Clinical behavior of advanced giant cell tumor: 15 years observation study in N.N. Blokhin National Medical Research Center of Oncology.

2021 ◽  
Vol 39 (15_suppl) ◽  
pp. e23501-e23501
Author(s):  
Anastasia Alekseevna Tararykova ◽  
Alexander A. Fedenko ◽  
Elmar R. Musaev
Keyword(s):  
Medical Research ◽  
Malignant Transformation ◽  
Pulmonary Metastases ◽  
Cell Tumor ◽  
Research Center ◽  
Tumor Localization ◽  
National Medical ◽  
Metastasis Development

e23501 Background: Giant cell tumor of bone (GCT) is a relatively rare, benign but locally aggressive osteolytic skeletal neoplasm of young adults, most frequently occurs at the epiphysis of long bones. Distant metastases occur approximately 2-6% of cases most often to the lungs. However, pulmonary metastases do not carry the same connotation as metastases associated with malignant tumors, such as lung cancer or sarcoma. Rarely, GCT undergoes true malignant transformation. The aim of this study is to analyze the clinical behavior of advanced GCT during long-term observer in N.N. Blokhin National Medical Research Center of Oncology. Methods: We observed 51 advanced cases among 298 GCT from 2005 till 2020 in N.N. Blokhin National Medical Research Center of Oncology. Disease was histologically confirmed by a sarcoma pathologist. Patients underwent CT/MRI every 2 or 3 months of treatment and every 3, 6 or 12 months of follow up period. Treatment options were included atypical pulmonary resection, palliative surgery, chemotherapy with CAP or AI combination, interferon alfa or observation until progression. After 2013 prefer treatment option for advanced GCT was denosumab with once in 3 month’s regimen after 2 years monthly therapy. All patients received daily calcium and vitamin D supplement. Logistic regression was using for statistic analyzes. Results: Median follow-up was 45 months. The average age of patients was 34,8 years, and the women and men ratio was about 1,8:1. The most commonly affected sites were tibia (23,5%, 12/21), sacrum (17,6%), radius (13,7%) and femur (9,8%). According Campanacci classification G3 was the most commonly grade (90%). 46 (56%) cases were anatomically compounded due to tumor localization and 19 (37%) cases were primary disease. 18/298 (6%) cases were with pulmonary metastases. Surgery in history (p < 0,001) and tumor localization in extremity (p < 0,001) were significant for metastasis development. The primary malignant GCT observed in 1% (3/298) cases and 1 % cases of malignant transformation GCT into sarcoma. Only 1/51 (1,9%) death was observed in advanced GCT group. Complications ≥3 grade were observed only in pre-denosumab era. Conclusions: In this study we showed long-term observation of advanced GCT and evaluated significant factors for metastasis development. Surgeries in history and tumor localization were associated with higher risk of metastasis. Denosumab for advanced GCT is a choice of treatment, we wasn’t observed any cases of GCT malignant transformation during denosumab treatment. Quality of live was better in compare with pre-denosumab era. Further investigation of long term denosumab complications is awaited.

IPILIMUMAB IN PATIENTS WITH DISSEMINATED MELANOMA: THE N.N. PETROV NATIONAL MEDICAL RESEARCH CENTER OF ONCOLOGY EXPANDED ACCESS PROGRAM EXPERIENCE

2018 ◽  
Vol 64 (3) ◽  
pp. 388-393
Author(s):  
Yekaterina Anokhina ◽  
V. Rubinchik ◽  
Yekaterina Yaremenko ◽  
Gulfiya Teletaeva ◽  
Dilorom Latipova ◽  
...  
Keyword(s):  
Overall Survival ◽  
Adverse Events ◽  
Medical Research ◽  
Research Center ◽  
Expanded Access ◽  
Risk Of Death ◽  
National Medical ◽  
Expanded Access Program ◽  
Access Program

Ipilimumab (IPI) provides a ten-year overall survival in almost 20 % of selected patients participated in several phase II-III trials. However, the expanded access program (EAP) looks more like routine practice than like clinical trials& This is why the results of such application could be different. Here we present the long-term follow-up data of single center EAP. Ninety-six patients with disseminated melanoma progressing after at least one lines of drug therapy were included at the N.N. Petrov National Medical Research Center of Oncology. Sixty-seven (70 %) patients had stage IV M1c, 35 patients (36 %) had elevated LDH before initiating IPI therapy. All patients received IPI 3 mg / kg IV every 3 weeks for a maximum of 4 cycles. Totally, 320 cycles (mean - 3.3 per patient) were conducted. Grade 3-4 immuno-mediated adverse events (imAE) observed in 18 (19 %) patients. Three patients died of adverse events, possibly associated with ongoing therapy. The median time to progression was 3 (95 % CI, 2.4 to 3.5) mo., the median overall survival was 13 (95 % CI, 8.3 to17.6) mo. Previous immunotherapy with dendritic cell vaccines decreased the risk of death by 48 % (Log-rank p = 0.049). The wild type BRAF status increased three-year overall survival from 29 to 68 % (p = 0.042). Our data confirms long-term safety and efficacy of IPI in patients with pretreated disseminated melanoma in the close to real practice setting.

