U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Desmoplastic/nodular medulloblastoma

MedGen UID:
148272
•Concept ID:
C0751291
•
Neoplastic Process
Synonyms: Medulloblastoma, desmoplastic; Medulloblastoma, desmoplastic, somatic
SNOMED CT: Desmoplastic nodular medulloblastoma (32456001); Desmoplastic medulloblastoma (32456001); Circumscribed arachnoidal cerebellar sarcoma (32456001)
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)
 
Monarch Initiative: MONDO:0016711
Orphanet: ORPHA251863

Definition

Medulloblastoma with extensive nodularity and advanced neuronal differentiation. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDesmoplastic/nodular medulloblastoma
Follow this link to review classifications for Desmoplastic/nodular medulloblastoma in Orphanet.

Professional guidelines

PubMed

An intrathecal limited postoperative chemotherapy regimen for the treatment of young children with nodular/desmoplastic medulloblastoma and medulloblastoma with extensive nodularity.
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
Differentiation of Outcomes by Treatment Regimen and Histology in Central Nervous System Primary Embryonal Tumors.
Bhimani AD, Ryoo JS, Reddy AK, Denyer S, McGuire LS, Alonso M, Mehta AI
World Neurosurg 2020 Sep;141:e289-e306. Epub 2020 May 17 doi: 10.1016/j.wneu.2020.05.103. PMID: 32434022
Desmoplastic nodular medulloblastoma in young children: a management dilemma.
AbdelBaki MS, Boué DR, Finlay JL, Kieran MW
Neuro Oncol 2018 Jul 5;20(8):1026-1033. doi: 10.1093/neuonc/nox222. PMID: 29156007Free PMC Article

Recent clinical studies

Etiology

Risk prediction in early childhood sonic hedgehog medulloblastoma treated with radiation-avoiding chemotherapy: Evidence for more than 2 subgroups.
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
Childhood Medulloblastoma Revisited.
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
Desmoplastic nodular medulloblastoma in young children: a management dilemma.
AbdelBaki MS, Boué DR, Finlay JL, Kieran MW
Neuro Oncol 2018 Jul 5;20(8):1026-1033. doi: 10.1093/neuonc/nox222. PMID: 29156007Free PMC Article
Medulloblastoma With Extensive Nodularity: Tailored Therapy in a Low-resource Setting.
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
Clinical, pathological, and molecular data on desmoplastic/nodular medulloblastoma: case studies and a review of the literature.
Siegfried A, Bertozzi AI, Bourdeaut F, Sevely A, Loukh N, Grison C, Miquel C, Lafon D, Sevenet N, Pietsch T, Dufour C, Delisle MB
Clin Neuropathol 2016 May-Jun;35(3):106-13. doi: 10.5414/NP300205. PMID: 26857864Free PMC Article

Diagnosis

Molecular Subgrouping Based on Immunohistochemistry in Medulloblastoma: A Single-Center Experience.
Babaoglu B, Hanalioglu S, Varan A, Oguz KK, Bilginer B, Dolgun A, Soylemezoglu F
Turk Neurosurg 2024;34(6):999-1008. doi: 10.5137/1019-5149.JTN.45863-23.2. PMID: 38874255
Extra-axial adult cerebellopontine angle medulloblastoma: Revisiting a rare entity.
Pant I, Chaturvedi S, S Gautam VK, Sarma P, Satti DK
J Cancer Res Ther 2022 Apr-Jun;18(3):770-773. doi: 10.4103/jcrt.JCRT_675_20. PMID: 35900553
Childhood Medulloblastoma Revisited.
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
Medulloblastoma With Extensive Nodularity: Tailored Therapy in a Low-resource Setting.
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
Clinical, pathological, and molecular data on desmoplastic/nodular medulloblastoma: case studies and a review of the literature.
Siegfried A, Bertozzi AI, Bourdeaut F, Sevely A, Loukh N, Grison C, Miquel C, Lafon D, Sevenet N, Pietsch T, Dufour C, Delisle MB
Clin Neuropathol 2016 May-Jun;35(3):106-13. doi: 10.5414/NP300205. PMID: 26857864Free PMC Article

