The rare case of a combination two brain tumors with total colon polyposis

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Abstract

Objectives of publication: Presentation of a rare clinical observation from our own practice. Key points: The combination of brain tumors and colon polyposis often described in the literature as a syndrome Turcot. Turcot's syndrome is a genetic disease with different clinical manifestations. This is a rare syndrome (a little more than 150 cases are described), and therefore, the standardization of approaches to diagnosis and treatment is not currently defined. We present the clinical case of manifestations of the Turcot’s syndrome of our practice. Сlinical observation: Male patients, from birth to the skin marked multiple cafe-au-lait spots. At age 14 years, according to a magnetic resonance imaging multiple brain tumors are revealed, two different histologically tumor brains (one of which is benign (pilocytic astrocytoma), the other is malignant (anaplastic ependymoma)) and total colon adenomatous polyposis were diagnosed. Currently, syndromic pathology is not verified. Receives one of polychemotherapy protocols for treating tumors of the central nervous system. It is planned to conduct additional studies aimed at clarifying the genetic defect, with a view to a possible optimization of the therapy (modification of the plane of polychemotherapy, use of an option of radiation therapy, use of a target therapy). Conclusions: The results of a comprehensive survey of the use of modern techniques of molecular biology and cytogenetics affect the prognosis of the disease and treatment strategy.

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Ekaterina Aleksandrovna Salnikova

Federal Research Center of Pediatric Hematology, Oncology, and Immunology named after Dmitry Rogachev, Ministry of Health of the Russian Federation

Email: ea_gerasimova@mail.ru
Junior researcher, Dep. of Neurooncology

Sergey Sergeevich Ozerov

Federal Research Center of Pediatric Hematology, Oncology, and Immunology named after Dmitry Rogachev, Ministry of Health of the Russian Federation

Email: Sergey.Ozerov@fccho-moscow.ru
MD, PhD, Project Leader, Dep. of Neurooncology

Ilya Vladimirovich Zakharov

Federal Research Center of Pediatric Hematology, Oncology, and Immunology named after Dmitry Rogachev, Ministry of Health of the Russian Federation

Email: ilya.zaharov@fccho-moscow.ru
Department of surgery of children and teenagers

Dmitry Sergeevich Abramov

Federal Research Center of Pediatric Hematology, Oncology, and Immunology named after Dmitry Rogachev, Ministry of Health of the Russian Federation

Email: pathmorf@mail.ru
Department of pathological anatomy

Anna Nikolaevna Kazakova

Federal Research Center of Pediatric Hematology, Oncology, and Immunology named after Dmitry Rogachev, Ministry of Health of the Russian Federation

Email: anna.kazakova@fccho-moscow.ru
Department of surgery of children and teenagers.

Marina Valeryevna Tikhonova

Federal Research Center of Pediatric Hematology, Oncology, and Immunology named after Dmitry Rogachev, Ministry of Health of the Russian Federation

Email: marina.tikhonova@fccho-moscow.ru
Department of hematology/oncology for teenagers and young adults

Natalya Ivanovna Pospekhova

State research center of coloproctology named after A. N. Ryzhikh, Ministry of Health of the Russian

Email: npospekhova@mail.ru
PhD Department of laboratory genetics

Galina Anatolyevna Novichkova

Federal Research Center of Pediatric Hematology, Oncology, and Immunology named after Dmitry Rogachev, Ministry of Health of the Russian Federation

Email: Galina.Novichkova@fnkc.ru
MD, PhD, Dr Med Sci, Professor, director, Department of the leading consultants

Dmitry Vitalyevich Litvinov

Federal Research Center of Pediatric Hematology, Oncology, and Immunology named after Dmitry Rogachev, Ministry of Health of the Russian Federation

Email: litvinov_d_v@mail.ru
MD, PhD, Department of hematology/oncology for teenagers and young adults

Irina Dmitrievna Borodina

Federal Research Center of Pediatric Hematology, Oncology, and Immunology named after Dmitry Rogachev, Ministry of Health of the Russian Federation

Email: irina.borodina@fccho-moscow.ru
MD, PhD, Ambulatory department

Ella Vyacheslavovna Kumirova

Federal Research Center of Pediatric Hematology, Oncology, and Immunology named after Dmitry Rogachev, Ministry of Health of the Russian Federation

Email: ella.kumirova@fccho-moscow.ru
MD, PhD, Dr Med Sci, Department of Neurooncology

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Copyright (c) 2015 Salnikova E.A., Ozerov S.S., Zakharov I.V., Abramov D.S., Kazakova A.N., Tikhonova M.V., Pospekhova N.I., Novichkova G.A., Litvinov D.V., Borodina I.D., Kumirova E.V.

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