[Supratentorial neuroepithelial tumor with PLAGL1 gene fusion - a new type of morphologically variable pediatric brain neoplasm defined by a distinct DNA methylation class. A case report and literature review]. / Supratentorial'naya neiroepitelial'naya opukhol' so sliyaniem gena PLAGL1 u detei ­ novyi tip opukholi mozga s raznoobraznoi morfologicheskoi kartinoi i otlichitel'nym metilyatsionnym profilem(sluchai iz praktiki i obzor literatury).

BACKGROUND: Methylation analysis has become a powerful diagnostic tool in modern neurooncology. This technique is valuable to diagnose new brain tumor types. OBJECTIVE: To describe the MRI and histological pattern of neuroepithelial tumor with PLAGL1 gene fusion. MATERIAL AND METHODS: We present a 6-year-old patient with small right frontal intraaxial tumor causing drug resistant epilepsy. Despite indolent preoperative clinical course and MRI features suggesting glioneuronal tumor, histological evaluation revealed characteristics of high-grade glioma, ependymoma and neuroblastoma. RESULTS: Methylation analysis of tumor DNA confirmed a new type of a recently discovered neoplasm - neuroepithelial tumor with PLAGL1 fusion (NET PLAGL1). PCR confirmed fusion of PLAGL1 and EWSR1 genes. No seizures were observed throughout the follow-up period. There was no tumor relapse a year after surgery. CONCLUSION: Methylation analysis in neurooncology is essential for unclear tumor morphology or divergence between histological and clinical data. In our case, this technique confirmed benign nature of tumor, and we preferred follow-up without unnecessary adjuvant treatment.

[Long-term epilepsy-associated tumors]. / Glioneironal'nye opukholi, assotsiirovannye s epilepsiei.

OBJECTIVE: The goal of this review is to summarize data concerning radiological and histological diagnosis of epileptogenic tumors, as well as to discuss their surgical treatment. MATERIAL AND METHODS: PubMed literature database was searched for relevant articles, we also used our own clinical experience. RESULTS: Benign glioneuronal tumors are the second most common cause of drug resistant epilepsy in adults after hippocampal sclerosis. Exceptionally slow growth (more than 2 years), localization in the brain cortex, presence of differentiated neuronal tissue in tumoral body and long history of epilepsy are the key features of these tumors called LEAT (long-term epilepsy-associated tumors). Management of LEAT requires epileptological as well as neurooncological approach. The epileptogenic zone commonly spreads beyond the tumor borders and simple lesionectomy alone may not be sufficient for achieving seizure freedom. CONCLUSION: LEAT typically exhibit low proliferative activity, however they should be thoroughly differentiated from more aggressive glial tumors; while this task is sometimes quite challenging, it's achievable by means of histological and immunohistochemical examination.

[Application of intraoperative electromagnetic frameless navigation in transcranial and endoscopic neurosurgical interventions]. / Primenenie bezramnoi sistemy intraoperatsionnoi élektromagnitnoi navigatsii pri transkranial'nykh i éndoskopicheskikh neirokhirurgicheskikh vmeshatel'stvakh.

