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Medulloblastoma with extensive nodularity(MBEN)

MedGen UID:
233759
Concept ID:
C1334970
Neoplastic Process
Synonyms: MBEN; Nodular medulloblastoma
SNOMED CT: Medulloblastoma with extensive nodularity (733902001)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0016710
Orphanet: ORPHA251858

Definition

Medulloblastoma with extensive nodularity (MBEN) is a histological variant of medulloblastoma (see this term), an embryonic malignancy, most often located in the inferior medullary velum and then growing into the fourth ventricle, and presenting in infants and young children with symptoms of increased intracranial pressure such as headache, listlessness, vomiting, diplopia and papilledema. It is often associated with Gorlin syndrome (see this term) and has a relatively good prognosis. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMedulloblastoma with extensive nodularity

Professional guidelines

PubMed

Naung H, Cohen KJ
J Neurooncol 2021 May;152(3):567-572. Epub 2021 Mar 10 doi: 10.1007/s11060-021-03727-5. PMID: 33689104
Sadighi Z, Vats T, Khatua S
J Child Neurol 2012 Oct;27(10):1302-7. Epub 2012 Jul 22 doi: 10.1177/0883073812449690. PMID: 22826514

Recent clinical studies

Etiology

Badiger S, Gudipati A, Uppin M, Konatam ML, Yeramneni VK, Bhattacharjee S, Saradhi MV, Patnaik S, Irukulla M
J Cancer Res Ther 2023 Jan 1;19(Suppl 2):S592-S602. Epub 2023 Apr 29 doi: 10.4103/jcrt.jcrt_1268_22. PMID: 38384024
Cassia GSE, Alves CAPF, Taranath A, López NS, Oztekin O, Gonçalves FG, Patay Z
Top Magn Reson Imaging 2018 Dec;27(6):479-502. doi: 10.1097/RMR.0000000000000184. PMID: 30516696
Korshunov A, Sahm F, Stichel D, Schrimpf D, Ryzhova M, Zheludkova O, Golanov A, Lichter P, Jones DTW, von Deimling A, Pfister SM, Kool M
Acta Neuropathol 2018 Aug;136(2):303-313. Epub 2018 Mar 22 doi: 10.1007/s00401-018-1840-0. PMID: 29569031
Millard NE, De Braganca KC
J Child Neurol 2016 Oct;31(12):1341-53. Epub 2015 Sep 2 doi: 10.1177/0883073815600866. PMID: 26336203Free PMC Article
Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, Burger PC, Jouvet A, Scheithauer BW, Kleihues P
Acta Neuropathol 2007 Aug;114(2):97-109. Epub 2007 Jul 6 doi: 10.1007/s00401-007-0243-4. PMID: 17618441Free PMC Article

Diagnosis

Cassia GSE, Alves CAPF, Taranath A, López NS, Oztekin O, Gonçalves FG, Patay Z
Top Magn Reson Imaging 2018 Dec;27(6):479-502. doi: 10.1097/RMR.0000000000000184. PMID: 30516696
Aristizabal P, Burns L, Rivera-Gomez R, Levy ML, Roberts W, Crawford JR
J Pediatr Hematol Oncol 2017 May;39(4):299-301. doi: 10.1097/MPH.0000000000000798. PMID: 28267079
Millard NE, De Braganca KC
J Child Neurol 2016 Oct;31(12):1341-53. Epub 2015 Sep 2 doi: 10.1177/0883073815600866. PMID: 26336203Free PMC Article
Sadighi Z, Vats T, Khatua S
J Child Neurol 2012 Oct;27(10):1302-7. Epub 2012 Jul 22 doi: 10.1177/0883073812449690. PMID: 22826514
Chelliah D, Mensah Sarfo-Poku C, Stea BD, Gardetto J, Zumwalt J
Pediatr Neurosurg 2010;46(5):381-4. Epub 2011 Mar 9 doi: 10.1159/000322896. PMID: 21389751

