N. Subbotina, I. Dolgopolov, Vidmante V. Daylidite, V. K. Boyarshinov, G. Mentkevich
Background. «Graft-versus-host» disease (GVHD) is a major complication of allogeneic Hematopoietic Stem Cell Transplantation (HSCT), the main cause of non-relapse mortality. The actual problems of alternative donors HSCTs are GVHD prophylaxis and its distinction from «graft-versus-tumor» (GVT) effect. Objective. Assessment of incidence and severity of GVHD in children with very high-risk cancer after non-T-depleted haplo-HSCT; searching some correlation between controlled chronic GVHD (chGVHD) and GVT effect after HSCT, compare cyclosporine (CSA) and tacrolimus based GVHD prophylaxis. Methods. We provided the GVHD assessment in 74 recipients of haplo-HSCs from relatives since 2001 till 2018 in retrospective cohort study. Effect of chGVHD on the HSCT results was assessed in 23 patients with acute myeloid leukemia (AML) and 15 patients with solid tumors. We analyzed the incidence of severe acute and chronic GVHD in patients who received CSA or tacrolimus-based prophylaxis. Results. The incidence of severe aGHVD in our study was 15%, chGVHD — 12%. Switch CSA to tacrolimus resulted in reduction of severe aGVHD from 21% to 9% and full elimination of severe extensive chGVHD. Controlled chGVHD after haplo-HSCT was associated with relapse reduction from 48.7% to 30% and improvement of overall survival (OS) from 46.5% to 80.8% in patients with AML. We haven’t seen any clinically significant antitumor effect of graft in patients with solid tumors. Conclusion. In our study the incidence of severe GVHD corresponds to world data after HSCTs from alternative donors, it went down after switch from CSA to tacrolimus. We found some correlation between controlled chGVHD development and HSCT results improvement in patients with AML. The GVT effect of haplo-graft in solid tumors is almost absent.
{"title":"«Graft-Versus-Host» Disease after Haploidentical Hematopoietic Stem Cell Transplantation from Relative Donors in Children with Cancer, Prophylaxis Regimens and Correlation with «Graft-Versus-Tumor» Effect: a Retrospective Cohort Study","authors":"N. Subbotina, I. Dolgopolov, Vidmante V. Daylidite, V. K. Boyarshinov, G. Mentkevich","doi":"10.15690/onco.v6i2.2023","DOIUrl":"https://doi.org/10.15690/onco.v6i2.2023","url":null,"abstract":"Background. «Graft-versus-host» disease (GVHD) is a major complication of allogeneic Hematopoietic Stem Cell Transplantation (HSCT), the main cause of non-relapse mortality. The actual problems of alternative donors HSCTs are GVHD prophylaxis and its distinction from «graft-versus-tumor» (GVT) effect. Objective. Assessment of incidence and severity of GVHD in children with very high-risk cancer after non-T-depleted haplo-HSCT; searching some correlation between controlled chronic GVHD (chGVHD) and GVT effect after HSCT, compare cyclosporine (CSA) and tacrolimus based GVHD prophylaxis. Methods. We provided the GVHD assessment in 74 recipients of haplo-HSCs from relatives since 2001 till 2018 in retrospective cohort study. Effect of chGVHD on the HSCT results was assessed in 23 patients with acute myeloid leukemia (AML) and 15 patients with solid tumors. We analyzed the incidence of severe acute and chronic GVHD in patients who received CSA or tacrolimus-based prophylaxis. Results. The incidence of severe aGHVD in our study was 15%, chGVHD — 12%. Switch CSA to tacrolimus resulted in reduction of severe aGVHD from 21% to 9% and full elimination of severe extensive chGVHD. Controlled chGVHD after haplo-HSCT was associated with relapse reduction from 48.7% to 30% and improvement of overall survival (OS) from 46.5% to 80.8% in patients with AML. We haven’t seen any clinically significant antitumor effect of graft in patients with solid tumors. Conclusion. In our study the incidence of severe GVHD corresponds to world data after HSCTs from alternative donors, it went down after switch from CSA to tacrolimus. We found some correlation between controlled chGVHD development and HSCT results improvement in patients with AML. The GVT effect of haplo-graft in solid tumors is almost absent.","PeriodicalId":37249,"journal":{"name":"Onkopediatria","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42322788","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
This publication focuses on skin toxicity and skin manifestations of a chronic graft versus host disease (chGVHD). The article provides analysis of contemporary literature on these pathologies, presents new approach to preventing its development and also introduces pathogenetically determined patterns of non-drug correction of skin manifestations.
