The results of multicenter treatment of atypical teratoid/rhabdoid  tumors of the central nervous system in children under 3 years

L. V. Olkhova; Ольхова Л. В.; O. G. Zheludkova; Желудкова О. Г.; L. S. Zubarovskaya; Зубаровская Л. С.; A. Yu. Smirnova; Смирнова А. Ю.; Yu. V. Dinikina; Диникина Ю. В.; Yu. V. Kushel; Кушель Ю. В.; A. G. Melikyan; Меликян А. Г.; S. K. Gorelyshev; Горелышев С. К.; M. V. Ryzhova; Рыжова М. В.; Yu. Yu. Trunin; Трунин Ю. Ю.; E. I. Shults; Шульц Е. И.; A. G. Gevorgyan; Геворгян А. Г.; S. V. Gorbatykh; Горбатых С. В.; A. N. Kislyakov; Кисляков А. Н.; V. E. Popov; Попов В. Е.; L. P. Privalova; Привалова Л. П.; N. B. Yudina; Юдина Н. Б.; E. M. Tarasova; Тарасова Е. М.; D. N. Pogorelov; Погорелов Д. Н.; O. B. Polushkina; Полушкина О. Б.; A. S. Levashov; Левашов А. С.; N. A. Vorobyov; Воробьёв Н. А.; N. A. Plakhotina; Плахотина Н. А.; N. I. Martynova; Мартынова Н. И.; T. Yu. Skvortsova; Скворцова Т. Ю.; A. N. Zaychikov; Зайчиков А. Н.; M. V. Mushinskaya; Мушинская М. В.; D. L. Sakun; Сакун Д. Л.; L. M. Minkina; Минкина Л. М.; T. V. Lukina; Лукина Т. В.; E. V. Shchepkina; Щепкина Е. В.; A. G. Korshunov; Коршунов А. Г.

doi:10.24287/1726-1708-2021-20-2-121-132

The results of multicenter treatment of atypical teratoid/rhabdoid tumors of the central nervous system in children under 3 years

Authors: Olkhova L.V.¹, Zheludkova O.G.², Zubarovskaya L.S.³, Smirnova A.Y.⁴, Dinikina Y.V.⁴^,5, Kushel Y.V.⁶, Melikyan A.G.⁶, Gorelyshev S.K.⁶, Ryzhova M.V.⁶, Trunin Y.Y.⁶, Shults E.I.⁶, Gevorgyan A.G.³, Gorbatykh S.V.⁷, Kislyakov A.N.⁷, Popov V.E.⁸, Privalova L.P.⁹, Yudina N.B.¹⁰, Tarasova E.M.¹, Pogorelov D.N.¹¹, Polushkina O.B.², Levashov A.S.¹², Vorobyov N.A.¹³^,14^,15, Plakhotina N.A.¹³, Martynova N.I.¹³, Skvortsova T.Y.¹⁶, Zaychikov A.N.¹⁷, Mushinskaya M.V.¹⁸, Sakun D.L.¹⁹, Minkina L.M.²⁰, Lukina T.V.²¹, Shchepkina E.V.²², Korshunov A.G.²³
Affiliations:
1. Russian Children's Clinical Hospital of the N.I. Pirogov Russian National Research Medical University of Ministry of Healthcare of the Russian Federation
2. V.F. Voino-Yasenetskiy Scientific and Practical Center of Specialized Healthсare for Children of the Department of Health of Moscow
3. R.M. Gorbacheva Research Institute for Pediatric Oncology, Hematology and Transplantation, I.P. Pavlov First Saint Petersburg State Medical University, Ministry of Healthcare of the Russian Federation
4. V.A. Almazov National Medical Research Centre of Ministry of Healthcare of the Russian Federation
5. Saint Petersburg State Pediatric Medical University of Ministry of Healthcare of the Russian Federation
6. N.N. Burdenko National Medical Research Center of Neurosurgery of Ministry of Healthcare of the Russian Federation
7. Morozov Children's City Clinical Hospital of the Department of Health of Moscow
8. M.F. Vladimirsky Moscow Regional Research Clinical Institute
9. Nizhny Novgorod Regional Children's Hospital
10. Voronezh Regional Children's Clinical Hospital No. 1
11. Regional Children's Hospital of Ministry of Healthcare of the Russian Federation, Lipetsk
12. N.N. Blokhin National Medical Research Center of Oncology of Ministry of Healthcare of the Russian Federation
13. Diagnostic and Treatment Center of the Sergey Berezin International Institute of Biological Systems
14. Saint Petersburg State University
15. I.I. Mechnikov North-Western State Medical University of Ministry of Healthcare of the Russian Federation
16. N.P. Bekhtereva Institute of the Human Brain, Russian Academy of Sciences
17. Regional Children's Clinical Hospital No.1, Yekaterinburg
18. F.P. Haass Center of Pediatric Oncology and Hematology, Regional Children's Clinical Hospital, Perm
19. Republican Children's Clinical Hospital, Simferopol
20. Regional Children's Clinical Hospital No. 1, Vladivostok
21. Diagnostic and Treatment Center of the Sergey Berezin International Institute of Biological Systems, Saint Petersburg
22. Techdepartament, Moscow Region, Khimki
23. German Cancer Research Center (DKFZ)
Issue: Vol 20, No 2 (2021)
Pages: 121-132
Section: ORIGINAL ARTICLES
Submitted: 21.05.2021
Accepted: 21.05.2021
Published: 21.05.2021
URL: https://hemoncim.com/jour/article/view/514
DOI: https://doi.org/10.24287/1726-1708-2021-20-2-121-132
ID: 514

