A5079210262- Chromatin Remodeling and Cancer
- Renal and related cancers
- Sarcoma Diagnosis and Treatment
- Neuroblastoma Research and Treatments
- Cancer Mechanisms and Therapy
- Tumors and Oncological Cases
- Congenital Diaphragmatic Hernia Studies
- Renal cell carcinoma treatment
- Neuroendocrine Tumor Research Advances
- Cancer, Hypoxia, and Metabolism
- Genetic and Kidney Cyst Diseases
- Congenital Anomalies and Fetal Surgery
- Mechanisms of cancer metastasis
- Cancer Diagnosis and Treatment
- Neonatal Respiratory Health Research
- Testicular diseases and treatments
- Ovarian cancer diagnosis and treatment
- Childhood Cancer Survivors' Quality of Life
- Cancer therapeutics and mechanisms
- Peptidase Inhibition and Analysis
- Organ Donation and Transplantation
- Vascular anomalies and interventions
- Neurofibromatosis and Schwannoma Cases
- Hepatocellular Carcinoma Treatment and Prognosis
- Cardiac tumors and thrombi
Dmitry Rogachev National Research Center of Pediatric Hematology, Oncology and Immunology
2014-2024
Ministry of Health of the Russian Federation
2015-2024
Introduction: Malignant rhabdoid tumors (MRT) predominantly affect infants and young children. Patients below six months of age represent a particularly therapeutically challenging group. Toxicity to developing organ sites limits intensity treatment. Information on prognostic factors, genetics, toxicity treatment long-term outcomes is sparse. Methods: Clinical, genetic, data 100 patients (aged 6 at diagnosis) from 13 European countries were analyzed (2005-2020). Tumors matching blood samples...
Treatment of patients with high-risk neuroblastoma (NB) is a complex challenge, and it based on response to certain elements therapy. The development introduction new treatment approaches, such as GD2-targeted immunotherapy (IT), leads improved survival in this cohort patients. aim the study was retrospectively assess effectiveness therapy NB before IT into clinical practice. We analyzed data 151 stratified group who had received accordance modified NB2004 protocol German Society for...
The malignant rhabdoid tumor (RT) is one of the most aggressive childhood neoplasm. RTs are characterized by presence inactivating mutations in SMARCB1 (hSNF5/INI1/BAF47) gene – a suppressor localized 22q11.2. Up to 30 % caused germline this gene, date those cases considered as manifestation predisposition syndrome type 1 (RTPS1). We have analyzed polymerase chain reaction and subsequent Sanger sequencing 18 patients with RT different localizations for improving genetic laboratory...
The current study analyses the frequency of positive family history and cancer predisposition syndromes in large cohort patients with sympathetic nervous system tumors treated Federal Scientific Clinical Center Pediatric Hematology, Oncology Immunology named after Dmitry Rogachev. Positive genetic were observed 0.5% 1.5% cases neuroblastoma, consequently. article contains review literature devoted to mechanisms neuroblastoma including genetics sporadic cases.
In the recent years, full exome sequencing helped to reveal a set of mutations in genes that are not oncogenes or tumor suppressor by definition, but play an important role carcinogenesis and encode proteins involved chromatin remodeling. Among remodeling systems, which operate through ATP-dependent mechanism, complex SWI/ SNF attracts great attention. The consists catalytic ATPase (SMARCA2/4), group conservative core subunits (SMARCB1, SMARCC1/2), variant subunits. Abnormalities coding for...
Metanephric tumors (MTs) are a group of rare childhood kidney consisting epithelial and/or stromal cellular elements and characterized by variety histopathological features. MTs include metanephric adenoma (MA), adenofibroma (MAF), tumor (MST). This study aimed to retrospectively analyse clinical molecular genetic characteristics MTs, verified at the Pathology Department Dmitry Rogachev NMRCPHOI Ministry Healthcare Russian Federation. The was approved Independent Ethics Committee Scientific...
Congenital mesoblastic nephroma (CMN) is a rare renal tumor of young children with intermediate biological behavior, accounting for 3.5-4% all tumors in children. СMN characterized by favorable prognosis case radical surgical treatment. Relapses CMN are considered to be quite occurrence (4% cases), however, both local and metastatic relapses possible. There no fully standardized treatment approaches patients relapsed CMN. In our study, we performed retrospective analysis (n = 3) verified...
