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Ciliary Body Malignant Medulloepithelioma

MedGen UID:
91104
•Concept ID:
C0346378
•
Neoplastic Process
Synonyms: Malignant Ciliary Body Medulloepithelioma; Malignant Medulloepithelioma of Ciliary Body; Malignant medulloepithelioma of ciliary body; Malignant Medulloepithelioma of the Ciliary Body; Medulloepithelioma of ciliary body
SNOMED CT: Malignant medulloepithelioma of ciliary body (416901002); Medulloepithelioma of ciliary body (416901002)

Definition

A rare, unilateral, malignant 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 Malignant Medulloepithelioma

Professional guidelines

PubMed

Intraocular medulloepithelioma clinical features and management of 11 cases.
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
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
Glaucoma secondary to intraocular tumors: mechanisms and management.
Camp DA, Yadav P, Dalvin LA, Shields CL
Curr Opin Ophthalmol 2019 Mar;30(2):71-81. doi: 10.1097/ICU.0000000000000550. PMID: 30562240

Recent clinical studies

Etiology

Usefulness of PAX8 Immunohistochemistry in Adult Intraocular Tumor Diagnosis.
Mudhar HS, Milman T, Eagle RC Jr, Sanderson T, Pheasey L, Paine S, Salvi S, Rennie IG, Rundle P, Shields CL, Shields JA
Ophthalmology 2021 May;128(5):765-778. Epub 2020 Sep 29 doi: 10.1016/j.ophtha.2020.09.033. PMID: 33002562
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
DICER1 and Associated Conditions: Identification of At-risk Individuals and Recommended Surveillance Strategies.
Schultz KAP, Williams GM, Kamihara J, Stewart DR, Harris AK, Bauer AJ, Turner J, Shah R, Schneider K, Schneider KW, Carr AG, Harney LA, Baldinger S, Frazier AL, Orbach D, Schneider DT, Malkin D, Dehner LP, Messinger YH, Hill DA
Clin Cancer Res 2018 May 15;24(10):2251-2261. Epub 2018 Jan 17 doi: 10.1158/1078-0432.CCR-17-3089. PMID: 29343557Free 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

Diagnosis

Extracranial medulloepithelioma: a review of the literature.
Bailey D, Mau C, Toepke C, Finch E, Rizk E
Childs Nerv Syst 2022 Jul;38(7):1259-1266. Epub 2022 Apr 27 doi: 10.1007/s00381-022-05525-4. PMID: 35474542
Imaging of DICER1 syndrome.
Guillerman RP, Foulkes WD, Priest JR
Pediatr Radiol 2019 Oct;49(11):1488-1505. Epub 2019 Oct 16 doi: 10.1007/s00247-019-04429-x. PMID: 31620849
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
DICER1 and Associated Conditions: Identification of At-risk Individuals and Recommended Surveillance Strategies.
Schultz KAP, Williams GM, Kamihara J, Stewart DR, Harris AK, Bauer AJ, Turner J, Shah R, Schneider K, Schneider KW, Carr AG, Harney LA, Baldinger S, Frazier AL, Orbach D, Schneider DT, Malkin D, Dehner LP, Messinger YH, Hill DA
Clin Cancer Res 2018 May 15;24(10):2251-2261. Epub 2018 Jan 17 doi: 10.1158/1078-0432.CCR-17-3089. PMID: 29343557Free PMC Article
Medulloepithelioma of the ciliary body and optic nerve: clinicopathologic, CT, and MR imaging features.
Vajaranant TS, Mafee MF, Kapur R, Rapoport M, Edward DP
Neuroimaging Clin N Am 2005 Feb;15(1):69-83. doi: 10.1016/j.nic.2005.02.008. PMID: 15927861

Therapy

Successful treatment of ciliary body medulloepithelioma with intraocular melphalan chemotherapy: a case report.
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
Glaucoma secondary to intraocular tumors: mechanisms and management.
Camp DA, Yadav P, Dalvin LA, Shields CL
Curr Opin Ophthalmol 2019 Mar;30(2):71-81. doi: 10.1097/ICU.0000000000000550. PMID: 30562240
Management of medulloepithelioma of the ciliary body with brachytherapy.
Davidorf FH, Craig E, Birnbaum L, Wakely P Jr
Am J Ophthalmol 2002 Jun;133(6):841-3. doi: 10.1016/s0002-9394(02)01432-0. PMID: 12036686
Presumed bilateral medulloepithelioma.
Lumbroso L, Desjardins L, Coue O, Ducourneau Y, Pechereau A
Arch Ophthalmol 2001 Mar;119(3):449-50. PMID: 11231783
MEDULLO-EPITHELIOMA.
Sayed BA, Sahgal KN, Desai VK
Br J Ophthalmol 1962 Jan;46(1):58-60. doi: 10.1136/bjo.46.1.58. PMID: 18170756Free PMC Article

Prognosis

Intraocular medulloepithelioma clinical features and management of 11 cases.
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
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
Updates on the Molecular Pathology of Selected Ocular and Ocular Adnexal Tumors: Potential Targets for Future Therapy.
Stagner AM, Jakobiec FA
Semin Ophthalmol 2016;31(1-2):188-96. doi: 10.3109/08820538.2015.1115257. PMID: 26959146
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

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
Usefulness of PAX8 Immunohistochemistry in Adult Intraocular Tumor Diagnosis.
Mudhar HS, Milman T, Eagle RC Jr, Sanderson T, Pheasey L, Paine S, Salvi S, Rennie IG, Rundle P, Shields CL, Shields JA
Ophthalmology 2021 May;128(5):765-778. Epub 2020 Sep 29 doi: 10.1016/j.ophtha.2020.09.033. PMID: 33002562
Imaging of DICER1 syndrome.
Guillerman RP, Foulkes WD, Priest JR
Pediatr Radiol 2019 Oct;49(11):1488-1505. Epub 2019 Oct 16 doi: 10.1007/s00247-019-04429-x. PMID: 31620849
TUMORS OF THE NONPIGMENTED EPITHELIUM OF THE CILIARY BODY: The Lorenz E. Zimmerman Tribute Lecture.
Shields JA, Eagle RC Jr, Ferguson K, Shields CL
Retina 2015 May;35(5):957-65. doi: 10.1097/IAE.0000000000000445. PMID: 25545484
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

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