Use of Neuroendoscopy in Combination Treatment of a Three-Year-Old Patient With Primary Disseminated Medulloblastoma: A Case Report.

A three-year-four-month-old boy with primary disseminated medulloblastoma M3 stage and secondary occlusive hydrocephalus underwent an endoscopic triventriculocisternostomy (ETVC) and tumor biopsy, followed by ventriculoperitoneal shunt (VPS) placement due to ETVC failure. The treatment regimen, which included intensive induction chemotherapy, proton beam therapy (PBT), and maintenance chemotherapy, led to significant clinical improvement and a complete radiological response. Four years post-treatment, the child remains in remission, illustrating the effectiveness of a multimodal approach in managing complex cases of medulloblastoma in pediatric patients.

[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].

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.

[Verification of the diagnosis of supratentorial ependymomas by real-time PCR].

Background: Differential diagnosis of supratentorial ependymomas is of particular difficulty in neurooncology due to nonspecific clinical and radiographic findings, a rare seen «classic» morphological picture, and a nonspecific immunophenotype. Thanks to molecular genetic methods, in particular real-time PCR, it has become possible to verify supratentorial ependymomas and identify their molecular group, on which further prognosis depends.

Objective: To develop a set of molecular genetic tests based on real-time PCR to verify supratentorial ependymomas.

Regional adaptation of the education in palliative and end-of-life Care Pediatrics (EPEC-Pediatrics) curriculum in Eurasia.

Background: Pediatric palliative care (PPC) is a priority to improve pediatric hematology oncology (PHO) care in Eurasia. However, there are limited regional opportunities for PPC education. We describe the adaptation and implementation of a bilingual end-user Education in Palliative and End-of-Life Care (EPEC)-Pediatrics course for PHO clinicians in Eurasia.

[Diffuse brainstem tumors in children. Tumor biology and hope for a better outcome. Current state of the problem].

Diffuse brainstem tumor is a fatal disease and the main cause of child mortality from neoplasms of central nervous system. So far, no effective therapy has been found for this disease. The authors discuss the modern aspects of clinical data, biology, diagnosis and treatment of patients with diffuse brainstem tumors.

Therapeutic implications of improved molecular diagnostics for rare CNS embryonal tumor entities: results of an international, retrospective study.

Background: Only few data are available on treatment-associated behavior of distinct rare CNS embryonal tumor entities previously treated as "CNS-primitive neuroectodermal tumors" (CNS-PNET). Respective data on specific entities, including CNS neuroblastoma, FOXR2 activated (CNS NB-FOXR2), and embryonal tumors with multilayered rosettes (ETMR) are needed for development of differentiated treatment strategies.

Methods: Within this retrospective, international study, tumor samples of clinically well-annotated patients with the original diagnosis of CNS-PNET were analyzed using DNA methylation arrays (n = 307).

Molecular analysis of pediatric CNS-PNET revealed nosologic heterogeneity and potent diagnostic markers for CNS neuroblastoma with FOXR2-activation.

Primitive neuroectodermal tumors of the central nervous system (CNS-PNETs) are highly malignant neoplasms posing diagnostic challenge due to a lack of defining molecular markers. CNS neuroblastoma with forkhead box R2 (FOXR2) activation (CNS_NBL) emerged as a distinct pediatric brain tumor entity from a pool previously diagnosed as primitive neuroectodermal tumors of the central nervous system (CNS-PNETs). Current standard of identifying CNS_NBL relies on molecular analysis.

Barriers to the early integration of palliative care in pediatric oncology in 11 Eurasian countries.

Background: The early integration of palliative care significantly improves quality of life for children with cancer. However, cultural, structural, and socioeconomic barriers can delay the integration of palliative care into cancer care, particularly in low-income and middle-income countries. To date, little is known regarding the timing of and barriers to palliative care integration in Eurasia.

A multicountry assessment in Eurasia: Alignment of physician perspectives on palliative care integration in pediatric oncology with World Health Organization guidelines.

Background: The World Health Organization (WHO) advocates for early integration of palliative care for all children with life-threatening illness. Provider awareness and misperceptions, however, can impede this imperative. In the Eurasian region, little is known about physician knowledge and perspectives on palliative care.

Transcriptional profiling of medulloblastoma with extensive nodularity (MBEN) reveals two clinically relevant tumor subsets with VSNL1 as potent prognostic marker.

Medulloblastoma with extensive nodularity (MBEN) is one of the few central nervous system (CNS) tumor entities occurring in infants which is traditionally associated with good to excellent prognosis. Some MBEN, however, have been reported with an unfavorable clinical course. We performed an integrated DNA/RNA-based molecular analysis of a multi-institutional MBEN cohort (n = 41) to identify molecular events which might be responsible for variability in patients' clinical outcomes.

