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Medulloepithelioma Not Otherwise Specified

MedGen UID:
87272
•Concept ID:
C0334596
•
Neoplastic Process
Synonyms: Medulloepithelioma; Medulloepitheliomas
SNOMED CT: Diktyoma, malignant (39005004); Medulloepithelioma (715903004); Medulloepithelioma (39005004); Diktyoma (39005004)

Definition

A rare, unilateral, benign or malignant embryonic neoplasm typically presenting as a cilliary body mass during childhood. It is composed of medullary epithelial cells. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMedulloepithelioma Not Otherwise Specified
Follow this link to review classifications for Medulloepithelioma Not Otherwise Specified in Orphanet.

Professional guidelines

PubMed

Management of Neuroblastoma in Pediatric Patients.
Croteau N, Nuchtern J, LaQuaglia MP
Surg Oncol Clin N Am 2021 Apr;30(2):291-304. Epub 2021 Feb 10 doi: 10.1016/j.soc.2020.11.010. PMID: 33706901
Updates in Diagnosis, Management, and Treatment of Neuroblastoma.
Swift CC, Eklund MJ, Kraveka JM, Alazraki AL
Radiographics 2018 Mar-Apr;38(2):566-580. doi: 10.1148/rg.2018170132. PMID: 29528815
Overview and recent advances in the treatment of neuroblastoma.
Whittle SB, Smith V, Doherty E, Zhao S, McCarty S, Zage PE
Expert Rev Anticancer Ther 2017 Apr;17(4):369-386. Epub 2017 Mar 15 doi: 10.1080/14737140.2017.1285230. PMID: 28142287

Recent clinical studies

Etiology

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
Embryonal brain tumors.
McGovern SL, Grosshans D, Mahajan A
Cancer J 2014 Nov-Dec;20(6):397-402. doi: 10.1097/PPO.0000000000000081. PMID: 25415685
Dysembryoplastic neuroepithelial tumours.
Daumas-Duport C
Brain Pathol 1993 Jul;3(3):283-95. doi: 10.1111/j.1750-3639.1993.tb00755.x. PMID: 8293188
Esthesioneuroblastoma.
Shah JP, Feghali J
Am J Surg 1981 Oct;142(4):456-8. doi: 10.1016/0002-9610(81)90374-3. PMID: 7283046
Esthesioneuroblastoma.
Elkon D, Hightower SI, Lim ML, Cantrell RW, Constable WC
Cancer 1979 Sep;44(3):1087-94. doi: 10.1002/1097-0142(197909)44:3<1087::aid-cncr2820440343>3.0.co;2-a. PMID: 383268

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
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
Ewing sarcoma.
Choi EY, Gardner JM, Lucas DR, McHugh JB, Patel RM
Semin Diagn Pathol 2014 Jan;31(1):39-47. Epub 2014 Jan 5 doi: 10.1053/j.semdp.2014.01.002. PMID: 24680181
Translocation-related sarcomas.
Mertens F, Antonescu CR, Hohenberger P, Ladanyi M, Modena P, D'Incalci M, Casali PG, Aglietta M, Alvegård T
Semin Oncol 2009 Aug;36(4):312-23. doi: 10.1053/j.seminoncol.2009.06.004. PMID: 19664492
Esthesioneuroblastoma.
Shah JP, Feghali J
Am J Surg 1981 Oct;142(4):456-8. doi: 10.1016/0002-9610(81)90374-3. PMID: 7283046

Therapy

Temozolomide with irinotecan versus temozolomide, irinotecan plus bevacizumab for recurrent medulloblastoma of childhood: Report of a COG randomized Phase II screening trial.
Levy AS, Krailo M, Chi S, Villaluna D, Springer L, Williams-Hughes C, Fouladi M, Gajjar A
Pediatr Blood Cancer 2021 Aug;68(8):e29031. Epub 2021 Apr 12 doi: 10.1002/pbc.29031. PMID: 33844469Free PMC Article
Effect of Tandem Autologous Stem Cell Transplant vs Single Transplant on Event-Free Survival in Patients With High-Risk Neuroblastoma: A Randomized Clinical Trial.
Park JR, Kreissman SG, London WB, Naranjo A, Cohn SL, Hogarty MD, Tenney SC, Haas-Kogan D, Shaw PJ, Kraveka JM, Roberts SS, Geiger JD, Doski JJ, Voss SD, Maris JM, Grupp SA, Diller L
JAMA 2019 Aug 27;322(8):746-755. doi: 10.1001/jama.2019.11642. PMID: 31454045Free PMC Article
Embryonal brain tumors.
McGovern SL, Grosshans D, Mahajan A
Cancer J 2014 Nov-Dec;20(6):397-402. doi: 10.1097/PPO.0000000000000081. PMID: 25415685
Neuroblastoma.
Davidoff AM
Semin Pediatr Surg 2012 Feb;21(1):2-14. doi: 10.1053/j.sempedsurg.2011.10.009. PMID: 22248965Free PMC Article
Addition of ifosfamide and etoposide to standard chemotherapy for Ewing's sarcoma and primitive neuroectodermal tumor of bone.
Grier HE, Krailo MD, Tarbell NJ, Link MP, Fryer CJ, Pritchard DJ, Gebhardt MC, Dickman PS, Perlman EJ, Meyers PA, Donaldson SS, Moore S, Rausen AR, Vietti TJ, Miser JS
N Engl J Med 2003 Feb 20;348(8):694-701. doi: 10.1056/NEJMoa020890. PMID: 12594313