{"title":"Principles of Non-Pharmacological Correction of Skin Toxicity and Skin Manifestations of a Chronic «Graft Versus Host» Disease","authors":"E. A. Shuginina, O. Rassokhina","doi":"10.15690/ONCO.V6I2.2020","DOIUrl":"https://doi.org/10.15690/ONCO.V6I2.2020","url":null,"abstract":"This publication focuses on skin toxicity and skin manifestations of a chronic graft versus host disease (chGVHD). The article provides analysis of contemporary literature on these pathologies, presents new approach to preventing its development and also introduces pathogenetically determined patterns of non-drug correction of skin manifestations.","PeriodicalId":37249,"journal":{"name":"Onkopediatria","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47075831","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
T. V. Gorbunova, A. Rodina, R. V. Shishkov, N. V. Ivanova, I. V. Glekov, Alina A. Malachova, V. Polyakov
The incidence of esthesioneuroblastoma in children under 15 years of age is 0.1 per 100.000 children. Distinctive histological features of this tumor are diffuse accumulation of neuron-specific enolase, synaptophysin, chromogranin, and variable expression of cytokeratins. Diagnosis of the tumor includes endoscopic examination of the nasal cavity and nasopharynx, magnetic resonance imaging (MRI) and computed tomography (CT) of the skull base, paranasal sinuses with intravenous contrast. PET-CT is advisable to use for the detection of regional and distant metastases, as well as for suspected relapse. In patients of adult age, a negative effect on the outcome of the disease was detected, the detection of metastases in the lymph nodes of the neck, the presence of tumor cells at the edges of tumor resection and a high degree of malignancy of the tumor according to the Hyams system. Therapeutic approaches depend on the stage of esthesioneuroblastoma by Kadish. In the A-stage, surgical treatment is advisable. In the presence of tumor cells at the edges of the resection or residual tumor, radiation therapy is performed. In case of B-stage, surgical treatment is combined with the mandatory irradiation of the primary tumor area. In patients with the C-stage, neoadjuvant chemotherapy or radiation is performed, followed by a surgical treatment, adjuvant chemotherapy and/or radiation therapy. Patients with D-stage chemoradiation therapy is indicated. There is no consensus on an effective drug regimen. Overall 5-year survival varies significantly depending on the design of the study — 55% to 98%. Further study of the features of the clinical picture, morphological and molecular features and the course of the disease will help to improve our understanding of the nature of the tumor.
{"title":"Approaches to the Treatment of Children with Esthesioneuroblastoma: Literature Review","authors":"T. V. Gorbunova, A. Rodina, R. V. Shishkov, N. V. Ivanova, I. V. Glekov, Alina A. Malachova, V. Polyakov","doi":"10.15690/onco.v6i2.2019","DOIUrl":"https://doi.org/10.15690/onco.v6i2.2019","url":null,"abstract":"The incidence of esthesioneuroblastoma in children under 15 years of age is 0.1 per 100.000 children. Distinctive histological features of this tumor are diffuse accumulation of neuron-specific enolase, synaptophysin, chromogranin, and variable expression of cytokeratins. Diagnosis of the tumor includes endoscopic examination of the nasal cavity and nasopharynx, magnetic resonance imaging (MRI) and computed tomography (CT) of the skull base, paranasal sinuses with intravenous contrast. PET-CT is advisable to use for the detection of regional and distant metastases, as well as for suspected relapse. In patients of adult age, a negative effect on the outcome of the disease was detected, the detection of metastases in the lymph nodes of the neck, the presence of tumor cells at the edges of tumor resection and a high degree of malignancy of the tumor according to the Hyams system. Therapeutic approaches depend on the stage of esthesioneuroblastoma by Kadish. In the A-stage, surgical treatment is advisable. In the presence of tumor cells at the edges of the resection or residual tumor, radiation therapy is performed. In case of B-stage, surgical treatment is combined with the mandatory irradiation of the primary tumor area. In patients with the C-stage, neoadjuvant chemotherapy or radiation is performed, followed by a surgical treatment, adjuvant chemotherapy and/or radiation therapy. Patients with D-stage chemoradiation therapy is indicated. There is no consensus on an effective drug regimen. Overall 5-year survival varies significantly depending on the design of the study — 55% to 98%. Further study of the features of the clinical picture, morphological and molecular features and the course of the disease will help to improve our understanding of the nature of the tumor.","PeriodicalId":37249,"journal":{"name":"Onkopediatria","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67157735","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Т. М. Михалевская, Е. В. Волочник, Н. Е. Конопля, Олег Иванович Быданов
