multifocal_medulloblastoma

Multifocal medulloblastomas (MMBs) in adults are exceedingly rare with only 5 reported cases to date 1) 2) 3) 4) 5).

Medulloblastoma in adult differ from its childhood counterpart by being more often lateral in location, desmoplastic in morphology, and better in clinical prognosis. Little is known, however, about the characteristic features of MMB. This is particularly true for their molecular profiles. To date, molecular characteristics of multifocal medulloblastoma have been reported only once. Saad et al., present the second case of multifocal medulloblastoma along with its detailed morphology, imaging features, and molecular profiles with a critical review of the literature. They believe that MMB should be reported in detail to better understand their behavior, characterize their molecular profiles, and establish therapeutic protocols 6).


Balik et al., illustrates a very rare rapid postoperative clinical deterioration due to diffuse cerebellar swelling (DCS) in an adult multifocal MB (MMB). To the best of their knowledge, authors for the first time performed genetic analysis of MMB and demonstrated expression patterns of selected markers that put the patient within the sonic hedgehog (SHH) molecular subgroup and at least partially explain her unsatisfactory clinical course. Herein, authors summarized the relevant literature concerning this issue with the aim to determine features that would facilitate diagnosis and therapy of such a scarce clinical entity 7).


1)
Gliemroth J, Kehler U, Knopp U, Reusche E, Nowak G. A multifocal cerebellar and supratentorial medulloblastoma in an adult. Acta Neurochir (Wien). 1998;140(7):723-4. PubMed PMID: 9781288.
2)
Shen WC, Yang CF. Multifocal cerebellar medulloblastoma: CT findings. J Comput Assist Tomogr. 1988 Sep-Oct;12(5):894. PubMed PMID: 3170856.
3)
Spagnoli D, Tomei G, Masini B, De Santis A, Grimoldi N, Lucarini C, Gaini SM. A case of multifocal cerebellar medulloblastoma in an adult patient. J Neurosurg Sci. 1990 Jul-Dec;34(3-4):323-5. PubMed PMID: 2098514.
4) , 7)
Balik V, Trojanec R, Holzerova M, Tuckova L, Sulla I, Megova M, Vaverka M, Hrabalek L, Ehrmann J. An adult multifocal medulloblastoma with diffuse acute postoperative cerebellar swelling: immunohistochemical and molecular genetics analysis. Neurosurg Rev. 2015 Jan;38(1):1-10; discussion 10. doi: 10.1007/s10143-014-0556-4. PubMed PMID: 24913771.
5) , 6)
Saad AG, Balik V, Parvez A, El Jamal SM. Multifocal Supra and Infratentorial Medulloblastoma in an Adult: Histologic, Immunohistochemical, and Molecular Evaluation of a Rare Case and Review of the Literature. Appl Immunohistochem Mol Morphol. 2016 Oct 31. [Epub ahead of print] PubMed PMID: 27801730.
  • multifocal_medulloblastoma.txt
  • Last modified: 2016/11/02 16:22
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