The paper summarizes the experience in using a system of electromagnetic intraoperative frameless navigation in various neurosurgical pathologies of the brain. The electromagnetic navigation technique was used for 102 operations in 98 patients, including 36 transnasal endoscopic interventions. There were no intraoprtative and postoperative complications associated with the use of the system. In the process of using the system, factors influencing the accuracy of navigation and requiring additional control by the surgeon were identified. PURPOSE: The study purpose was to evaluate the use of electromagnetic navigation in surgical treatment of patients with various brain lesions. MATERIAL AND METHODS: The system of electromagnetic navigation was used for 102 operations in 98 patients (42 males and 56 females, including 18 children; median age, 34.8 years (min, 2.2 years; max, 69 years)) in the period from December 2012 to December 2016. In 36 patients, the system was used for endoscopic interventions. In 19 patients, electromagnetic navigation was used in combination with neurophysiological monitoring. RESULTS: In our series of cases, the frameless electromagnetic navigation system was used in 66 transcranial operations. The mean error of navigation was 1.9±0.5 mm. In 5 cases, we used the data of preoperative functional MRI (fMRI) and tractography for navigation. At the same time, in all 7 operations with simultaneous direct stimulation of the cortex, there was interference and significant high-frequency noise, which distorted the electrophysiological data. A navigation error of more than 3 mm was associated with the use of neuroimaging data with an increment of more than 3 mm, image artifacts from the head locks, high rate of patient registration, inconsequence of touching points on the patient's head, and unsatisfactory fixation to the skin or subsequent displacement of a non-invasive localizer of the patient. In none of the cases, there was a significant effect of standard metal surgical tools (clamps, tweezers, aspirators) located near the patient's head on the navigation system. In two cases, the use of massive retractors located near the patient's localizer caused noise in the localizer and navigation errors of more than 10 mm due to significant distortions of the electromagnetic field. Thirty-six transnasal endoscopic interventions were performed using the electromagnetic frameless navigation system. The mean navigation error was 2.5±0.8 mm. CONCLUSION: In general, electromagnetic navigation is an accurate, safe, and effective technique that can be used in surgical treatment of patients with various brain lesions. The mean navigation error in our series of cases was 1.9±0.5 mm for transcranial surgery and 2.5±0.8 mm for endoscopic surgery. Electromagnetic navigation can be used for different, both transcranial and endoscopic, neurosurgical interventions. Electromagnetic navigation is most convenient for interventions that do not require fixation of the patient's head, in particular for CSF shunting procedures, drainage of various space-occupying lesions (cysts, hematomas, and abscesses), and optimization of the size and selection of options for craniotomy. In repeated interventions, disruption of the normal anatomical relationships and landmarks necessitates application of neuronavigation systems in almost mandatory manner. The use of electromagnetic navigation does not limit application of the entire range of necessary intraoperative neurophysiological examinations at appropriate surgical stages. Succession in application of neuronavigation should be used to get adequate test results.

[Surgical anatomy of the peri-insular association tracts. Part I.The superior longitudinal fascicle system]. / Khirurgicheskaya anatomiya periinsulyarnykh assotsiativnykh provodyashchikh putei. Chast' I. Sistema verkhnego prodol'nogo puchka.

AIM: To study the peri-insular association tract anatomy and define the permissible anatomical boundaries for resection of glial insular tumors with allowance for the surgical anatomy of the peri-insular association tracts. MATERIAL AND METHODS: In an anatomic study of the superior longitudinal fascicle system (SLF I, SLF II, SLF III, arcuate fascicle), we used 12 anatomical specimens (6 left and 6 right hemispheres) prepared according to the Klingler's fiber dissection technique. To confirm the dissection data, we used MR tractography (HARDI-CSD-tractography) of the conduction tracts, which was performed in two healthy volunteers. RESULTS: Except the SLF I (identified in 7 hemispheres by fiber dissection), all fascicles of the SLF system were found in all investigated hemispheres by both fiber dissection and MR tractography. The transcortical approach to the insula through the frontal and (or) parietal operculum is associated with a significant risk of transverse transection of the SLF III fibers passing in the frontal and parietal opercula. The most optimal area for the transcortical approach to the insula is the anterior third of the superior temporal gyrus that lacks important association tracts and, consequently, a risk of their injury. The superior peri-insular sulcus is an intraoperative landmark for the transsylvian approach, which enables identification of the SLF II and arcuate fascicle in the surgical wound. CONCLUSION: Detailed knowledge of the peri-insular association tract anatomy is the prerequisite for neurosurgery in the insular region. Our findings facilitate correct identification of both the site for cerebral operculum dissection upon the transcortical approach and the intraoperative landmarks for locating the association tracts in the surgical wound upon the transsylvian approach to the insula.

[Hippocampal sclerosis: pathogenesis, clinical features, diagnosis, and treatment]. / Skleroz gippokampa: patogenez, klinika, diagnostika, lechenie.

