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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
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
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

Concurrent medulloblastoma and cardiac fibroma: a rare presentation of Gorlin-Goltz syndrome.
Alanazi R, Alkhaibary A, Alfaqawwy W, AlSufiani F, Ahmad N, Aljared T
Childs Nerv Syst 2023 Sep;39(9):2499-2504. Epub 2023 May 9 doi: 10.1007/s00381-023-05970-9. PMID: 37160435
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
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

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

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
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

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
Cytotoxic effects of chemotherapeutic drugs and heterocyclic compounds at application on the cells of primary culture of neuroepithelium tumors.
Kulchitsky VA, Potkin VI, Zubenko YS, Chernov AN, Talabaev MV, Demidchik YE, Petkevich SK, Kazbanov VV, Gurinovich TA, Roeva MO, Grigoriev DG, Kletskov AV, Kalunov VN
Med Chem 2012 Jan;8(1):22-32. doi: 10.2174/157340612799278298. PMID: 22420547

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