Email this article Clinical and molecular characteristics of WNT medulloblastomas
- Authors: Papusha L.I.1, Yasko L.A.1, Ektova A.P.1, Voronin K.A.1, Merishavyan A.A.1, Storozhenko I.G.1, Salnikova E.A.1, Borodina I.D.1, Nechesnyuk A.V.1, Tereshchenko G.V.1, Strumila N.A.1, Zaichikov A.N.2, Yunusova I.M.3, Khasmagomadova H.E.4, Rumyantsev A.A.5, Kubirov M.S.6, Botalova I.V.7, Mikava N.V.7, Grishina E.N.8, Gorelyshev S.K.9, Kovalenko S.G.10, Druy A.E.1, Novichkova G.A.1, Karachunsky A.I.1
-
Affiliations:
- Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation
- Regional Children’s Clinical Hospital № 1
- N.М. Kuraev Children's Republican Clinical Hospital
- Republican Oncological Dispensary
- Altai Regional Clinical Center of Protection of Motherhood and Childhood
- Morozov Children's Clinical Hospital
- Regional Children's Clinical Hospital
- Children's Republican Clinical Hospital
- N.N. Burdenko National Medical Research Center of Neurosurgery of the Ministry of Health of the Russian Federation
- Regional Oncohematological Center for Children and Adolescents
- Issue: Vol 18, No 4 (2019)
- Pages: 11-18
- Section: PROMISING STUDIES
- Submitted: 28.12.2019
- Accepted: 28.12.2019
- Published: 28.12.2019
- URL: https://hemoncim.com/jour/article/view/279
- DOI: https://doi.org/10.24287/1726-1708-2019-18-4-11-18
- ID: 279
Cite item
Full Text
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.
Keywords
About the authors
L. I. Papusha
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation
Author for correspondence.
Email: lyudmila.papusha@fcchomoscow.ru
ORCID iD: 0000-0001-7750-5216
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
Russian FederationL. A. Yasko
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation
ORCID iD: 0000-0003-3007-3772
Moscow Russian Federation
A. P. Ektova
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation
ORCID iD: 0000-0001-5461-7442
Moscow Russian Federation
K. A. Voronin
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation
ORCID iD: 0000-0001-7578-9657
Moscow Russian Federation
A. A. Merishavyan
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation
ORCID iD: 0000-0002-5310-5928
Moscow Russian Federation
I. G. Storozhenko
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation
ORCID iD: 0000-0001-6296-4305
Moscow Russian Federation
E. A. Salnikova
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation
ORCID iD: 0000-0002-9846-2793
Moscow Russian Federation
I. D. Borodina
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation
ORCID iD: 0000-0002-6805-7797
Moscow Russian Federation
A. V. Nechesnyuk
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation
ORCID iD: 0000-0002-2537-6157
Moscow Russian Federation
G. V. Tereshchenko
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation
ORCID iD: 0000-0001-7317-7104
Moscow Russian Federation
N. A. Strumila
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation
ORCID iD: 0000-0003-4164-004X
Moscow Russian Federation
A. N. Zaichikov
Regional Children’s Clinical Hospital № 1
ORCID iD: 0000-0002-0462-6591
Ekaterinburg Russian Federation
I. M. Yunusova
N.М. Kuraev Children's Republican Clinical HospitalMakhachkala Russian Federation
H. E. Khasmagomadova
Republican Oncological DispensaryGrozny Russian Federation
A. A. Rumyantsev
Altai Regional Clinical Center of Protection of Motherhood and ChildhoodBarnaul Russian Federation
M. S. Kubirov
Morozov Children's Clinical Hospital
ORCID iD: 0000-0003-1308-2175
Moscow Russian Federation
I. V. Botalova
Regional Children's Clinical HospitalPerm Russian Federation
N. V. Mikava
Regional Children's Clinical HospitalKrasnodar Russian Federation
E. N. Grishina
Children's Republican Clinical HospitalKazan Russian Federation
S. K. Gorelyshev
N.N. Burdenko National Medical Research Center of Neurosurgery of the Ministry of Health of the Russian FederationMoscow Russian Federation
S. G. Kovalenko
Regional Oncohematological Center for Children and AdolescentsChelyabinsk Russian Federation
A. E. Druy
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation
ORCID iD: 0000-0003-1308-8622
Moscow Russian Federation
G. A. Novichkova
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation
ORCID iD: 0000-0002-2322-5734
Moscow Russian Federation
A. I. Karachunsky
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian FederationMoscow Russian Federation
References
Supplementary files