[Central nervous system atypical teratoid/rhabdoid tumor without loss of nuclear expression of INI1].

The paper describes a clinical case of atypical teratoid/rhabdoid tumor with preserved INI1 expression and SMARCA4 gene mutations in an 8-month-old girl. Genome-wide DNA methylation, hierarchical clustering, and next-generation sequencing were used to make a tumor diagnosis. However, BRG1 immunohistochemical examination may be recommended in the routine practice of diagnosis and study of childhood CNS malignant tumors.

The use of a smartphone-assisted ventricle catheter guide for Ommaya reservoir placement-experience of a retrospective bi-center study.

Background: For intraventricular chemotherapy (IVC) as part of many oncological treatment protocols, Ommaya reservoir is enabling repeated access to the cerebro-spinal fluid (CSF). The correct placement of the catheter in the ventricle is essential for correct application of drugs, which is enabled by sophisticated techniques such as neuronavigation.

Objective: In a bi-center retrospective study, we reviewed our experience using a smartphone-assisted ventricle catheter guide as simple solution for correct Ommaya reservoir placement.

[Placement of a ventricular catheter into narrow lateral ventricles. Popular navigation].

Unlabelled: Placement of a ventricular catheter is the most common and easiest procedure in neurosurgery. Usually, the procedure is performed using anatomical landmarks. However, despite the apparent ease of this manipulation, its results are not always satisfactory.

H3-/IDH-wild type pediatric glioblastoma is comprised of molecularly and prognostically distinct subtypes with associated oncogenic drivers.

Pediatric glioblastoma (pedGBM) is an extremely aggressive pediatric brain tumor, accounting for ~6% of all central nervous system neoplasms in children. Approximately half of pedGBM harbor recurrent somatic mutations in histone 3 variants or, infrequently, IDH1/2. The remaining subset of pedGBM is highly heterogeneous, and displays a variety of genomic and epigenetic features.

Somatic mutations of DICER1 and KMT2D are frequent in intraocular medulloepitheliomas.

Intraocular medulloepithelioma (IO-MEPL) is an uncommon embryonal neuroepithelial neoplasm of the eye. Little is known about the cytogenetics, molecular biology, and pathogenesis of this tumor. In the present study we investigated the mutational landscape of 19 IO-MEPL using targeted next-generation sequencing.

MRI diagnosis of spinal metastasis of medulloblastoma. A clinical case and literature review.

Objective: the study objective was to improve the quality of detection of medulloblastoma metastases.

Material And Methods: Magnetic resonance imaging (MRI) of the spinal cord in a child with medulloblastoma of the posterior cranial fossa, which was performed on the first day after surgery, detected contrast-positive thickenings of the meninges in the cervical, thoracic, and lumbar spinal cord that might be erroneously diagnosed as metastasis. These lesions spontaneously regressed within 3 weeks, which was verified by control MRI.

Histologically distinct neuroepithelial tumors with histone 3 G34 mutation are molecularly similar and comprise a single nosologic entity.

In contrast to the relative morphological uniformity of histone H3 K27-mutant high-grade gliomas, H3 G34-mutant tumors present as a histopathologically heterogeneous group of neoplasms, with microscopic characteristics typical of either glioblastoma (GBM) or central nervous system primitive neuroectodermal tumors (CNS-PNET). In the current study, we performed an integrative clinical, histopathological and molecular analysis of 81 G34-mutant CNS tumors. Routinely prepared tumor tissues were investigated for genomic and epigenomic alterations.

[The main principles of concept of development of palliative care to children and adolescents].

The article substantiates actuality of elaboration of the concept of development of palliative care of children and adolescents. The analysis of legislative and normative legal base, international documents, scientific studies related to this issue are analyzed. The situation in the regions ofthe Russian Federation is examined concerning organization of palliative care of children and its forms, provision of pharmaceuticals and specialized equipment and professional training of specialists.

Embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma, and medulloepithelioma share molecular similarity and comprise a single clinicopathological entity.

Three histological variants are known within the family of embryonal rosette-forming neuroepithelial brain tumors. These include embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma (EBL), and medulloepithelioma (MEPL). In this study, we performed a comprehensive clinical, pathological, and molecular analysis of 97 cases of these rare brain neoplasms, including genome-wide DNA methylation and copy number profiling of 41 tumors.

Recurrence patterns across medulloblastoma subgroups: an integrated clinical and molecular analysis.

Background: Recurrent medulloblastoma is a therapeutic challenge because it is almost always fatal. Studies have confirmed that medulloblastoma consists of at least four distinct subgroups. We sought to delineate subgroup-specific differences in medulloblastoma recurrence patterns.

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.