Therapy

Badiger S, Gudipati A, Uppin M, Konatam ML, Yeramneni VK, Bhattacharjee S, Saradhi MV, Patnaik S, Irukulla M
J Cancer Res Ther 2023 Jan 1;19(Suppl 2):S592-S602. Epub 2023 Apr 29 doi: 10.4103/jcrt.jcrt_1268_22. PMID: 38384024
Mynarek M, von Hoff K, Pietsch T, Ottensmeier H, Warmuth-Metz M, Bison B, Pfister S, Korshunov A, Sharma T, Jaeger N, Ryzhova M, Zheludkova O, Golanov A, Rushing EJ, Hasselblatt M, Koch A, Schüller U, von Deimling A, Sahm F, Sill M, Riemenschneider MJ, Dohmen H, Monoranu CM, Sommer C, Staszewski O, Mawrin C, Schittenhelm J, Brück W, Filipski K, Hartmann C, Meinhardt M, Pietschmann K, Haberler C, Slavc I, Gerber NU, Grotzer M, Benesch M, Schlegel PG, Deinlein F, von Bueren AO, Friedrich C, Juhnke BO, Obrecht D, Fleischhack G, Kwiecien R, Faldum A, Kortmann RD, Kool M, Rutkowski S
J Clin Oncol 2020 Jun 20;38(18):2028-2040. Epub 2020 Apr 24 doi: 10.1200/JCO.19.03057. PMID: 32330099
Lafay-Cousin L, Bouffet E, Strother D, Rudneva V, Hawkins C, Eberhart C, Horbinski C, Heier L, Souweidane M, Williams-Hughes C, Onar-Thomas A, Billups CA, Fouladi M, Northcott P, Robinson G, Gajjar A
J Clin Oncol 2020 Jan 20;38(3):223-231. Epub 2019 Nov 27 doi: 10.1200/JCO.19.00845. PMID: 31774708Free PMC Article
Aristizabal P, Burns L, Rivera-Gomez R, Levy ML, Roberts W, Crawford JR
J Pediatr Hematol Oncol 2017 May;39(4):299-301. doi: 10.1097/MPH.0000000000000798. PMID: 28267079

Prognosis

Badiger S, Gudipati A, Uppin M, Konatam ML, Yeramneni VK, Bhattacharjee S, Saradhi MV, Patnaik S, Irukulla M
J Cancer Res Ther 2023 Jan 1;19(Suppl 2):S592-S602. Epub 2023 Apr 29 doi: 10.4103/jcrt.jcrt_1268_22. PMID: 38384024
Korshunov A, Sahm F, Stichel D, Schrimpf D, Ryzhova M, Zheludkova O, Golanov A, Lichter P, Jones DTW, von Deimling A, Pfister SM, Kool M
Acta Neuropathol 2018 Aug;136(2):303-313. Epub 2018 Mar 22 doi: 10.1007/s00401-018-1840-0. PMID: 29569031
Fudyma IA, Wadhwani NR
Pol J Pathol 2017;68(4):364-366. doi: 10.5114/pjp.2017.73934. PMID: 29517209
Millard NE, De Braganca KC
J Child Neurol 2016 Oct;31(12):1341-53. Epub 2015 Sep 2 doi: 10.1177/0883073815600866. PMID: 26336203Free PMC Article
Jóźwiak J, Sontowska I, Bikowska B, Grajkowska W, Galus R, Roszkowski M
Folia Neuropathol 2011;49(4):257-61. PMID: 22212915

Clinical prediction guides

Badiger S, Gudipati A, Uppin M, Konatam ML, Yeramneni VK, Bhattacharjee S, Saradhi MV, Patnaik S, Irukulla M
J Cancer Res Ther 2023 Jan 1;19(Suppl 2):S592-S602. Epub 2023 Apr 29 doi: 10.4103/jcrt.jcrt_1268_22. PMID: 38384024
Tonn S, Korshunov A, Obrecht D, Sill M, Spohn M, von Hoff K, Milde T, Pietsch T, Goschzik T, Bison B, Juhnke BO, Struve N, Sturm D, Sahm F, Bockmayr M, Friedrich C, von Bueren AO, Gerber NU, Benesch M, Jones DTW, Kool M, Wefers AK, Schüller U, Pfister SM, Rutkowski S, Mynarek M
Neuro Oncol 2023 Aug 3;25(8):1518-1529. doi: 10.1093/neuonc/noad027. PMID: 36715306Free PMC Article
Naung H, Cohen KJ
J Neurooncol 2021 May;152(3):567-572. Epub 2021 Mar 10 doi: 10.1007/s11060-021-03727-5. PMID: 33689104
Alexiou GA, Vartholomatos G, Stefanaki K, Patereli A, Dova L, Karamoutsios A, Lallas G, Sfakianos G, Moschovi M, Prodromou N
J Neurosurg Pediatr 2013 Nov;12(5):452-7. Epub 2013 Aug 30 doi: 10.3171/2013.7.PEDS1376. PMID: 23992239
Yeom KW, Mobley BC, Lober RM, Andre JB, Partap S, Vogel H, Barnes PD
AJR Am J Roentgenol 2013 Apr;200(4):895-903. doi: 10.2214/AJR.12.9249. PMID: 23521467

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