U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Medulloepithelioma

MedGen UID:
1702218
•Concept ID:
C5231013
•
Neoplastic Process
Synonyms: Medulloepithelioma of central nervous system; Medulloepithelioma of the central nervous system
SNOMED CT: Medulloepithelioma of central nervous system (818967003)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
HPO: HP:0030071
Monarch Initiative: MONDO:0003144
Orphanet: ORPHA251883

Definition

A primitive neuroectodermal tumor that originates from the cells of the embryonic medullary canal. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMedulloepithelioma

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
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
Molecular Classification and Management of Rare Pediatric Embryonal Brain Tumors.
Sin-Chan P, Li BK, Ho B, Fonseca A, Huang A
Curr Oncol Rep 2018 Jul 11;20(9):69. doi: 10.1007/s11912-018-0717-7. PMID: 29995179

Recent clinical studies

Etiology

Brain and Spinal Cord Tumors of Embryonic Origin.
Lampros M, Alexiou GA
Adv Exp Med Biol 2023;1405:405-420. doi: 10.1007/978-3-031-23705-8_15. PMID: 37452947
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
DICER1 Syndrome.
Hořínová V, Drábová K, Nosková H, Bajčiová V, Šoukalová J, Černá L, Hůrková V, Slabý O, Štěrba J
Klin Onkol 2019 Summer;32(Supplementum2):123-127. doi: 10.14735/amko2019S123. PMID: 31409088
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

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
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
Intraocular medulloepithelioma.
Saunders T, Margo CE
Arch Pathol Lab Med 2012 Feb;136(2):212-6. doi: 10.5858/arpa.2010-0669-RS. PMID: 22288972
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

Therapy

Systemic adjuvant chemotherapy for advanced malignant ocular medulloepithelioma.
Sheriff IHN, Karaa EK, Chowdhury T, Scheimberg I, Duncan C, Reddy MA, Sagoo MS
Eye (Lond) 2023 Apr;37(5):947-952. Epub 2022 Feb 1 doi: 10.1038/s41433-022-01936-4. PMID: 35105935Free 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
Curative treatment for central nervous system medulloepithelioma despite residual disease after resection. Report of two cases treated according to the GPHO Protocol HIT 2000 and review of the literature.
Müller K, Zwiener I, Welker H, Maass E, Bongartz R, Berthold F, Pietsch T, Warmuth-Metz M, von Bueren A, Rutkowski S
Strahlenther Onkol 2011 Nov;187(11):757-62. Epub 2011 Oct 28 doi: 10.1007/s00066-011-2256-0. PMID: 22037651
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

Brain and Spinal Cord Tumors of Embryonic Origin.
Lampros M, Alexiou GA
Adv Exp Med Biol 2023;1405:405-420. doi: 10.1007/978-3-031-23705-8_15. PMID: 37452947
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
Intraocular medulloepithelioma.
Saunders T, Margo CE
Arch Pathol Lab Med 2012 Feb;136(2):212-6. doi: 10.5858/arpa.2010-0669-RS. PMID: 22288972
Aggressive infantile embryonal tumors.
MacDonald TJ
J Child Neurol 2008 Oct;23(10):1195-204. doi: 10.1177/0883073808321769. PMID: 18952586Free PMC Article

Clinical prediction guides

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
DICER1 Syndrome.
Hořínová V, Drábová K, Nosková H, Bajčiová V, Šoukalová J, Černá L, Hůrková V, Slabý O, Štěrba J
Klin Onkol 2019 Summer;32(Supplementum2):123-127. doi: 10.14735/amko2019S123. PMID: 31409088
DICER1 Syndrome: Characterization of the Ocular Phenotype in a Family-Based Cohort Study.
Huryn LA, Turriff A, Harney LA, Carr AG, Chevez-Barrios P, Gombos DS, Ram R, Hufnagel RB, Hill DA, Zein WM, Schultz KAP, Bishop R, Stewart DR
Ophthalmology 2019 Feb;126(2):296-304. Epub 2018 Oct 17 doi: 10.1016/j.ophtha.2018.09.038. PMID: 30339877Free PMC Article
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

Supplemental Content

Table of contents

    Clinical resources

    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.

    Reviews

    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...