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Subependymal nodules

MedGen UID:
369895
Concept ID:
C1968958
Finding
HPO: HP:0009716

Definition

Small nodular masses which originate in the subependymal region of the lateral ventricles and protrude into the ventricular cavity. They may represent subependymal hamartomas of tuberous sclerosis. [from HPO]

Term Hierarchy

Conditions with this feature

Tuberous sclerosis 1
MedGen UID:
344288
Concept ID:
C1854465
Disease or Syndrome
Tuberous sclerosis complex (TSC) involves abnormalities of the skin (hypomelanotic macules, confetti skin lesions, facial angiofibromas, shagreen patches, fibrous cephalic plaques, ungual fibromas); brain (subependymal nodules, cortical tubers, and subependymal giant cell astrocytomas [SEGAs], seizures, intellectual disability / developmental delay, psychiatric illness); kidney (angiomyolipomas, cysts, renal cell carcinomas); heart (rhabdomyomas, arrhythmias); and lungs (lymphangioleiomyomatosis [LAM], multifocal micronodular pneumonocyte hyperplasia). Central nervous system tumors are the leading cause of morbidity and mortality; renal disease is the second leading cause of early death.
Tuberous sclerosis 2
MedGen UID:
348170
Concept ID:
C1860707
Disease or Syndrome
Tuberous sclerosis complex (TSC) involves abnormalities of the skin (hypomelanotic macules, confetti skin lesions, facial angiofibromas, shagreen patches, fibrous cephalic plaques, ungual fibromas); brain (subependymal nodules, cortical tubers, and subependymal giant cell astrocytomas [SEGAs], seizures, intellectual disability / developmental delay, psychiatric illness); kidney (angiomyolipomas, cysts, renal cell carcinomas); heart (rhabdomyomas, arrhythmias); and lungs (lymphangioleiomyomatosis [LAM], multifocal micronodular pneumonocyte hyperplasia). Central nervous system tumors are the leading cause of morbidity and mortality; renal disease is the second leading cause of early death.

Professional guidelines

PubMed

Bekiesinska-Figatowska M, Sobieraj P, Pasieczna M, Szymkiewicz-Dangel J
AJNR Am J Neuroradiol 2023 Sep;44(9):1070-1076. Epub 2023 Aug 3 doi: 10.3174/ajnr.A7952. PMID: 37536734Free PMC Article
Shang S, Mei Y, Wang T, Zheng X, Chen K, Xiong S, Dong Y, Chang Y, Wu X, Kong X, Tan M, Wu L, Zhang Y, Xiao Y, Xie Y, Cai G, Chen X, Li Q
Clin Nephrol 2022 Jun;97(6):328-338. doi: 10.5414/CN110476. PMID: 35142283
Rovira À, Ruiz-Falcó ML, García-Esparza E, López-Laso E, Macaya A, Málaga I, Vázquez É, Vicente J
J Neurooncol 2014 Jun;118(2):205-223. Epub 2014 Apr 27 doi: 10.1007/s11060-014-1429-y. PMID: 24771286

Recent clinical studies

Etiology

Bekiesinska-Figatowska M, Sobieraj P, Pasieczna M, Szymkiewicz-Dangel J
AJNR Am J Neuroradiol 2023 Sep;44(9):1070-1076. Epub 2023 Aug 3 doi: 10.3174/ajnr.A7952. PMID: 37536734Free PMC Article
Patniyot I, Qubty W
Curr Pain Headache Rep 2022 Apr;26(4):281-288. Epub 2022 Feb 18 doi: 10.1007/s11916-022-01032-y. PMID: 35179724
Barnett JR, Freedman JH, Zheng H, Thiele EA, Caruso P
AJNR Am J Neuroradiol 2021 Oct;42(10):1891-1897. doi: 10.3174/ajnr.A7231. PMID: 34615647Free PMC Article
Wang MX, Segaran N, Bhalla S, Pickhardt PJ, Lubner MG, Katabathina VS, Ganeshan D
Radiographics 2021 Nov-Dec;41(7):1992-2010. Epub 2021 Sep 17 doi: 10.1148/rg.2021210103. PMID: 34534018
Dabora SL, Jozwiak S, Franz DN, Roberts PS, Nieto A, Chung J, Choy YS, Reeve MP, Thiele E, Egelhoff JC, Kasprzyk-Obara J, Domanska-Pakiela D, Kwiatkowski DJ
Am J Hum Genet 2001 Jan;68(1):64-80. Epub 2000 Dec 8 doi: 10.1086/316951. PMID: 11112665Free PMC Article

Diagnosis

Wang MX, Segaran N, Bhalla S, Pickhardt PJ, Lubner MG, Katabathina VS, Ganeshan D
Radiographics 2021 Nov-Dec;41(7):1992-2010. Epub 2021 Sep 17 doi: 10.1148/rg.2021210103. PMID: 34534018
Becker B, Strowd RE 3rd
Dermatol Clin 2019 Oct;37(4):583-606. doi: 10.1016/j.det.2019.05.015. PMID: 31466597
Dragoumi P, O'Callaghan F, Zafeiriou DI
Eur J Paediatr Neurol 2018 Nov;22(6):1027-1034. Epub 2018 Sep 12 doi: 10.1016/j.ejpn.2018.08.005. PMID: 30279084
Lu DS, Karas PJ, Krueger DA, Weiner HL
Am J Med Genet C Semin Med Genet 2018 Sep;178(3):291-298. Epub 2018 Sep 19 doi: 10.1002/ajmg.c.31647. PMID: 30230171
Boronat S, Barber I
Am J Med Genet C Semin Med Genet 2018 Sep;178(3):348-354. Epub 2018 Aug 29 doi: 10.1002/ajmg.c.31648. PMID: 30156054