Hippocampal sclerosis, also known as Ammon horn sclerosis or mesial temporal sclerosis, is usually associated with intractable epilepsy and characterized by specific patterns of neuronal loss and gliosis in the medial temporal lobe structures. Hippocampal sclerosis manifests clinically as epilepsy, often intractable epilepsy; in most cases, this condition is surgically treatable. As the most common histological diagnosis in adult patients subjected to epilepsy surgery, hippocampal sclerosis is characterized by complex pathogenesis and requires a multidisciplinary approach to its diagnosis and treatment. This article reviews the pathologic features, natural history, pathogenesis, and electroclinical and MRI signs of hippocampal sclerosis.

[3D-TOF MR-angiography with high spatial resolution for surgical planning in insular lobe gliomas].

UNLABELLED: Despite the obvious progress in modern neurosurgery, surgery for glial tumors of the insular lobe is often associated with a high risk of postoperative neurological deficit, which is primarily caused by damage to perforating arteries of the M1 segment of the middle cerebral artery. OBJECTIVE: The work is aimed at evaluating the effectiveness of high resolution time-of-flight (3D-TOF) MR angiography in imaging of medial and lateral lenticulostriate arteries and determining their relationship to tumor edge in patients with gliomas of the insula. MATERIAL AND METHODS: 3D-TOF MR angiography data were analyzed in 20 patients with primarily diagnosed cerebral gliomas involving the insula. All patients underwent non-contrast enhanced 3D-TOF MR angiography. In 6 cases, 3D-TOF MRA was performed before and after contrast enhancement. RESULTS: 3D-TOF angiography before intravenous contrast injection was capable of visualizing the medial lenticulostriate arteries in 19 patients (95% of all cases) and lateral lenticulostriate arteries in 18 patients (90% of all cases). Contrast-enhanced 3D-TOF angiography allows for better visualization of both the proximal and distal segments of lenticulostriate arteries. Three variants of relationship between the tumor and lenticulostriate arteries were identified. Variant I: the tumor grew over the arteries without their displacement in 2 cases (10% of the total number of observations); variant II: the tumor caused medial displacement of arteries without growing over them in 11 cases (55% of the total number of observations); variant III: the tumor partially grew over and displaced arteries in 2 cases (10%). In 25% of cases (5 patients), tumor was poorly visualized on 3D-TOF MR angiograms because their signal characteristics did not differ from those of the medulla (tumor tissue was T1 isointense). As a result, it was impossible to determine the relationship between the tumor and lenticulostriate arteries. CONCLUSION: High spatial resolution time-of-flight MR angiography can be recommended for preoperative imaging of lenticulostriate arteries to plan the extent of neurosurgical resection in patients with glial tumors of the insular lobe.

[Supracerebellar transtentorial approach to tumors of the posterior portions of the medial temporal region].

OBJECTIVE: Despite the advances in microsurgery, the choice of the most adequate approach to the posterior part of the medial temporal region (MTR) remains a very controversial issue. The supracerebellar transtentorial approach (STA) is considered as the most preferable one, since it provides the optimal balance between retraction, incision, and resection of the brain tissue. Here, we present our consecutive series of 20 patients who underwent STA surgery. MATERIAL AND METHODS: Twenty patients with glial tumors affecting the posterior MTR underwent STA surgery between 2006 and 2014. The mean age of the patients was 20 years. Benign tumors were predominant (18 out of 20 cases). RESULTS: Resection of the posterior and middle MTRs was conducted in 16 cases. The anterior MTR was accessed through STA in 1 patient only; in 2 patients, STA was combined with the infraoccipital approach. Cerebellar edema occurred in 4 patients, with hemiparesis persisting in one of the cases for 1 year after surgery. Of 8 patients with drug resistant epilepsy, the Engel class 1 or 2 outcome was achieved in 6 cases within 1 year after surgery. CONCLUSION: STA provides an excellent surgical route to the posterior and middle MTR portions; however, the anterior MTR portions cannot be reached safely. The operative risks of STA increase as the surgeon proceeds with resection of the anterior MTR portions. Anterior MTR structures can be removed using a combination of the supracerebellar and infraoccipital transtentorial approaches or two-stage resection.