Prognosis

Embryonal tumors in the WHO CNS5 classification: A Review.
Gianno F, Miele E, Antonelli M, Giangaspero F
Indian J Pathol Microbiol 2022 May;65(Supplement):S73-S82. doi: 10.4103/ijpm.ijpm_1049_21. PMID: 35562137
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
Ewing sarcoma.
Choi EY, Gardner JM, Lucas DR, McHugh JB, Patel RM
Semin Diagn Pathol 2014 Jan;31(1):39-47. Epub 2014 Jan 5 doi: 10.1053/j.semdp.2014.01.002. PMID: 24680181
Translocation-related sarcomas.
Mertens F, Antonescu CR, Hohenberger P, Ladanyi M, Modena P, D'Incalci M, Casali PG, Aglietta M, Alvegård T
Semin Oncol 2009 Aug;36(4):312-23. doi: 10.1053/j.seminoncol.2009.06.004. PMID: 19664492
Olfactory esthesioneuroblastoma.
Jensen KJ, Elbrond O, Lund C
J Laryngol Otol 1976 Nov;90(11):1007-13. doi: 10.1017/s0022215100083043. PMID: 1003025

Clinical prediction guides

Metabolism of pancreatic neuroendocrine tumors: what can omics tell us?
Jannin A, Dessein AF, Do Cao C, Vantyghem MC, Chevalier B, Van Seuningen I, Jonckheere N, Coppin L
Front Endocrinol (Lausanne) 2023;14:1248575. Epub 2023 Oct 16 doi: 10.3389/fendo.2023.1248575. PMID: 37908747Free PMC Article
A preoperative computed tomography radiomics model to predict disease-free survival in patients with pancreatic neuroendocrine tumors.
Homps M, Soyer P, Coriat R, Dermine S, Pellat A, Fuks D, Marchese U, Terris B, Groussin L, Dohan A, Barat M
Eur J Endocrinol 2023 Oct 17;189(4):476-484. doi: 10.1093/ejendo/lvad130. PMID: 37787635
FDA Approval Summary: Belzutifan for von Hippel-Lindau Disease-Associated Tumors.
Fallah J, Brave MH, Weinstock C, Mehta GU, Bradford D, Gittleman H, Bloomquist EW, Charlab R, Hamed SS, Miller CP, Dorff SE, Chambers WA, Mixter BD, Dinin J, Pierce WF, Ricks TK, Tang S, Donoghue M, Pazdur R, Amiri-Kordestani L, Ibrahim A, Beaver JA
Clin Cancer Res 2022 Nov 14;28(22):4843-4848. doi: 10.1158/1078-0432.CCR-22-1054. PMID: 35727604Free PMC Article
Temozolomide with irinotecan versus temozolomide, irinotecan plus bevacizumab for recurrent medulloblastoma of childhood: Report of a COG randomized Phase II screening trial.
Levy AS, Krailo M, Chi S, Villaluna D, Springer L, Williams-Hughes C, Fouladi M, Gajjar A
Pediatr Blood Cancer 2021 Aug;68(8):e29031. Epub 2021 Apr 12 doi: 10.1002/pbc.29031. PMID: 33844469Free PMC Article
Renal Ewing tumors.
Zöllner S, Dirksen U, Jürgens H, Ranft A
Ann Oncol 2013 Sep;24(9):2455-61. Epub 2013 Jun 11 doi: 10.1093/annonc/mdt215. PMID: 23761687

Recent systematic reviews

Metformin and pancreatic neuroendocrine tumors: A systematic review and meta-analysis.
Cigrovski Berkovic M, Coppola A, Sesa V, Mrzljak A, Lai Q
World J Gastroenterol 2024 Feb 21;30(7):759-769. doi: 10.3748/wjg.v30.i7.759. PMID: 38515954Free PMC Article
Metabolism of pancreatic neuroendocrine tumors: what can omics tell us?
Jannin A, Dessein AF, Do Cao C, Vantyghem MC, Chevalier B, Van Seuningen I, Jonckheere N, Coppin L
Front Endocrinol (Lausanne) 2023;14:1248575. Epub 2023 Oct 16 doi: 10.3389/fendo.2023.1248575. PMID: 37908747Free PMC Article
Agricultural exposures and risk of childhood neuroblastoma: a systematic review and meta-analysis.
Hymel E, Degarege A, Fritch J, Farazi E, Napit K, Coulter D, Schmidt C, Watanabe-Galloway S
Environ Sci Pollut Res Int 2023 Nov;30(53):113193-113204. Epub 2023 Oct 19 doi: 10.1007/s11356-023-30315-z. PMID: 37858025
The SH-SY5Y human neuroblastoma cell line, a relevant in vitro cell model for investigating neurotoxicology in human: Focus on organic pollutants.
Lopez-Suarez L, Awabdh SA, Coumoul X, Chauvet C
Neurotoxicology 2022 Sep;92:131-155. Epub 2022 Jul 29 doi: 10.1016/j.neuro.2022.07.008. PMID: 35914637
ROHHAD(NET) Syndrome: Systematic Review of the Clinical Timeline and Recommendations for Diagnosis and Prognosis.
Harvengt J, Gernay C, Mastouri M, Farhat N, Lebrethon MC, Seghaye MC, Bours V
J Clin Endocrinol Metab 2020 Jul 1;105(7) doi: 10.1210/clinem/dgaa247. PMID: 32407531

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