Background. Low-grade gliomas are the most common brain tumors in children. Gliomas have a favorable prognosis, but in some cases relapses or continued tumor growth occur. With relatively similar clinical and morphological characteristics of tumors, it is rather difficult to select a group of patients who may have progression. Objective. Our aim was to study the impact of certain clinical, histological and molecular characteristics of tumors on the progression/recurrence. Methods. A retrospective cohort study was carried out. Clinical data, histological features and molecular markers (overexpression of phosphorylated ERK1/2 (pERK1/2), mutation of B-Raf kinase (BRAF V600E), deletion of CDKN2A gene (delCDKN2A) were studied in 90 patients with low-grade pediatric gliomas, who were treated in the Center for Pediatric Oncology, Hematology and Immunology during 2010–2018. In gliomas with signs of anaplasia expression of gene of the X-linked alpha-thalassemia syndrome (ATRX), a trymethylated form of histone 3 (H3K27me), p53, and mutation of the dehydrogenase 1 isocitrate 1 gene (IDH1R132H) were also evaluated. Immunohistochemistry and the hybridization in situ (FISH) was performed to evaluate the molecular markers. Results. Statistical analysis confirmed the importance of such factors as non-radical tumor removal (p<0.0001), repeated treatment (p<0.0025), overexpression of pERK1/2 (p<0.0001), histological signs of anaplasia (p<0.0022), areas of diffuse growth (p<0.001), BRAF V600E (p<0.0001), delCDKN2A (p<0.0099). In tissue of gliomas with anaplasia overexpression of pERK1/2, mutation BRAF V600E, delCDKN2A and ATRX loss were more common. When conducting multivariate analysis, non-radical tumor removal and the presence of one of the molecular markers significantly influenced the prognosis (p<0.0001). Conclusion. The definition of molecular markers and the simultaneous assessment of the degree of tumor resection allows us to distinguish a group of patients with a high risk of tumor recurrence / progression.
{"title":"Клиническое значение молекулярных маркеров — мутации BRAF V600E и делеции CDKN2A — при низкозлокачественных глиомах у детей: когортное исследование","authors":"Т. М. Михалевская, Е. В. Волочник, Н. Е. Конопля, Олег Иванович Быданов","doi":"10.15690/ONCO.V6I1/1996","DOIUrl":"https://doi.org/10.15690/ONCO.V6I1/1996","url":null,"abstract":"Background. Low-grade gliomas are the most common brain tumors in children. Gliomas have a favorable prognosis, but in some cases relapses or continued tumor growth occur. With relatively similar clinical and morphological characteristics of tumors, it is rather difficult to select a group of patients who may have progression. Objective. Our aim was to study the impact of certain clinical, histological and molecular characteristics of tumors on the progression/recurrence. Methods. A retrospective cohort study was carried out. Clinical data, histological features and molecular markers (overexpression of phosphorylated ERK1/2 (pERK1/2), mutation of B-Raf kinase (BRAF V600E), deletion of CDKN2A gene (delCDKN2A) were studied in 90 patients with low-grade pediatric gliomas, who were treated in the Center for Pediatric Oncology, Hematology and Immunology during 2010–2018. In gliomas with signs of anaplasia expression of gene of the X-linked alpha-thalassemia syndrome (ATRX), a trymethylated form of histone 3 (H3K27me), p53, and mutation of the dehydrogenase 1 isocitrate 1 gene (IDH1R132H) were also evaluated. Immunohistochemistry and the hybridization in situ (FISH) was performed to evaluate the molecular markers. Results. Statistical analysis confirmed the importance of such factors as non-radical tumor removal (p<0.0001), repeated treatment (p<0.0025), overexpression of pERK1/2 (p<0.0001), histological signs of anaplasia (p<0.0022), areas of diffuse growth (p<0.001), BRAF V600E (p<0.0001), delCDKN2A (p<0.0099). In tissue of gliomas with anaplasia overexpression of pERK1/2, mutation BRAF V600E, delCDKN2A and ATRX loss were more common. When conducting multivariate analysis, non-radical tumor removal and the presence of one of the molecular markers significantly influenced the prognosis (p<0.0001). Conclusion. The definition of molecular markers and the simultaneous assessment of the degree of tumor resection allows us to distinguish a group of patients with a high risk of tumor recurrence / progression.","PeriodicalId":37249,"journal":{"name":"Onkopediatria","volume":"6 1","pages":"34-44"},"PeriodicalIF":0.0,"publicationDate":"2019-04-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67157655","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Leonid A. Martynov, N. Matinyan, E. Belousova, I. A. Letyagin, Tatyana L. Ushakova, V. Polyakov
Background. Retinoblastoma is the most common intraocular malignant tumor in children. Substantial advances have been made in field of local treatment, in particular, through the development of selective intraarterial chemotherapy (SIAC). Life-threatening cardio-respiratory disorders were reported during the course of SIAC at the moment of a.ophthalmica catheterization. Objective. Our aim was to improve the safety and effectiveness of anesthetic management in children with retinoblastoma. Methods. The retrospective study included patients (n=203) who underwent SIAC (517) under general anesthesia in 2013–2018. Results. Hemodynamic parameters in patients who underwent SIAC procedure for the first time (211) were analyzed, in 186 (88%) cases, smooth anesthesia was observed, the deviations of blood pressure and heart rate did not exceed 20% of the baseline values. In 25 (12%) cases, patients who underwent the first SIAC procedure developed bronchospasm with a decrease in respiratory volume of up to 30% from the initial values 10–20 seconds after catheterization of a.ophthalmica with microcatheter. When analyzing hemodynamic parameters in patients who underwent repeated SIAC procedures (2nd, 3rd sessions, etc.) (306), in 177 (58%) cases, a clinical picture of the trigemino-pulmonary reflex of varying severity manifested. Conclusion. Severe cardio-respiratory complications are often observed during SIAC in children with retinoblastoma. These reactions occur predominantly during the second or subsequent procedures and can be potentially life-threatening. All patients with retinoblastoma who undergo SIAC should be considered at high risk.
{"title":"Life-Threatening Complications During Superselective Ophthalmic Arterial Chemotherapy for Retinoblastoma: Retrospective Cohort Study","authors":"Leonid A. Martynov, N. Matinyan, E. Belousova, I. A. Letyagin, Tatyana L. Ushakova, V. Polyakov","doi":"10.15690/ONCO.V6I1/1995","DOIUrl":"https://doi.org/10.15690/ONCO.V6I1/1995","url":null,"abstract":"Background. Retinoblastoma is the most common intraocular malignant tumor in children. Substantial advances have been made in field of local treatment, in particular, through the development of selective intraarterial chemotherapy (SIAC). Life-threatening cardio-respiratory disorders were reported during the course of SIAC at the moment of a.ophthalmica catheterization. Objective. Our aim was to improve the safety and effectiveness of anesthetic management in children with retinoblastoma. Methods. The retrospective study included patients (n=203) who underwent SIAC (517) under general anesthesia in 2013–2018. Results. Hemodynamic parameters in patients who underwent SIAC procedure for the first time (211) were analyzed, in 186 (88%) cases, smooth anesthesia was observed, the deviations of blood pressure and heart rate did not exceed 20% of the baseline values. In 25 (12%) cases, patients who underwent the first SIAC procedure developed bronchospasm with a decrease in respiratory volume of up to 30% from the initial values 10–20 seconds after catheterization of a.ophthalmica with microcatheter. When analyzing hemodynamic parameters in patients who underwent repeated SIAC procedures (2nd, 3rd sessions, etc.) (306), in 177 (58%) cases, a clinical picture of the trigemino-pulmonary reflex of varying severity manifested. Conclusion. Severe cardio-respiratory complications are often observed during SIAC in children with retinoblastoma. These reactions occur predominantly during the second or subsequent procedures and can be potentially life-threatening. All patients with retinoblastoma who undergo