Clinical and Molecular Characteristics of WNT Medulloblastomas

L. I. Papusha,L. A. Yasko,A. P. Ektova,K. A. Voronin,A. A. Merishavyan, I. G. Storozhenko,E. A. Salnikova,I. D. Borodina,A. V. Nechesnyuk,G. V. Tereshchenko,N. A. Strumila,A. N. Zaichikov, I. M. Yunusova, H. E. Khasmagomadova, A. A. Rumyantsev, M. S. Kubirov, I. V. Botalova, N. V. Mikava, E. N. Grishina,S. K. Gorelyshev, S. G. Kovalenko,A. E. Druy,G. A. Novichkova,A. I. Karachunsky

Voprosy gematologii/onkologii i immunopatologii v pediatrii(2019)

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Abstract
WNTMBaccounts for about 10% of all medulloblastomas and have a favorable outcome in patients under 16 years according international data. We analyzed clinical and molecular characteristics of 20 patients with WNTMBpatients. The study was approved by the Independent Ethics Committee of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology. The molecular group verification was performed by Nanostring gene expression profiling. The detection ofCTNNB1andTP53gene mutations was carried out with Sanger sequencing. WNT Medulloblastoma are mainly classic (18/20 patients), non-metastatic (19/20 patients). Gross total resection was performed in 18 patients. SomaticCTNNB1mutations were found in 17 patients, somaticTP53were identified in 2 patients. 19/20 assessable patients are disease-free with a median follow-up of 23 months. One patient died from the progression of the disease. WNT MB patients have an overall favorable outcome, even for metastatic andTP53positive tumors. The reduction in the intensity of therapy is indicated forWNTMB in case of reliable identification ofWNTMB.
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