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 https://doi.org/10.24287/1726-1708-2019-18-4-11-18
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 of CTNNB1 and TP53 gene 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. Somatic CTNNB1 mutations were found in 17 patients, somatic TP53 were 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 and TP53 positive tumors. The reduction in the intensity of therapy is indicated forWNTMB in case of reliable identification ofWNTMB.
About the Authors
L. I. Papusha
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation
Russian Federation
Russian Federation
Correspondence: Lyudmila I. Papusha, MD, PhD,Oncologist, Department of pediatric hematology, oncology Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology,Immunology Ministry of HealthcareofRussian Federation.
Address: Russia 117997, Moscow,Samory Mashela st., 1
L. A. Yasko
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation
Russian Federation
Moscow
Russian Federation
Moscow
A. P. Ektova
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation
Russian Federation
Moscow
Russian Federation
Moscow
K. A. Voronin
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation
Russian Federation
Moscow
Russian Federation
Moscow
A. A. Merishavyan
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation
Russian Federation
Moscow
Russian Federation
Moscow
I. G. Storozhenko
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation
Russian Federation
Moscow
Russian Federation
Moscow
E. A. Salnikova
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation
Russian Federation
Moscow
Russian Federation
Moscow
I. D. Borodina
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation
Russian Federation
Moscow
Russian Federation
Moscow
A. V. Nechesnyuk
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation
Russian Federation
Moscow
Russian Federation
Moscow
G. V. Tereshchenko
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation
Russian Federation
Moscow
Russian Federation
Moscow
N. A. Strumila
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation
Russian Federation
Moscow
Russian Federation
Moscow
A. N. Zaichikov
Regional Children’s Clinical Hospital № 1
Russian Federation
Ekaterinburg
Russian Federation
Ekaterinburg
I. M. Yunusova
N.М. Kuraev Children's Republican Clinical Hospital
Russian Federation
Makhachkala
Russian Federation
Makhachkala
H. E. Khasmagomadova
Republican Oncological Dispensary
Russian Federation
Grozny
Russian Federation
Grozny
A. A. Rumyantsev
Altai Regional Clinical Center of Protection of Motherhood and Childhood
Russian Federation
Barnaul
Russian Federation
Barnaul
M. S. Kubirov
Morozov Children's Clinical Hospital
Russian Federation
Moscow
Russian Federation
Moscow
I. V. Botalova
Regional Children's Clinical Hospital
Russian Federation
Perm
Russian Federation
Perm
N. V. Mikava
Regional Children's Clinical Hospital
Russian Federation
Krasnodar
Russian Federation
Krasnodar
E. N. Grishina
Children's Republican Clinical Hospital
Russian Federation
Kazan
Russian Federation
Kazan
S. K. Gorelyshev
N.N. Burdenko National Medical Research Center of Neurosurgery of the Ministry of Health of the Russian Federation
Russian Federation
Moscow
Russian Federation
Moscow
S. G. Kovalenko
Regional Oncohematological Center for Children and Adolescents
Russian Federation
Chelyabinsk
Russian Federation
Chelyabinsk
A. E. Druy
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation
Russian Federation
Moscow
Russian Federation
Moscow
G. A. Novichkova
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation
Russian Federation
Moscow
Russian Federation
Moscow
A. I. Karachunsky
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation
Russian Federation
Moscow
Russian Federation
Moscow
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Review
For citations:
Papusha L.I., Yasko L.A., Ektova A.P., Voronin K.A., Merishavyan A.A., Storozhenko I.G., Salnikova E.A., Borodina I.D., Nechesnyuk A.V., Tereshchenko G.V., Strumila N.A., Zaichikov A.N., Yunusova I.M., Khasmagomadova H.E., Rumyantsev A.A., Kubirov M.S., Botalova I.V., Mikava N.V., Grishina E.N., Gorelyshev S.K., Kovalenko S.G., Druy A.E., Novichkova G.A., Karachunsky A.I. Clinical and molecular characteristics of WNT medulloblastomas. Pediatric Hematology/Oncology and Immunopathology. 2019;18(4):11-18. (In Russ.) https://doi.org/10.24287/1726-1708-2019-18-4-11-18
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