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Ciliary Body Teratoid Medulloepithelioma

MedGen UID:
363504
•Concept ID:
C1881244
•
Neoplastic Process
Synonym: Intraocular Teratoid Medulloepithelioma

Definition

A rare, unilateral, benign or malignant embryonal neoplasm typically presenting as a ciliary body mass during childhood. It arises from primitive medullary epithelium and contains heterologous elements, particularly cartilage, skeletal muscle, and brain tissue. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCiliary Body Teratoid 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

Intraocular medulloepithelioma: an unusual and challenging entity in paediatric population.
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
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
Growth of congenital malignant teratoid medulloepithelioma of the ciliary body: a case study.
Cerase A, De Francesco S, Citterio A, Hadjistilianou T, Malandrini A, Mastrangelo D, Toti P, Venturi C
J Neurooncol 2010 Feb;96(3):443-8. Epub 2009 Aug 8 doi: 10.1007/s11060-009-9982-5. PMID: 19669095

Diagnosis

Malignant teratoid intraocular ciliary body medulloepithelioma in a 5-year-old male with corresponding somatic copy number alteration profile of aqueous humor cell-free DNA.
Pike S, Iyengar R, Peng CC, Chevez-Barrios P, Brown B, Shah R, Biegel J, Yellapantula V, Nagiel A, Reiser BJ, Xu L, Berry JL
Ophthalmic Genet 2022 Dec;43(6):855-861. Epub 2022 Oct 31 doi: 10.1080/13816810.2022.2138457. PMID: 36314385Free 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
Medulloepithelioma (diktyoma).
Canning CR, McCartney AC, Hungerford J
Br J Ophthalmol 1988 Oct;72(10):764-7. doi: 10.1136/bjo.72.10.764. PMID: 3056510Free PMC Article

Prognosis

DICER1 tumor predisposition syndrome: an evolving story initiated with the pleuropulmonary blastoma.
González IA, Stewart DR, Schultz KAP, Field AP, Hill DA, Dehner LP
Mod Pathol 2022 Jan;35(1):4-22. Epub 2021 Oct 1 doi: 10.1038/s41379-021-00905-8. PMID: 34599283Free 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
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
Growth of congenital malignant teratoid medulloepithelioma of the ciliary body: a case study.
Cerase A, De Francesco S, Citterio A, Hadjistilianou T, Malandrini A, Mastrangelo D, Toti P, Venturi C
J Neurooncol 2010 Feb;96(3):443-8. Epub 2009 Aug 8 doi: 10.1007/s11060-009-9982-5. PMID: 19669095
Congenital malignant teratoid neoplasm of the eye and orbit: a case report and review of the literature.
Steinkuller PG, Font RL
Ophthalmology 1997 Jan;104(1):38-42. doi: 10.1016/s0161-6420(97)30364-9. PMID: 9022102

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
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
A clinicopathologic study of 56 cases of intraocular medulloepitheliomas.
Broughton WL, Zimmerman LE
Am J Ophthalmol 1978 Mar;85(3):407-18. doi: 10.1016/s0002-9394(14)77739-6. PMID: 655220

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