Somatic translocations involving the NTRK genes occur in 0.34–2.2% of all malignant neoplasms children. TRK inhibitors whose efficacy has been demonstrated prospective clinical studies expand treatment options for patients with solid tumors harboring gene rearrangements. The aim our study was to summarize first Russian experience use inhibitor entrectinib extracranial fusion-positive included compassionate program. This approved by Independent Ethics Committee and Academic Council Dmitry...
Three years ago, our patient, at that time a 16-month-old boy, was discovered to have bilateral kidney lesions with giant tumor in the right kidney. Chemotherapy and nephron-sparing surgery (NSS) for Wilms nephroblastomatosis carried out. The patient also had eye affection, including glaucoma, enlargement, megalocornea, severe corneal swelling opacity, complete aniridia, nystagmus. diagnosis of WAGR syndrome suspected. De novo complex chromosomal rearrangement balanced translocation...
An urgent issue of pediatric oncology is the search for new methods therapy patients with malignant rhabdoid tumors (MRT), a type rare neoplasms childhood. Insufficient knowledge, difficulties in diagnosis lead to fact that confirmed most common stages process and cases fatal. The review literature presents modern ideas about origin MRT, approaches patients.
Abstract BACKGROUND: Individuals with rhabdoid tumor predisposition syndrome (RTPS1 – SMARCB1, RTPS2 SMARCA4) have a propensity to develop malignant tumors (MRT). Affected patients typically present < age 12 months synchronous (SYN) exhibiting an unusually aggressive clinical behavior. Due the rarity of RTPS, standards for management are evolving. METHODS: Clinical, genetic, and treatment data 90 RTPS from 16 countries were analyzed (2004 2020). Therapy followed EU-RHAB...
Radical tumor removal remains the key task in hepatoblastoma surgery. Patients who need extended resection and have a risk of posthepatectomy liver failure are particularly difficult. In cases with insufficient future volume, method choice is two-staged hepatectomy or transplantation. Despite wide spread laparoscopic surgery adults, minimally invasive approach has not been studied pediatrics, literature on this issue scarce. current publication case successful partial associating partition...
Children with malignancies have a high risk of developing inanition for variety reasons associated both tumor and the toxicity performed therapy. Enteral feeding via percutaneous endoscopic gastrostomy (PEG) is one procedures to correction nutritional status. The authors show feasibility safety PEG in children solid malignancies, as well possibility using patients receiving cycles intensive chemoradiation paper analyzes case histories undergoing correct status, indications its application,...
Clear cell sarcoma of the kidney (CCSK) is a rare malignant renal tumor in children, which accounts for 2–5% pediatric malignancies. The aim study was to analyze results therapy patients with CCSK treated Dmitry Rogachev National Medical Research Center оf Pediatric Hematology, Oncology and Immunology. Retrospective analysis histologically confirmed diagnosis period 01.2012–02.2020 (98 months) done. approved by Independent Ethics Committee Scientific Council D. NMRCPHOI Ministry Healthcare...
Malignant rhabdoid tumor (MRT) is a rare malignant neoplasm of childhood, characterized by an aggressive course and extremely unfavorable prognosis. The frequency MRT outside the central nervous system (extracranial MRT) 0.02–0.03 per 100,000 children. In most cases, based on inactivating mutations suppressor gene SMARCB1, which leads to absence expression SMARCB1 ((INI1/hSNF5/BAF47) protein in cells. Aberrations SMARCA4 gene, molecular event, have been described among MRTs expressing...
<h3></h3> Опухолевый тромбоз нижней полой вены и правого предсердия считают редким осложнением в детской солидной онкологии. Лечение таких пациентов сопряжено с высокими рисками осложнений неблагоприятного исхода, что требует мультидисциплинарного подхода использования высокотехнологичного оборудования на всех этапах комбинированного или комплексного лечения. На сегодняшний день не решен ряд вопросов, касающихся деталей оперативного вмешательства, подготовки к хирургическому лечению...
Neuroblastoma (NB) is the most common extracranial solid embryonic tumor in children. The age of patient, prevalence process and molecular genetic profile formed basis risk-adapted therapy protocols. High-risk patients receive complex treatment, including various combinations chemotherapy drugs, surgical removal tumor, radiation radioisotope therapy. Since end 90s last century, immunotherapy has been introduced into clinical practice as a post-consolidation phase with aim affecting minimal...