scholarly journals Therapy intensification in high-risk neuroblastoma patients with poor response to standard induction: experience of Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology

2019 ◽  
Vol 18 (4) ◽  
pp. 19-28
Author(s):  
T. V. Shamanskaya ◽  
D. Y. Kachanov ◽  
A. V. Dumacheva ◽  
M. V. Teleshova ◽  
D. V. Shevtcov ◽  
...  
Keyword(s):  
High Risk ◽  
Medical Research ◽  
Induction Therapy ◽  
Poor Response ◽  
Research Center ◽  
Long Term Results ◽  
Type I ◽  
Pediatric Hematology ◽  
National Medical

High-risk neuroblastoma (NB) is characterized by unsatisfactory treatment results and low probability of long-term survival despite the multimodal therapeutic approach (chemotherapy, surgical treatment, radiation therapy, autologous hematopoietic stem cell transplantation, etc.). One of the prognostic factors in this cohort of patients is the response to induction therapy. The article presents the experience of the intensification of induction therapy in 12 patients with high-risk NB with a poor response (mixed response, stable disease) to standard induction therapy who received treatment at Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, assessing its impact on the prognosis of the disease. The study was approved by the Independent Ethics Committee of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology. Patients received an additional two courses of chemotherapy with the inclusion of a type I topoisomerase inhibitor topotecan (TCE – topotecan, cyclophosphamide, etoposide). This regimen of intensification of therapy has demonstrated its feasibility. The main grade 3–4 toxicity was hematologic. An improvement in response was achieved in 5/12 (41.6%) patients. However, long-term results of therapy remained unsatisfactory. The 3-year EFS was 16.7% (95% CI 0.0–37.8), the 3-year OS was 50.0% (95% CI 21.7–78.3). Thus, the intensification of therapy in patients with high-risk NB with a poor response to standard induction therapy did not improve treatment outcomes.

scholarly journals P11.64 Specialized neurooncologial biorepository of the N.N. Burdenko National Medical Research Center for Neurosurgery: high-quality tumor bank for personalized medicine

2019 ◽  
Vol 21 (Supplement_3) ◽  
pp. iii58-iii58
Author(s):  
D Golbin ◽  
G Pavlova ◽  
M Shifrin ◽  
S Shugay ◽  
T Tsukanova ◽  
...  
Keyword(s):  
Medical Research ◽  
Data Storage ◽  
Tumor Tissue ◽  
Response To Therapy ◽  
Information Support ◽  
Research Center ◽  
Response To Treatment ◽  
Cns Tumor ◽  
National Medical

Abstract BACKGROUND Specialized biorepositories in neurooncology serve for storage of tissue samples derived from patients with central nervous system (CNS) tumors. In 2016 the new facility was launched for collection of CNS tumor specimens. The principal aim of the repository is preparation of frozen CNS tumor samples accompanied by associated clinical, pathological, molecular, and follow-up data. MATERIAL AND METHODS Each surgical biopsy was divided into several aliquots (usually three), registered, and stored in LN2. Since August 2018 from all aliquots a lesser fragment was separated for paraffin block processing. This histological control was applied for quality assurance of frozen samples. Each specimen record was accompanied with demographic, clinical, perioperative, and histological data. In the follow-up, oncological treatment and response to therapy were added to the databank as well as molecular data. All tumor samples are characterized, passportized, stored, and systemically revised. The following biomarkers were evaluated: Cdk4, Cdk6, FGFR, NANOG, OCT4, SOX2, MELK, Nestin, Notch2, Olig2, GFAP, MAP2, β-III-tubulin, PDGFRA. Dedicated original flexible electronic data storage system was designed for information support of tumor collection. Specimen acquisition, procurement, and storage was encoded using SPREC 2.0 coding system. RESULTS Between March 2016 till January 2019 a total of 596 biopsy samples were stored in the repository. All of them were obtained from the patients operated on in N.N. Burdenko National Medical Research Center for Neurosurgery. Among all entities brain gliomas prevailed and comprised 539 biopsy specimens (90,4%). Specimen quality control using histology, immunohistochemistry, fluorescent in situ hybridization, and molecular methods was performed. The frequency of appropriate aliquots is as high as 83,4%. Tumor sample collection included primary and recurrent cases including those, which underwent primary and secondary in N.N. Burdenko National Medical Research Center for Neurosurgery. CONCLUSION Specialized neurooncological biorepository is advantageous due to possibility of tumor tissue collection at different stages of the disease. The associated databank contains patients’ data, tumor tissue data, treatment data, response to treatment, and follow-up data. Further development of the facility will provide collection of the larger spectrum of neurooncological entities, creation of tumor cell culture bank, and experimental therapies for the development of personalized neurooncological treatment.

