopos GJ, Dou Y, Rao RC
Invest Ophthalmol Vis Sci 2016 Nov 1;57(14):6242-6246. doi: 10.1167/iovs.16-20463. PMID: 27842164Free PMC Article

Diagnosis

Yi X, Meng F, Bi Y, He L, Qian J, Xue K
Br J Ophthalmol 2024 Mar 20;108(4):566-570. doi: 10.1136/bjo-2022-322449. PMID: 36997291
Imam U, Hameed M, Anis I, Bhutto I, Ahmed SH, Rizvi A, Ahmad Z
J Pak Med Assoc 2023 Dec;73(12):2469-2472. doi: 10.47391/JPMA.8253. PMID: 38083935
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
Sansgiri RK, Wilson M, McCarville MB, Helton KJ
Pediatr Radiol 2013 Oct;43(10):1344-56. Epub 2013 Aug 30 doi: 10.1007/s00247-013-2718-x. PMID: 23989751
Saunders T, Margo CE
Arch Pathol Lab Med 2012 Feb;136(2):212-6. doi: 10.5858/arpa.2010-0669-RS. PMID: 22288972

Therapy

Stathopoulos C, Gaillard MC, Schneider J, Munier FL
BMC Ophthalmol 2020 Jun 18;20(1):239. doi: 10.1186/s12886-020-01512-y. PMID: 32552699Free PMC Article
Shields CL, Shields JA, De Potter P, Singh AD
Br J Ophthalmol 1994 Sep;78(9):702-6. doi: 10.1136/bjo.78.9.702. PMID: 7947552Free PMC Article

Prognosis

Yi X, Meng F, Bi Y, He L, Qian J, Xue K
Br J Ophthalmol 2024 Mar 20;108(4):566-570. doi: 10.1136/bjo-2022-322449. PMID: 36997291
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
Ashour OZ, Stalling M, Ramsey J, Straka DG, Pierson CR, Martin LC
Radiographics 2018 Jan-Feb;38(1):194-199. doi: 10.1148/rg.2018170160. PMID: 29320332
Avedschmidt SE, Stagner AM, Eagle RC Jr, Harocopos GJ, Dou Y, Rao RC
Invest Ophthalmol Vis Sci 2016 Nov 1;57(14):6242-6246. doi: 10.1167/iovs.16-20463. PMID: 27842164Free PMC Article
Saunders T, Margo CE
Arch Pathol Lab Med 2012 Feb;136(2):212-6. doi: 10.5858/arpa.2010-0669-RS. PMID: 22288972

Clinical prediction guides

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
Lindegaard J, Heegaard S, Toft PB, Nysom K, Prause JU
Orbit 2010 Jun;29(3):161-4. doi: 10.3109/01676830903421200. PMID: 20497085
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
Shields CL, Shields JA, De Potter P, Singh AD
Br J Ophthalmol 1994 Sep;78(9):702-6. doi: 10.1136/bjo.78.9.702. PMID: 7947552Free PMC Article
Shields CL, Shields JA, De Potter P, Singh AD
Trans Am Ophthalmol Soc 1993;91:177-89; discussion 189-95. PMID: 8140690Free PMC Article

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