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Ryzhova M.V.

FGBU "NII neĭrokhirurgii im. akad. N.N. Burdenko" RAMN, Moskva

Zheludkova O.G.

GU FNKTs detskoĭ gematologii, onkologii i immunologii Minzdrava RF im. D. Rogacheva, Moskva

Kumirova .V.

FGBU "Federal'nyĭ nauchno-klinicheskiĭ tsentr detskoĭ gematologii, onkologii i immunologii im. Dmitriia Rogacheva" Minzdrava RF

Shishkina L.V.

Burdenko Neurosurgery Institute, Moscow, Russia

Panina T.N.

NII neĭrokhirurgii im. akad. N.N. Burdenko RAMN, Moskva

Gorelyshev S.K.

GBU NII neĭrokhirurgii im. N.N. Burdenko

Khukhlaeva E.A.

FGBU "NII neĭrokhirurgii im. akad. N.N. Burdenko" RAMN, Moskva

Mazerkina N.A.

FGBU "NII neĭrokhirurgii im. akad. N.N. Burdenko", Moskva

Matuev K.B.

FGBU "NII neĭrokhirurgii im. akad. N.N. Burdenko" RAMN, Moskva

Medvedeva O.A.

Burdenko Neurosurgery Institute, Moscow, Russia

Tarasova E.M.

FGBU "Nauchno-issledovatel'skiĭ institut neĭrokhirurgii im. akad. N.N. Burdenko" RAMN

Kholodov B.V.

GBU NII neĭrokhirurgii im. N.N. Burdenko

Kapitul'skaia O.Iu.

Blagotvoritel'nyĭ fond pomoshchi detiam s onkogematologicheskimi i inymi tiazhelymi zabolevaniiami "Podari Zhizn'", Moskva

Medulloblastoma in children under age of three years: pathological features and clinical management

Authors:

Ryzhova M.V., Zheludkova O.G., Kumirova .V., Shishkina L.V., Panina T.N., Gorelyshev S.K., Khukhlaeva E.A., Mazerkina N.A., Matuev K.B., Medvedeva O.A., Tarasova E.M., Kholodov B.V., Kapitul'skaia O.Iu.

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Journal: Burdenko's Journal of Neurosurgery. 2013;77(1): 3‑11

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To cite this article:

Ryzhova MV, Zheludkova OG, Kumirova V, et al. . Medulloblastoma in children under age of three years: pathological features and clinical management. Burdenko's Journal of Neurosurgery. 2013;77(1):3‑11. (In Russ., In Engl.)

 
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We present a series of 51 medulloblastoma in children under three years, collected in N.N. Burdenko Neurosurgical Institute from 2000 to 2010. 57% of the tumors showed desmoplastic/nodular histology. Performed fluorescence in situ hybridization (FISH) analysis revealed the MYC oncogene amplification in 4%, the MYCN oncogene amplification - in 8%, isochromosome 17q - in 16% of cases. 9q deletion was found in 8% of desmoplastic/ nodular medulloblastomas. Our results showed that desmoplastic/nodular medulloblastoma has a positive predictive value for progression-free survival. Another feature of a biology of medulloblastomas in children younger than three years is the lack of nuclear accumulation of -catenin, and 6q deletion. Medulloblastomas with MYCN oncogene amplification often exhibit desmoplastic/nodular histology and a relatively favorable outcome. The most unfavorable prognostic marker is the MYC oncogene amplification, which in our series of 100% combined with the large cell/anaplastic medulloblastoma and isochromosome 17q - such tumors should be included in the "high risk" protocol.

Keywords:

medulloblastoma
infants
copy number aberrations

Authors:

Ryzhova M.V.

FGBU "NII neĭrokhirurgii im. akad. N.N. Burdenko" RAMN, Moskva

Zheludkova O.G.

GU FNKTs detskoĭ gematologii, onkologii i immunologii Minzdrava RF im. D. Rogacheva, Moskva

Kumirova .V.

FGBU "Federal'nyĭ nauchno-klinicheskiĭ tsentr detskoĭ gematologii, onkologii i immunologii im. Dmitriia Rogacheva" Minzdrava RF

Shishkina L.V.

Burdenko Neurosurgery Institute, Moscow, Russia

Panina T.N.

NII neĭrokhirurgii im. akad. N.N. Burdenko RAMN, Moskva

Gorelyshev S.K.

GBU NII neĭrokhirurgii im. N.N. Burdenko

Khukhlaeva E.A.

FGBU "NII neĭrokhirurgii im. akad. N.N. Burdenko" RAMN, Moskva

Mazerkina N.A.

FGBU "NII neĭrokhirurgii im. akad. N.N. Burdenko", Moskva

Matuev K.B.

FGBU "NII neĭrokhirurgii im. akad. N.N. Burdenko" RAMN, Moskva

Medvedeva O.A.

Burdenko Neurosurgery Institute, Moscow, Russia

Tarasova E.M.

FGBU "Nauchno-issledovatel'skiĭ institut neĭrokhirurgii im. akad. N.N. Burdenko" RAMN

Kholodov B.V.

GBU NII neĭrokhirurgii im. N.N. Burdenko

Kapitul'skaia O.Iu.

Blagotvoritel'nyĭ fond pomoshchi detiam s onkogematologicheskimi i inymi tiazhelymi zabolevaniiami "Podari Zhizn'", Moskva

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