Therapy

Desmoplastic nodular medulloblastoma in young children: a management dilemma.
AbdelBaki MS, Boué DR, Finlay JL, Kieran MW
Neuro Oncol 2018 Jul 5;20(8):1026-1033. doi: 10.1093/neuonc/nox222. PMID: 29156007Free PMC Article
Medulloblastoma With Extensive Nodularity: Tailored Therapy in a Low-resource Setting.
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
Recurrent Cerebellar Desmoplastic/Nodular Medulloblastoma in Cerebrospinal Fluid (CSF) in the Elderly. A Cytologic Diagnosis.
Kalogeraki A, Tamiolakis D, Mavrigiannaki P, Karvelaskalogerakis M, Datseri G, Agelaki S, Papadopoulos S
Rom J Intern Med 2016 Apr-Jun;54(2):137-9. doi: 10.1515/rjim-2016-0018. PMID: 27352444
Cavernous angioma after chemotherapy for desmoplastic/nodular medulloblastoma associated with anhidrotic ectodermal dysplasia.
Yamasaki F, Takayasu T, Nosaka R, Kawaguchi H, Sugiyama K, Kobayashi M, Kurisu K
Childs Nerv Syst 2016 Feb;32(2):395-8. Epub 2015 Aug 1 doi: 10.1007/s00381-015-2848-6. PMID: 26231569
Medulloblastoma in China: clinicopathologic analyses of SHH, WNT, and non-SHH/WNT molecular subgroups reveal different therapeutic responses to adjuvant chemotherapy.
Zhang ZY, Xu J, Ren Y, Li KK, Ng HK, Mao Y, Zhong P, Yao Y, Zhou LF
PLoS One 2014;9(6):e99490. Epub 2014 Jun 16 doi: 10.1371/journal.pone.0099490. PMID: 24932704Free PMC Article

Prognosis

Molecular Subgrouping Based on Immunohistochemistry in Medulloblastoma: A Single-Center Experience.
Babaoglu B, Hanalioglu S, Varan A, Oguz KK, Bilginer B, Dolgun A, Soylemezoglu F
Turk Neurosurg 2024;34(6):999-1008. doi: 10.5137/1019-5149.JTN.45863-23.2. PMID: 38874255
Risk prediction in early childhood sonic hedgehog medulloblastoma treated with radiation-avoiding chemotherapy: Evidence for more than 2 subgroups.
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
Desmoplastic nodular medulloblastoma in young children: a management dilemma.
AbdelBaki MS, Boué DR, Finlay JL, Kieran MW
Neuro Oncol 2018 Jul 5;20(8):1026-1033. doi: 10.1093/neuonc/nox222. PMID: 29156007Free PMC Article
Characteristics of medulloblastoma in children under age of three years.
Ryzhova MV, Zheludkova OG, Kumirova ÉV, Shishkina LV, Panina TN, Gorelyshev SK, Khukhlaeva EA, Mazerkina NA, Matuev KB, Medvedeva OA, Tarasova EM, Kholodov BV, Kapitul'skaia OIu
Zh Vopr Neirokhir Im N N Burdenko 2013;77(1):3-10; discussion 11. PMID: 23659115
Histologic features and prognosis in pediatric medulloblastoma.
Verma S, Tavaré CJ, Gilles FH
Pediatr Dev Pathol 2008 Sep-Oct;11(5):337-43. Epub 2008 Jan 17 doi: 10.2350/07-09-0353.1. PMID: 18201118

Clinical prediction guides

Molecular Subgrouping Based on Immunohistochemistry in Medulloblastoma: A Single-Center Experience.
Babaoglu B, Hanalioglu S, Varan A, Oguz KK, Bilginer B, Dolgun A, Soylemezoglu F
Turk Neurosurg 2024;34(6):999-1008. doi: 10.5137/1019-5149.JTN.45863-23.2. PMID: 38874255
Risk prediction in early childhood sonic hedgehog medulloblastoma treated with radiation-avoiding chemotherapy: Evidence for more than 2 subgroups.
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
Congenital medulloblastoma: Fetal and postnatal longitudinal observation with quantitative MRI.
Korostyshevskaya AM, Savelov AA, Papusha LI, Druy AE, Yarnykh VL
Clin Imaging 2018 Nov-Dec;52:172-176. Epub 2018 Jun 7 doi: 10.1016/j.clinimag.2018.06.001. PMID: 30096555
SHH desmoplastic/nodular medulloblastoma and Gorlin syndrome in the setting of Down syndrome: case report, molecular profiling, and review of the literature.
Mangum R, Varga E, Boué DR, Capper D, Benesch M, Leonard J, Osorio DS, Pierson CR, Zumberge N, Sahm F, Schrimpf D, Pfister SM, Finlay JL
Childs Nerv Syst 2016 Dec;32(12):2439-2446. Epub 2016 Jul 21 doi: 10.1007/s00381-016-3185-0. PMID: 27444290
Characteristics of medulloblastoma in children under age of three years.
Ryzhova MV, Zheludkova OG, Kumirova ÉV, Shishkina LV, Panina TN, Gorelyshev SK, Khukhlaeva EA, Mazerkina NA, Matuev KB, Medvedeva OA, Tarasova EM, Kholodov BV, Kapitul'skaia OIu
Zh Vopr Neirokhir Im N N Burdenko 2013;77(1):3-10; discussion 11. PMID: 23659115

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

    • ClinVar
      Related medical variations
    • GTR
      Related information in GTR
    • GTR(Clinical)
      Clinical tests in GTR
    • MeSH
      Related Medical Subject Headings
    • 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...