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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 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
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

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 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
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

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
Long-term survival in a child with a central nervous system medulloepithelioma.
Moftakhar P, Fan X, Hurvitz CH, Black KL, Danielpour M
J Neurosurg Pediatr 2008 Nov;2(5):339-45. doi: 10.3171/PED.2008.2.11.349. PMID: 18976104
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

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
Neuroectodermal ovarian tumors: a brief overview.
Morovic A, Damjanov I
Histol Histopathol 2008 Jun;23(6):765-71. doi: 10.14670/HH-23.765. PMID: 18366014

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

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