[Basal ganglia germinomas in children. Four clinical cases and a literature review].

UNLABELLED: Basal ganglia germinomas are a specific group of intracranial germinomas. Their early diagnosis is complicated due to their atypical localization and diversity of neuroimaging and clinical signs. MATERIAL AND METHODS: We describe 4 cases of basal ganglia germinoma in boys of 13, 14, 15, and 16 years of age. The medical history data, clinical features, neuroimaging and histological characteristics of basal ganglia germonomas, and preliminary results of the treatment are presented. CONCLUSION: Basal ganglia germinomas are usually verified at the late stage of the disease when patients are detected with extended lesions of the basal ganglia and severe neurological and neuroendocrine deficits. This situation is due to clinical and imaging signs that are untypical of common germinomas.

[Biological features and long-term results of comprehensive treatment of brain tumors in infants].

UNLABELLED: Complete removal is the treatment standard for most brain tumors. Outcomes of subsequent comprehensive treatment depend on biological features, the histological structure of the tumor, and radicality of surgery. The aim of this work was to study the morphological features of brain tumors in infants and to analyze long-term outcomes of surgical and comprehensive treatment. MATERIAL AND METHODS: The study included 80 infants with brain tumors aged from 1 to 12 months who had been operated on at the Burdenko Neurosurgical Institute during the period from 2000 to 2010. RESULTS: Maximal radicality was achieved in the group of tumors of the lateral and third ventricles (85%), in the group of tumors of hemispheric localization (82%), and in the group of tumors of the posterior fossa (83%). The lowest percentage of radical tumor removal (15%) accrued to tumors of the chiasmosellar area, most of which were large visual pathway gliomas. The overall five-year survival rate associated with the treatment in the studied series of patients was 92 and 48% for Grade I-II and Grade III-IV tumors, respectively. CONCLUSION: The features of biology of brain tumors in infants include the increased proliferative activity (high Ki-67 index of 10% and higher) revealed at the diagnosis of choroid papillomas, a series of pilocytic astrocytomas of the chiasm and Grade III astrocytomas, which do not affect the clinical course. The best long-term results of the treatment were obtained in infants with complete resection of Grade I and III astrocytomas and in infants with choroid papillomas. Radical removal of such histological forms as anaplastic astrocytoma, choroidal carcinoma, and anaplastic ependymoma improves the prognosis, provides favorable conditions for adjuvant therapy, and increases the period of progression-free survival in infants.

[Characteristics of medulloblastoma in children under age of three years].

We present a series of 51 medulloblastoma in children under three years, collected in N.N. Burdenko Neurosurgical Institute from 2000 to 2010. 57% of the tumors showed desmoplastic/nodular histology. Performed fluorescence in situ hybridization (FISH) analysis revealed the MYC oncogene amplification in 4%, the MYCN oncogene amplification - in 8%, isochromosome 17q - in 16% of cases. 9q deletion was found in 8% of desmoplastic/ nodular medulloblastomas. Our results showed that desmoplastic/nodular medulloblastoma has a positive predictive value for progression-free survival. Another feature of a biology of medulloblastomas in children younger than three years is the lack of nuclear accumulation of beta-catenin, and 6q deletion. Medulloblastomas with MYCN oncogene amplification often exhibit desmoplastic/nodular histology and a relatively favorable outcome. The most unfavorable prognostic marker is the MYC oncogene amplification, which in our series of 100% combined with the large cell/anaplastic medulloblastoma and isochromosome 17q - such tumors should be included in the "high risk" protocol.

[Glioneuronal tumor with neuropil-like islands in a neonate]. / Glioneironal'naia opukhol' s uchastkami neiropilia u rebenka grudnogo vozrasta.

Glioneuronal tumor with neuropil-like islands - GTNI is a rare histopatological subtype of anaplastic astrocytoma. English-language literature contains only 43 observations, among them only 4 observations in children up to 18 years. Neuropil is an eosinophilic stained a cellular foci of tumor tissue. These cells demonstrate less proliferation activity than prevailing glial component, which is characterized by a high degree of atypia - Gr III according to WHO classification. Single reports about verification of the glioneuronal tumor with neuropil-like islands in children are supplemented with an observation of the case with tumor, located in lateral ventricular in infant with the onset of hypertension symptoms at the age of 11 months. The potential for complete removal of these tumors despite its large size and young age of the child is presented. However, the prognosis in infants with GTNI despite completeness of removal and chemotherapy might be extremely unfavorable - in case of intraventricular localization progression in the form of tumor implantation on ventricular ependyma is possible.

[Diffuse bithalamic astrocytomas in children and adults].

Diffuse bithalamic glioma is rare entity. A few cases have been described so far. We present the data of 13 patients (both children and adults) with diffuse bithalamic gliomas observed and treated at Burdenko Neurosurgery Institute within the period of 2004-2011. Age of patients varied from 3 to 29 y.o. Bithalamic gliomas appear as symmetric enlargement of both thalami on MRI with frequent spreading to adjacent structures (brainstem, temporal lobes). Histologically they appear as astrocytomas (Grade II-IV). Bithalamic gliomas posess different clinical course. There is no indication for surgical treatment because of diffuse growth of these tumors. Stereotactic biopsy is indicated to verify histological origin. Treatment of bithalamic gliomas confined with radio- and chemotherapy. In cases with obstructive hydrocephalus CSF diverting operations are indicated. Attempts of surgical excision lead to worsening of patient's neurological condition. Most patients with bithalamic gliomas have unfavorable prognosis. Tumors remain stable with adjuvant therapy for a very short period of time in most cases. Benign course of this pathology with long-term follow-up is observed only in a few cases.

Localization of the motor and speech zones of the cerebral cortex by functional magnetic resonance tomography.

Functional magnetic resonance tomography provides a non-invasive method for mapping the cerebral cortex. The aim of the present work was to assess the potential and suitability of this method in a series of brain disorders. Studies were performed on 32 volunteers (mean age 37.8 +/- 20.9 years) and 16 patients with tumors of the cerebral cortex (mean age 36.2 +/- 24.2 years). Initial functional images were processed by statistical methods. Computed activation maps were superimposed on anatomical images. In 89% of cases, functional magnetic resonance tomography allowed the motor cortex and Broca's area to be localized; in almost 69%, the method impinged on the therapeutic tactics used in patients with cerebral cortex lesions. Thus, functional magnetic resonance tomography can be used in clinical conditions to obtain information not yielded by other diagnostic methods and which can be used to plan the neurosurgical treatment of patients with supratentorial brain tumors with maximum preservation of cerebral cortex function. Assessments of the state of the motor and speech areas by this tomographic method has potential applications in neurosurgery and neurophysiology.

[Localization of motor and speech cortex in the brain with functional magnetic resonance tomography]. / Lokalizatsiia motornoi i rechevoi zon kory golovnogo mozga s pomoshch'iu funktsional'noi magnitno-rezonansnoi tomografii.

Functional magnetic resonance imaging (MRI) can be used for non-invasive mapping of cerebral cortex. The purpose of the study was evaluation of applicability of functional MRI for studies of neurosurgical patients. 32 volunteers (mean age 37.8 +/- 20.9 years) and 16 patients with brain tumors (mean age 36.2 +/- 24.2 years) were included in the study. Statistical analysis of the data obtained was performed. Activation maps were superimposed on anatomical images and discussed with neurosurgeons. Functional MRI studies were successful in localising the motor cortex and Broca's area in 89% of cases. In 69% of cases, results of the functional MRI influenced the patients' treatment.