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Abstract

Atypical teratoid/rhabdoid tumor (AT/RT) of the central nervous system (CNS) is an aggressive malignant tumor that is mainly found in younger children and is associated with poor prognosis. Our objectives: to present the results of treatment of children with CNS AT/RT under 3 years of age and assess the impact of various prognostic factors on patient survival. The study was approved by the Independent Ethics Committee and the Scientific Council of the N.I. Pirogov Russian National Research Medical University of Ministry of Healthcare of the Russian Federation. The study included 106 patients with CNS AT/RT aged 0–3 years who had been treated and monitored from 2008 to 2020. The median age was 16 (9; 23) months. All the patients underwent primary tumor resection with subsequent chemotherapy according to various protocols. At the time of the analysis, 47 patients (44.4%) were alive, 1 patient (0.9%) was lost to follow-up and 58 patients (54.7%) were dead, of whom 52 patients (90%) had died of disease progression and 6 (10%) – of polychemotherapy complications. One patient developed shunt-related intraabdominal metastasis within 10 months of the diagnosis. The 1-year progression-free survival (PFS) was 0.50; the 2-year PFS was 0.29; the 5-year PFS – 0.27. The median PFS was 12 months. The 1-year overall survival (OS) was 0.72; the 2-year OS was 0.53; the 5-year OS – 0.40. The median OS was 27 months. An analysis of patients with CNS AT/RT under 3 years of age showed that PFS was statistically significantly higher in: children aged > 12 months; children with totally resected tumours; children who had received polychemotherapy in accordance with the ATRT-2006 protocol that included radiotherapy and regional administration of a triplet of chemotherapeutic agents. The OS in patients with CNS AT/RT aged < 3 years was statistically significantly higher in: children aged > 12 months; children who had been treated with radiation therapy; patients who had received cytosar/etoposide intrathecally/intraventricularly; patients who had undergone high-dose chemotherapy with subsequent autologous hematopoietic stem cell transplantation. A multivariate analysis revealed that PFS was influenced by age, tumor location, extent of resection and exposure to radiation therapy, regional chemotherapy or high-dose chemotherapy with autologous hematopoietic stem cell transplantation, while OS was affected by age and exposure to radiation therapy.

Keywords

children, younger age, atypical teratoid/rhabdoid tumor of the central nervous system, high-dose chemotherapy, chemotherapy, radiation therapy, treatment results

About the authors

L. V. Olkhova

Russian Children's Clinical Hospital of the N.I. Pirogov Russian National Research Medical University of Ministry of Healthcare of the Russian Federation

Author for correspondence.
Email: rylkova87@mail.ru
ORCID iD: 0000-0002-7531-6443

Lyudmila V. Olkhova, a pediatric oncologist at the Department
of Bone Marrow Transplantation

117 Leninskiy Prosp, Moscow 117997

Russian Federation

O. G. Zheludkova

V.F. Voino-Yasenetskiy Scientific and Practical Center of Specialized Healthсare for Children of the Department of Health of Moscow

ORCID iD: 0000-0002-8607-3635
Russian Federation

L. S. Zubarovskaya

R.M. Gorbacheva Research Institute for Pediatric Oncology, Hematology and Transplantation, I.P. Pavlov First Saint Petersburg State Medical University, Ministry of Healthcare of the Russian Federation

ORCID iD: 0000-0003-2594-7703
Russian Federation

A. Yu. Smirnova

V.A. Almazov National Medical Research Centre of Ministry of Healthcare of the Russian Federation

ORCID iD: 0000-0002-5293-9568
Russian Federation

Yu. V. Dinikina

V.A. Almazov National Medical Research Centre of Ministry of Healthcare of the Russian Federation; Saint Petersburg State Pediatric Medical University of Ministry of Healthcare of the Russian Federation

ORCID iD: 0000-0002-2003-0982
Russian Federation