scholarly journals Pharmacokinetics of 13-cis-Retinoic acid in high-risk neuroblastoma patients

2020 ◽  
Vol 19 (4) ◽  
pp. 20-31
Author(s):  
E. A. Litvin ◽  
D. T. Utalieva ◽  
D. Yu. Kachanov ◽  
A. V. Pshonkin ◽  
M. Ya. Yadgarov ◽  
...  
Keyword(s):  
Retinoic Acid ◽  
High Risk ◽  
Medical Research ◽  
High Performance ◽  
Plasma Concentrations ◽  
Neuroblastoma Cells ◽  
Research Center ◽  
Therapeutic Drug ◽  
Pediatric Hematology ◽  
National Medical

13-cis-Retinoic acid is a differentiation agent for neuroblastoma cells and is a part of post-consolidation therapy for high-risk patients. The effectiveness of this therapeutic approach is currently under study. 26 patients with high-risk neuroblastoma treated at Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology were included in the study of 13-cis-Retinoic acid pharmacokinetics by high-performance liquid chromatography assay with ultraviolet detector depending on the method of administration of drug (swallowed capsules or opened capsules before administration). This study is supported by the Independent Ethics Committee and approved by the Academic Council of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology. The current study showed that the therapeutic concentration of > 2 μM when taking 13-cis-Retinoic acid at a dose of 160 mg/m2/day was achieved in two groups, regardless of the method of drug administration. However, plasma concentrations of 13-cis-Retinoic acid at 4 hours after administration on the 14th day of therapy were higher in the group of patients who swallowed the capsules (4.1 ± 1.8 μM), compared to those who could not do it (1.9 ± 1.5 μM) (p = 0.022). The introduction into the clinical practice of therapeutic drug monitoring of 13-cis-retinoic acid in high-risk neuroblastoma patients with an assessment of peak concentration and dose adjustment of the following courses may be an important point in the attempt to optimize postconsolidation therapy and improve prognosis.

scholarly journals Cervical Cytology–Histology Correlation Based on the American Society of Cytopathology Guideline (2017) at the Russian National Medical Research Center for Obstetrics, Gynecology, and Perinatology

Diagnostics ◽  
2022 ◽  
Vol 12 (1) ◽  
pp. 210
Author(s):  
Aleksandra Asaturova ◽  
Darya Dobrovolskaya ◽  
Alina Magnaeva ◽  
Anna Tregubova ◽  
Guldana Bayramova ◽  
...  
Keyword(s):  
Retrospective Study ◽  
Medical Research ◽  
Sampling Error ◽  
Pap Test ◽  
Research Center ◽  
National Medical ◽  
American Society ◽  
Screening Errors ◽  
Discrepancy Detection ◽  
Self Learning

Recent evidence suggests that a cytology–histology correlation (CHC) with discrepancy detection can both evaluate errors and improve the sensitivity and specificity of the cytologic method. We aimed to analyze the errors in cytologic–histologic discrepancies according to the CHC protocol guideline of the American Society of Cytopathology (2017). This retrospective study included 273 patients seen at the National Medical Research Center of Obstetrics, Gynecology and Perinatology (Moscow, Russia) between January 2019 and September 2021. The patients’ mean age was 34 ± 8.1 years. The cytology–histology agreement was noted in 158 cases (57.9%). Major discrepancies were found in 21 cases (7.6%), while minor discrepancies were noted in 93 cases (34.1%). The reason for 13 (4.8%) discrepancies was a colposcopy sampling error and, in 46 (16.8%) cases, the reason was a Papanicolaou (PAP) test sampling error. The discrepancy between primary and reviewed cytology was due interpretive errors in 13 (4.8%) cases and screening errors in 42 (15.4%) cases. We demonstrated that the ASC guidelines facilitate cervical CHC. A uniform application of these guidelines would standardize cervical CHCs internationally, provide a scope for the inter-laboratory comparison of data, and enhance self-learning and peer learning.

scholarly journals Non-spherocytic hemolytic anemia caused by erythrocyte pyruvate kinase defiiency: the analysis of genetic defects in pediatric patients, living in Russian Federation

