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EMBRYONAL TUMORS OF THE CENTRAL NERVOUS SYSTEM IN ADULTS: A REPORT OF THREE CASES. REVIEW OF THE LITERATURE

https://doi.org/10.21294/1814-4861-2021-20-1-105-114

Abstract

Background. Еmbryonal tumors of the central nervous system are malignant neoplasms that mainly occur in pediatric patients with a peak incidence at the age of 4 years. These tumors usually have small round blue cell histology and low differentiation.

Method and case description. A report of three cases with embryonal CNS tumors of supratentorial localization has been presented. Immunohistochemical analysis classified these tumors as neuroblastoma (2 cases: Syn (+), NSE (+), CD (+) and Ki67 10/40 %; ages were 33 and 52 years) or ganglioneuroblastoma (1 case: Syn (+), NSE (+), CD 99 (+) and Ki67 40 %; age was 37 year). All patients underwent RT in a total dose of 60 Gy delivered to the area of the removed tumor and 6 cycles of adjuvant chemotherapy: patients with neuroblastoma received chemotherapy using EP regimen (cisplatin + etoposide), and patient with ganglioneuroblastoma received temozolomide.

Results. An objective response to therapy was achieved in all 3 patients. The relapse-free survival (RFS) in the first case of neuroblastoma was 51 months, the overall survival (OS ) was more than 105 months (8 years 9 months); in the second case of neuroblastoma, RFS was 25 months 2 weeks and OS was more than 26 months. Both neuroblastomas contained ID H1(R132H) mutation. In the patient with ganglioneuroblastoma, the RFS was 87 months, and the OS was over 93 months (7 years, 9 months, 3 weeks).

Conclusion. Supratentorial embryonal tumors of the central nervous system in adults are exceptionally rare and have a relatively favorable response to the standard treatment.

About the Authors

M. V. Matsko
Polenov Russian Neurosurgical Institute – the branch of Almazov NMRC; Clinical Scientific-Practical Center of Oncology; Saint-Petersburg State University; Saint-Petersburg Medico-Social Institute
Russian Federation

MD, PhD, Senior Researcher; Oncology Department

Researcher ID (WOS): W-9626-2018.

12, Mayakovskogo Street, 191014, St. Petersburg, Russia

68a, Leningradskaya Street, 197758, St. Petersburg, Russia

7/9, Universitetskaya nab., 199034, St. Petersburg, Russia

72a, Kondratyevsky pr., 195271, St. Petersburg, Russia



D. E. Matsko
Polenov Russian Neurosurgical Institute – the branch of Almazov NMRC; Clinical Scientific-Practical Center of Oncology; Saint-Petersburg State University; Saint-Petersburg Medico-Social Institute
Russian Federation

MD, Professor

12, Mayakovskogo Street, 191014, St. Petersburg, Russia

68a, Leningradskaya Street, 197758, St. Petersburg, Russia

7/9, Universitetskaya nab., 199034, St. Petersburg, Russia

72a, Kondratyevsky pr., 195271, St. Petersburg, Russia



E. N. Imyanitov
N.N. Petrov NMRC of Oncology; I.I. Mechnikov North-Western Medical University; Saint-Petersburg State Pediatric Medical University
Russian Federation

MD, Professor, Corresponding Member of the Russian Academy of Sciences, Head of the Department of Biology of Tumor Growth

Author ID (Scopus): 7003644486

68, Leningradskaya Street, 197758, St. Petersburg, Russia

47, Piskarevsky pr., 195067, St. Petersburg, Russia

2, Litovskaya street, 194100, St. Petersburg, Russia



S. S. Sklyar
Polenov Russian Neurosurgical Institute – the branch of Almazov NMRC
Russian Federation

MD, Junior Researcher

12, Mayakovskogo Street, 191014, St. Petersburg, Russia



A. Yu. Ulitin
Polenov Russian Neurosurgical Institute – the branch of Almazov NMRC; I.I. Mechnikov North-Western Medical University
Russian Federation

MD, DSc

12, Mayakovskogo Street, 191014, St. Petersburg, Russia

47, Piskarevsky pr., 195067, St. Petersburg, Russia



I. V. Yakovenko
I.I. Mechnikov North-Western Medical University
Russian Federation

MD, Professor, Neurosurgery Department

Researcher ID (WOS): K-9705-2014

47, Piskarevsky pr., 195067, St. Petersburg, Russia


A. G. Ievleva
N.N. Petrov NMRC of Oncology; Saint-Petersburg State Pediatric Medical University
Russian Federation

MD, PhD, Senior Researcher, Department of Biology of Tumor Growth

Researcher ID (WOS): P-8305-2016. Author ID (Scopus): 6506417697

68, Leningradskaya Street, 197758, St. Petersburg, Russia

2, Litovskaya street, 194100, St. Petersburg, Russia



G. A. Ershov
Saint-Petersburg State University
Russian Federation

Student

Researcher ID (WOS): AAA-7236-2019.

7/9, Universitetskaya nab., 199034, St. Petersburg, Russia



A. A. Romanko
N.N. Petrov NMRC of Oncology
Russian Federation

Researcher, Laboratory of Molecular Oncology

68, Leningradskaya Street, 197758, St. Petersburg, Russia



A. S. Nechaeva
Polenov Russian Neurosurgical Institute – the branch of Almazov NMRC
Russian Federation

MD, Researcher

12, Mayakovskogo Street, 191014, St. Petersburg, Russia



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For citations:


Matsko M.V., Matsko D.E., Imyanitov E.N., Sklyar S.S., Ulitin A.Yu., Yakovenko I.V., Ievleva A.G., Ershov G.A., Romanko A.A., Nechaeva A.S. EMBRYONAL TUMORS OF THE CENTRAL NERVOUS SYSTEM IN ADULTS: A REPORT OF THREE CASES. REVIEW OF THE LITERATURE. Siberian journal of oncology. 2021;20(1):105-114. (In Russ.) https://doi.org/10.21294/1814-4861-2021-20-1-105-114

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ISSN 1814-4861 (Print)
ISSN 2312-3168 (Online)