Therapy

Craiu DC, Bastian AE, Zurac SA, Băilă SL, Croitoru M, Craiu M, Diaconu R, Vinţan MA, Bârcă DG
Rom J Morphol Embryol 2022 Jan-Mar;63(1):181-189. doi: 10.47162/RJME.63.1.19. PMID: 36074682Free PMC Article
d'Orio P, Pelliccia V, Biondi D, Scarpa P, Gozzo F, Revay M, Cardinale F, Tassi L, Cossu M
Seizure 2022 Apr;97:8-14. Epub 2022 Feb 27 doi: 10.1016/j.seizure.2022.02.013. PMID: 35247673
Shrot S, Lawson P, Shlomovitz O, Hoffmann C, Shrot A, Ben-Zeev B, Tzadok M
Neuroradiology 2022 Mar;64(3):611-620. Epub 2021 Sep 16 doi: 10.1007/s00234-021-02789-6. PMID: 34532765
Pluym ID, Sklansky M, Wu JY, Afshar Y, Holliman K, Devore GR, Walden A, Platt LD, Krakow D
Prenat Diagn 2020 Feb;40(3):358-364. Epub 2020 Jan 2 doi: 10.1002/pd.5613. PMID: 31742705
Cheng S, Hawkins C, Taylor MD, Bartels U
Pediatr Neurol 2015 Sep;53(3):238-242.e1. Epub 2015 Jun 14 doi: 10.1016/j.pediatrneurol.2015.05.020. PMID: 26173783

Prognosis

d'Orio P, Pelliccia V, Biondi D, Scarpa P, Gozzo F, Revay M, Cardinale F, Tassi L, Cossu M
Seizure 2022 Apr;97:8-14. Epub 2022 Feb 27 doi: 10.1016/j.seizure.2022.02.013. PMID: 35247673
Shang S, Mei Y, Wang T, Zheng X, Chen K, Xiong S, Dong Y, Chang Y, Wu X, Kong X, Tan M, Wu L, Zhang Y, Xiao Y, Xie Y, Cai G, Chen X, Li Q
Clin Nephrol 2022 Jun;97(6):328-338. doi: 10.5414/CN110476. PMID: 35142283
Vignoli A, La Briola F, Turner K, Peron A, Vannicola C, Chiesa V, Zambrelli E, Bruschi F, Viganò I, Canevini MP
Acta Neurol Scand 2021 Jul;144(1):29-40. Epub 2021 Mar 22 doi: 10.1111/ane.13416. PMID: 33748956Free PMC Article
Boy N, Heringer J, Brackmann R, Bodamer O, Seitz A, Kölker S, Harting I
Orphanet J Rare Dis 2017 Apr 24;12(1):77. doi: 10.1186/s13023-017-0612-6. PMID: 28438223Free PMC Article
Michelozzi C, Di Leo G, Galli F, Silva Barbosa F, Labriola F, Sardanelli F, Cornalba G
Childs Nerv Syst 2013 Feb;29(2):249-54. Epub 2012 Aug 31 doi: 10.1007/s00381-012-1892-8. PMID: 22936080

Clinical prediction guides

Mammadova D, Vecko J, Hofmann M, Schüssler SC, Deiters L, Canda A, Wieland AK, Gollwitzer S, Hamer H, Trollmann R
Orphanet J Rare Dis 2023 Nov 9;18(1):349. doi: 10.1186/s13023-023-02959-0. PMID: 37946245Free PMC Article
Shrot S, Lawson P, Shlomovitz O, Hoffmann C, Shrot A, Ben-Zeev B, Tzadok M
Neuroradiology 2022 Mar;64(3):611-620. Epub 2021 Sep 16 doi: 10.1007/s00234-021-02789-6. PMID: 34532765
Słowińska M, Jóźwiak S, Peron A, Borkowska J, Chmielewski D, Sadowski K, Jurkiewicz E, Vignoli A, La Briola F, Canevini MP, Kotulska-Jóźwiak K
Orphanet J Rare Dis 2018 Jan 29;13(1):25. doi: 10.1186/s13023-018-0764-z. PMID: 29378663Free PMC Article
Bai D, Zhao J, Li L, Gao J, Wang X
Sci China Life Sci 2017 Jul;60(7):763-771. Epub 2017 Jun 14 doi: 10.1007/s11427-017-9091-x. PMID: 28623545
Feliciano DM, Lin TV, Hartman NW, Bartley CM, Kubera C, Hsieh L, Lafourcade C, O'Keefe RA, Bordey A
Int J Dev Neurosci 2013 Nov;31(7):667-78. Epub 2013 Feb 26 doi: 10.1016/j.ijdevneu.2013.02.008. PMID: 23485365Free PMC Article

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