Erschienen in:
27.04.2019 | Correspondence
Molecular progression of SHH-activated medulloblastomas
verfasst von:
Andrey Korshunov, Konstantin Okonechnikov, Felix Sahm, Marina Ryzhova, Damian Stichel, Philipp Sievers, Jochen Meyer, Daniel Schrimpf, Olga Zheludkova, Andrey Golanov, Peter Lichter, David T. W. Jones, Stefan M. Pfister, Andreas von Deimling, Marcel Kool
Erschienen in:
Acta Neuropathologica
|
Ausgabe 2/2019
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Excerpt
Medulloblastoma (MB) comprises four main molecular MB subgroups (WNT, SHH, Group 3 and Group 4) with divergent biology, outcomes and subgroup-specific differences in relapses [
1‐
6]. Metastatic recurrences are most common in Group 3 and 4 MB, where metastases from a single patient are genetically similar to each other, but highly divergent from the corresponding primary tumor [
2‐
6]. Local recurrences in the tumor bed are more frequent in SHH-activated tumors but progression-associated molecular aberrations for this MB variant remain unclear [
2,
6]. To assess a biological evolution of SHH MB, we analyzed molecular changes appearing during local regrowth of desmoplastic nodular MB (DNMB). Eleven pairs of primary and recurrent DNMB samples were analyzed with DNA- and RNA-based methods. Tumor samples were obtained by resection from three infants, two children, and six adults older than 17 years (all at M0 stage; Suppl. Table). After primary resection, infants received chemotherapy alone, while children and adults were treated according to HIT protocols that included cranio-spinal radiotherapy as well. Event-free survival varied widely for these 11 patients, ranging from 8 to 192 months, with infants on average relapsing earlier than non-infants (11 months vs. 82 months for others); all 11 “secondary” samples were obtained via resection of the local DNMB recurrences. …