Primary multiple tumors of the nervous system of different histological types in the structure of hereditary tumor syndromes
https://doi.org/10.56618/2071-2693_2024_16_2_150
EDN: JSXZVH
Abstract
It is known that most cancers arise as a result of genetic mutations leading either to loss of function of tumor suppressor genes or to activation of oncogenes against the background of a decrease in the body’s antitumor immune surveillance.
A number of patients with primary multiple cerebral tumors of various histological types may have one or another hereditary tumor syndrome.
This article discusses 27 hereditary diseases, one of the phenotypic manifestations of which are tumors of the nervous system, such as: neurofibromatosis 1 (Recklinghausen disease) and types 2; schwannomatosis; tuberous sclerosis; retinoblastoma; syndromes: Noonan, LEOPARD, Costello, Legius, Turcot 1 and 2 types 1, Li – Fraumeni, DICER, von Hippil – Lindau, Cowden, Gorlin, rhabdoid tumor predisposition, familial paraganglioma, melanoma-astrocytoma, BAP1, ELP1 tumor predisposition – medulloblastomas, Louis – Bar, Nijmegen, Wiskott – Aldrich, Bloom, Rubinstein – Taybi, as well as Fanconi anemia and Carney complex.
Supporting agencies: The work was carried out as part of the state assignment No. 123021000128-4 “Development of a new technology for treating patients with secondary brain tumors and recurrent meningiomas”.
About the Authors
N. E. Voinov
Polenov Neurosurgery Institute – the branch of Almazov National Medical Research Centre
Russian Federation
Russian Federation
Nikita E. Voinov – Neurosurgeon
12 Mayakovskogo street, St. Petersburg, 191025
A. Yu. Ulitin
Polenov Neurosurgery Institute – the branch of Almazov National Medical Research Centre
Russian Federation
Russian Federation
Alexey Yu. Ulitin – Dr. of Sci. (Med.), Full Professor, Honored Doctor of Russian Federation, Neurosurgeon of the Highest Qualification Category, Head at the Department of Neurosurgery No. 4
12 Mayakovskogo street, St. Petersburg, 191025
A. P. Gerasimov
Polenov Neurosurgery Institute – the branch of Almazov National Medical Research Centre
Russian Federation
Russian Federation
Alexander P. Gerasimov – Neurologist, Geneticist, Senior Researcher at the Research Laboratory of Pediatric Neurosurgery
12 Mayakovskogo street, St. Petersburg, 191025
K. K. Kukanov
Polenov Neurosurgery Institute – the branch of Almazov National Medical Research Centre
Russian Federation
Russian Federation
Konstantin K. Kukanov – Cand. of Sci. (Med.), Neurosurgeon of the Highest Qualification Category at the Neurosurgical Department No. 4, Senior Researcher at the Institute of Neuro-Oncology
12 Mayakovskogo street, St. Petersburg, 191025
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Voinov N.E., Ulitin A.Yu., Gerasimov A.P., Kukanov K.K. Primary multiple tumors of the nervous system of different histological types in the structure of hereditary tumor syndromes. Russian Neurosurgical Journal named after Professor A. L. Polenov. 2024;16(2):150-164. (In Russ.) https://doi.org/10.56618/2071-2693_2024_16_2_150. EDN: JSXZVH
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