Ciliary Body Benign Teratoid Medulloepithelioma

MedGen UID:: 361199
•Concept ID:: C1879809
•: Neoplastic Process

Synonym:

Benign Intraocular Teratoid Medulloepithelioma

Definition

A rare, unilateral, benign 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

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVCiliary Body Benign Teratoid Medulloepithelioma

Neoplasm by Special Category
- Benign neoplasm
  - Benign Eye Neoplasm
    - Benign Uveal Neoplasm
      - Benign neoplasm of ciliary body
        Ciliary Body Benign Medulloepithelioma
        Ciliary Body Benign 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: 31124483 Free PMC Article

See all (1)

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: 33028642 Free 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: 31124483 Free 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

See all (6)

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: 31124483 Free PMC Article

Ciliary body medulloepithelioma: analysis of 41 cases.

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

See all (8)

Prognosis

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: 33028642 Free 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: 31124483 Free 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

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

See all (6)