Subgroup and subtype-specific outcomes in adult medulloblastoma

Medulloblastoma, a common pediatric malignant central nervous system tumour, represent a small proportion of brain tumours in adults. Previously it has been shown that in adults, Sonic Hedgehog (SHH)-activated tumours predominate, with Wingless-type (WNT) and Group 4 being less common, but molecular...

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Veröffentlicht in:Acta neuropathologica 2021-11, Vol.142 (5), p.859-871
Hauptverfasser: Coltin, Hallie, Sundaresan, Lakshmikirupa, Smith, Kyle S., Skowron, Patryk, Massimi, Luca, Eberhart, Charles G., Schreck, Karisa C., Gupta, Nalin, Weiss, William A., Tirapelli, Daniela, Carlotti, Carlos, Li, Kay K. W., Ryzhova, Marina, Golanov, Andrey, Zheludkova, Olga, Absalyamova, Oksana, Okonechnikov, Konstantin, Stichel, Damian, von Deimling, Andreas, Giannini, Caterina, Raskin, Scott, Van Meir, Erwin G., Chan, Jennifer A., Fults, Daniel, Chambless, Lola B., Kim, Seung-Ki, Vasiljevic, Alexandre, Faure-Conter, Cecile, Vibhakar, Rajeev, Jung, Shin, Leary, Sarah, Mora, Jaume, McLendon, Roger E., Pollack, Ian F., Hauser, Peter, Grajkowska, Wieslawa A., Rubin, Joshua B., van Veelen, Marie-Lise C., French, Pim J., Kros, Johan M., Liau, Linda M., Pfister, Stefan M., Kool, Marcel, Kijima, Noriyuki, Taylor, Michael D., Packer, Roger J., Northcott, Paul A., Korshunov, Andrey, Ramaswamy, Vijay
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Sprache:eng
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Zusammenfassung:Medulloblastoma, a common pediatric malignant central nervous system tumour, represent a small proportion of brain tumours in adults. Previously it has been shown that in adults, Sonic Hedgehog (SHH)-activated tumours predominate, with Wingless-type (WNT) and Group 4 being less common, but molecular risk stratification remains a challenge. We performed an integrated analysis consisting of genome-wide methylation profiling, copy number profiling, somatic nucleotide variants and correlation of clinical variables across a cohort of 191 adult medulloblastoma cases identified through the Medulloblastoma Advanced Genomics International Consortium. We identified 30 WNT, 112 SHH, 6 Group 3, and 41 Group 4 tumours. Patients with SHH tumours were significantly older at diagnosis compared to other subgroups ( p  
ISSN:0001-6322
1432-0533
DOI:10.1007/s00401-021-02358-4