U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

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

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
Imaging features of medulloepithelioma: report of four cases and review of the literature.
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
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 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

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

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.

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...