Embryonal tumor with multilayered rosettes (ETMR): case report and literature review

Kadyrov Sh.U.; Datsieva A.A.; Terentyeva A.I.; Grigorieva M.V.; Galstyan S.A.

doi:https://doi.org/10.17116/neiro20228603163

Kadyrov Sh.U.

Burdenko Neurosurgical Center

RSCI AuthorID: 669386
Scopus AuthorID: 23473552800
ORCID: 0000-0001-5879-1333

Datsieva A.A.

Burdenko National Medical Research Center of Neurosurgery

Terentyeva A.I.

Burdenko National Medical Research Center of Neurosurgery

Grigorieva M.V.

Burdenko National Medical Research Center of Neurosurgery

Galstyan S.A.

Burdenko Neurosurgical Center

ORCID: 0000-0001-9953-6654

Embryonal tumor with multilayered rosettes (ETMR): case report and literature review

Authors:

Kadyrov Sh.U., Datsieva A.A., Terentyeva A.I., Grigorieva M.V., Galstyan S.A.

More about the authors

Journal: Burdenko's Journal of Neurosurgery. 2022;86(3): 63‑70

DOI: 10.17116/neiro20228603163

Read: 5100 times

To cite this article:

Kadyrov ShU, Datsieva AA, Terentyeva AI, Grigorieva MV, Galstyan SA. Embryonal tumor with multilayered rosettes (ETMR): case report and literature review. Burdenko's Journal of Neurosurgery. 2022;86(3):63‑70. (In Russ., In Engl.)
https://doi.org/10.17116/neiro20228603163

Подписка 2025 Журнал «Вопросы нейрохирургии» имени Н.Н. Бурденко (Burdenko's Journal of Neurosurgery)

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Abstract:

Embryonal tumor with multilayered rosettes (ETMR) is a rare and highly malignant brain tumor that develops in children younger 4 years old. This neoplasm is characterized by extremely aggressive course, low sensitivity to chemotherapy and radiotherapy. Thanks to the progress of pathologists, diagnosis of ETMR is now available using immunohistochemical examination with LIN28A and SALL4 antibodies. Moreover, detection of microRNA amplification in the 19q13.42 locus by fluorescent hybridization in situ allows an unmistakable diagnosis.

The authors describe clinical course and treatment outcome in a 2-year-old patient with a giant tumor of the right parietotemporal region. Postoperative histological examination verified ETMR. Despite adjuvant treatment, we observed fast progression of disease and unfavorable outcome after 5 months. Case report is supplemented by literature review.

CONCLUSION

ETMR is very rare and poorly understood neoplasm. The authors present a giant hemispheric ETMR in a 2-year-old boy with an extremely aggressive course of disease. Despite the advances in diagnosis and treatment of CNS tumors in children, there are currently more questions than answers regarding ETMR. Pooled analysis of all available data with large-scale studies is needed. It is necessary to emphasize an exceptional role of each clinical case for global study of this tumor. Timely adjuvant/neoadjuvant therapy in highly specialized centers is also essential.

Keywords:

embryonic tumor

ETMR

adjuvant/neoadjuvant therapy

Authors:

Kadyrov Sh.U.

Burdenko Neurosurgical Center

RSCI AuthorID: 669386
Scopus AuthorID: 23473552800
ORCID: 0000-0001-5879-1333

Datsieva A.A.

Burdenko National Medical Research Center of Neurosurgery

Terentyeva A.I.

Burdenko National Medical Research Center of Neurosurgery

Grigorieva M.V.

Burdenko National Medical Research Center of Neurosurgery

Galstyan S.A.

Burdenko Neurosurgical Center

ORCID: 0000-0001-9953-6654

Received:

23.04.2021

Accepted:

25.03.2022

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References:

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