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 ◽  
...  

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.

2021 ◽  
Vol 20 (2) ◽  
pp. 111-120
Author(s):  
M. A. Senchenko ◽  
D. S. Abramov ◽  
G. A. Nasirdinova ◽  
E. V. Volchkov ◽  
D. M. Konovalov ◽  
...  

Lymphocyte-predominant Hodgkin's lymphoma (NLPHL) is a unique variant of Hodgkin's lymphoma (LH) with a relatively good prognosis. The tumor differs markedly from classic LH and is one of the forms B cell lymphoma. Despite the indolent course, it has a tendency to multiple and often late relapses. Microscopically, the tumor has 6 distinguishable morphological patterns. Despite the prevalence in all age groups, most of the original studies were performed among adult patients, while there are only several publications among the children's population. The aim of this study – retrospective analysis pediatric group of the NLPHL, evaluate the prognostic implication of histopathologic variants. Сomparing our own data with another study groups. 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. Study was included the biopsies aged 3 to 18 years (median 10.5 years) of 28 patients with NLPHL from the archive by Department of Pathology Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology from 2014 to 2020. The tumor more commonly affects males (20 boys and 8 girls, male-female ratio, 2.5:1). Complete clinical information was available in 24 patients. The clonal molecular assays were performed in 2 cases of relapse/progression of the disease. The Fisher's exact test was used to compare and evaluate the statistical significance of the differences in groups of patterns. There were no significant differences between typical patterns and variants, probably due to the small number of the patients. Further research will create a predictive scale for stratification by the risk groups. In cases of poor response to therapy, there is a risk that the pattern will turn into a prognostically more unfavorable variant.


2021 ◽  
Vol 39 (15_suppl) ◽  
pp. e23501-e23501
Author(s):  
Anastasia Alekseevna Tararykova ◽  
Alexander A. Fedenko ◽  
Elmar R. Musaev

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.


2018 ◽  
Vol 64 (3) ◽  
pp. 388-393
Author(s):  
Yekaterina Anokhina ◽  
V. Rubinchik ◽  
Yekaterina Yaremenko ◽  
Gulfiya Teletaeva ◽  
Dilorom Latipova ◽  
...  

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.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 489.3-489
Author(s):  
I. Kriulin ◽  
E. Alexeeva ◽  
T. Dvoryakovskaya ◽  
K. Isaeva ◽  
A. Chomakhidze ◽  
...  

Background:Anti-IL-17A biologic drug secukinumab (SEC) proved to be effective for treatment of psoriatic arthritis. However data about its efficacy in juvenile idiopathic arthritis (JIA) are restricted to off-label experience.Objectives:To evaluate the effectiveness and safety of SEC in JIA patients in the National Medical Research Center of Children`s health, Moscow, Russia.Methods:25 patients started SEC therapy from 12/2017 to 11/2019 in single-center prospective study. 3 patients withdrew treatment: two patients (8%) due to AE (1 - allergy followed by MAS after first injection and 1 – leukopenia) and one patient (4%) – after 10 months of treatment due to secondary inefficacy. Among others, 14 patients which were successfully treated for 6 months or longer were included into analysis. At the baseline, information was collected on the characteristics of the onset of the disease, previous therapy and its success. Patients were monitored at least 1 time per year. At each visit, clinical and laboratory characteristics of JIA severity were assessed. Response to therapy was assessed using the ACRPedi 30/50/70/90 criteria, the C.Wallace criteria for inactive disease (WID) and clinical remission. AEs were assessed at each visit.Results:Among 14 patients received SEC for at least 6 months, 7 (50%) have enthesitis-related arthritis, one (7.1%) – persistent oligoarthritis, 4 (28.6%) – RF-negative polyarthritis, 2 (14.3%) – psoriatic arthritis. 6 patients (42.9%) were HLA-B27 positive. Median age of JIA onset was 8.8 (IQR 5:11), age at SEC initiation – 14 (9.9:16.1), disease duration before SEC start – 3.3 (2.7:5.8). 7 (50%) were biologics-naïve, 2 (14.3%) were previously treated with anti-TNF drug, 5 (35.7%) have 2 or more different biologics in anamnesis.SEC demonstrated high efficacy after the first injection resulting in JADAS-71 decreasing in all patients by median 4.3 (1.6:7.1) points and 7/7/5/2 patients (50%/50%/35.7%/14.3%) achieved ACR Pedi 30/50/70/90 response.After 6 months of treatment, WID was achieved by 7 (50%) patients, JADAS-71 decreased from baseline level 15.2 (12.7:20.5) to 0.8 (0:4.2) points, and 14/13/11/9 patients (100%/92.9%/78.6%/64.3%) achieved ACR Pedi 30/50/70/90 response. One patients who had active uveitis at SEC initiation remained with subactive uveitis; one patient with uveitis remission had not flare episodes during follow-up period. One patient (7.1%) had successfully treated evaluation of transaminases after 4-th injection.Conclusion:Secukinumab showed high effectiveness and safety in children with JIA and can be further used both as a first-line drug in JIA associated with HLA-B27, and as an alternative drug for the ineffectiveness of the standard treatment regimen with biologics. No serious adverse events were registered during follow-up period.Disclosure of Interests:Ivan Kriulin: None declared, Ekaterina Alexeeva Grant/research support from: Roche, Pfizer, Centocor, Novartis, Speakers bureau: Roche, Novartis, Pfizer., Tatyana Dvoryakovskaya: None declared, Ksenia Isaeva: None declared, Aleksandra Chomakhidze: None declared, Evgeniya Chistyakova: None declared, Olga Lomakina: None declared, Rina Denisova: None declared, Anna Mamutova: None declared, Anna Fetisova: None declared, Marina Gautier: None declared, Dariya Vankova: None declared, Elizaveta Krekhova: None declared, Meyri Shingarova: None declared, Alina Alshevskaya: None declared, Andrey Moskalev: None declared


2020 ◽  
Vol 19 (4) ◽  
pp. 20-31
Author(s):  
E. A. Litvin ◽  
D. T. Utalieva ◽  
D. Yu. Kachanov ◽  
A. V. Pshonkin ◽  
M. Ya. Yadgarov ◽  
...  

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.


2020 ◽  
pp. 133-140
Author(s):  
Rei Ogawa

AbstractThe Vancouver scar scale, the Manchester scar scale, and the Patient and Observer Scar Assessment Scale (POSAS) are all very well-known scar evaluation methods. These tools are based on a number of scar variables, including color, height, and pliability. However, since all were mainly developed to evaluate burn scars, they are difficult to use in clinical practice for keloids and hypertrophic scars. This is because these pathological scars require both differential diagnosis and a way to evaluate their response to therapy. The Japan Scar Workshop (JSW) has sought to develop a scar assessment scale that meets these clinical needs. The first version of this scar assessment tool was named the JSW scar scale (JSS), and it was reported in 2011. In 2015, the revised second version was reported. The JSS consists of two tables. One is a scar classification table that is used to determine whether the scar is a normal mature scar, a hypertrophic scar, or a keloid. This grading system helps the user to select the most appropriate treatment method for the scar. The other table in the JSS is an evaluation table that is used to judge the response to treatment and for follow-up. Both tables contain sample images of each subjective keloid/hypertrophic scar item that allow the user to evaluate each item without hesitation.


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