2021 ◽  
Vol 20 (2) ◽  
pp. 84-96
Author(s):  
E. A. Cherniak ◽  
N. E. Sokolova ◽  
K. V. Semiglazova ◽  
I. N. Lavrentyeva ◽  
E. K. Donush ◽  
...  
Keyword(s):  
Medical Research ◽  
Hemolytic Anemia ◽  
Research Center ◽  
Compound Heterozygous ◽  
Missense Mutations ◽  
Pediatric Hematology ◽  
Transfusion Independence ◽  
National Medical ◽  
Retrospective Data Analysis ◽  
Minimum Age

The article presents retrospective data analysis of a cohort of patients with PKD (n = 41 patients, aged 4 months – 26,5 years, median of age – 5 years 1 month) who were examined at the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology for unspecifid hereditary hemolytic anemia during the period 2013–2020. The study was approved by the Independent Ethics Committee of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology. In all patients, the diagnosis was confimed by Next Generation sequencing (NGS). The homozygous mutations in the PKLR gene were found in 10 patients (24.39%), compound heterozygous mutations in 31 patients (75.61%), 77.78% of them were missense mutations. Gender distribution (male:female) was 1:1.73. At least once transfusion of erythrocyte suspension was required to 40 (97.56%) patients. The minimum age at the time of the debut of transfusion dependence was the fist day of life, the maximum was 4 years. Exchange blood transfusion was performed in 13 children, severe normocytic hyperregenerative anemia with transfusion of red blood cells in the fist days of life was noted in 12 children, at the 1st month of life – in 9 children, at the 2nd month of life – in 8 children, at the 3rd month – in 6 children, at the 5th month – in 2 children, at the 1st year – in 1 child, and 2 children underwent single transfusions on the background of infectious episodes at 3 and 4 years respectively. Splenectomy due to high transfusion dependence was performed in 10 patients: transfusion independence was achieved in 5 patients, in 5 – an increase in the interval between blood transfusions. Median of surgical intervention (9 patients): 7 years 4 months, minimum age – 1 year 4 months, maximum – 14 years 4 months. In total, 36 genotypes were described in 41 patients, among them were: c.1529G>A in 3 patients, c.1137_1139del / c.1456C>T – in 2 patients, c.1079G>A/c.1529G>A in 2 patients, c.1130T>C/c.1456C>T in 2 patients, other genotypes occurred once. Two mutations were the most frequent: c.1456C>T (16.67%) and c.1529G>A (16.67%). 19 (46,34%) of patients had previously not described mutations.

Gamma Knife radiosurgery for skull base meningioma: long-term results of low-dose treatment

2008 ◽  
Vol 109 (5) ◽  
pp. 804-810 ◽  
Author(s):  
Yoshiyasu Iwai ◽  
Kazuhiro Yamanaka ◽  
Hidetoshi Ikeda
Keyword(s):  
Malignant Transformation ◽  
Gamma Knife ◽  
Low Dose ◽  
Gamma Knife Radiosurgery ◽  
Cranial Base ◽  
Local Failure ◽  
Long Term Results ◽  
Early Results

Object In this study, the authors evaluate the long-term results after Gamma Knife radiosurgery of cranial base meningiomas. This study is a follow-up to their previously published report on the early results. Methods Between January 1994 and December 2001, the authors treated benign cranial base meningiomas in 108 patients using low-dose Gamma Knife radiosurgery. The tumor volumes ranged from 1.7 to 55.3 cm3 (median 8.1 cm3), and the radiosurgery doses ranged from 8 to 12 Gy (median 12 Gy) to the tumor margin. Results The mean duration of follow-up was 86.1 months (range 20–144 months). Tumor volume decreased in 50 patients (46%), remained stable in 51 patients (47%), and increased (local failure) in 7 patients (6%). Eleven patients experienced tumor recurrence outside the treatment field. Among these patients, marginal failure was seen in 5 and distant recurrence was seen in 6. Seven patients were thought to have malignant transformation based on histological or radiological characteristics of the lesion. The actuarial progression-free survival rate, including malignant transformation and outside recurrence, was 93% at 5 years and 83% at 10 years. Neurological status improved in 16 patients (15%). Permanent radiation injury occurred in 7 patients (6%). Conclusions Gamma Knife radiosurgery is a safe and effective treatment for cranial base meningiomas as demonstrated with a long-term follow-up period of > 7 years. Surgeons must be aware of the possibility of treatment failure, defined as local failure, marginal failure, and malignant transformation; however, this may be the natural course of meningiomas and not related to radiosurgery.

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