SIAC should be considered at high risk.","PeriodicalId":37249,"journal":{"name":"Onkopediatria","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-04-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46530503","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Background. The social significance of pediatric oncology and attention to the problems of treating children, both representatives of the authorities and the community, a small number of primary patients detected annually, a shortage of pediatric oncologists and pediatric oncological beds explain problems in organizing medical care for this category of patients. This requires a search for new approaches to the organization of medical care, based on scientific calculations. Objective. Our aim was to improve the organization of medical care for children with cancer in the Russian Federation. Methods: The operative reports for 2017 of executive bodies in the sphere of health care 81/85 (95.3%) of the subjects of the Russian Federation were analyzed. Results. The number of children was 28 132 685 people. (0–17 years), the number of child oncology departments — 47, children›s oncological beds — 1925 (0.7 for 10,000), the average number of days of berth employment in the year — 315.3 days. In 30 (35.3%) subjects of the department of pediatric oncology are absent, in 12 (14.1%) — there are no children›s oncological beds. The number of physicians that provide medical care to children with cancer — 392, of which 259 (66%, 0.09 for 10 thousand) have a certificate, a pediatrician oncologist. In 12 (14.1%) subjects, there are no doctors-children oncologists, 6 (7%) did not provide these data. In 6 (7%) subjects there are no children›s oncological beds and doctors-children›s oncologists. The incidence of malignant tumors was 13.2 (100,000 thousand) prevalence — 91.3, death rate — 2.5, a one-year lethality — 6.5%. Actually revealed 8.3% of patients. 1385 (37.4%) of primary patients were sent to medical organizations of federal subordination, 61 (1.6%) primary patients left the territory of the Russian Federation. Conclusion. It is necessary to take measures aimed at modernizing the system of rendering medical care to children with cancer, namely: to increase the reliability of statistical data, to analyze the needs of subjects in the number of children’s oncological beds and pediatric oncologists, to introduce criteria for selecting patients for children’s oncological beds as part of medical organizations of various levels, to minimize the deficit of doctors and children’s oncologists, to systematically increase the number of children’s cancer beds simultaneously dividing existing in accordance with the needs of the population and the level of congestion, as well as ensure the routing of patients in accordance with a three-tier model.
{"title":"Medical Care for Children with Cancer in the Russian Federation: Current Situation and Development Prospects","authors":"M. Rykov","doi":"10.15690/onco.v6i1/1993","DOIUrl":"https://doi.org/10.15690/onco.v6i1/1993","url":null,"abstract":"Background. The social significance of pediatric oncology and attention to the problems of treating children, both representatives of the authorities and the community, a small number of primary patients detected annually, a shortage of pediatric oncologists and pediatric oncological beds explain problems in organizing medical care for this category of patients. This requires a search for new approaches to the organization of medical care, based on scientific calculations. Objective. Our aim was to improve the organization of medical care for children with cancer in the Russian Federation. Methods: The operative reports for 2017 of executive bodies in the sphere of health care 81/85 (95.3%) of the subjects of the Russian Federation were analyzed. Results. The number of children was 28 132 685 people. (0–17 years), the number of child oncology departments — 47, children›s oncological beds — 1925 (0.7 for 10,000), the average number of days of berth employment in the year — 315.3 days. In 30 (35.3%) subjects of the department of pediatric oncology are absent, in 12 (14.1%) — there are no children›s oncological beds. The number of physicians that provide medical care to children with cancer — 392, of which 259 (66%, 0.09 for 10 thousand) have a certificate, a pediatrician oncologist. In 12 (14.1%) subjects, there are no doctors-children oncologists, 6 (7%) did not provide these data. In 6 (7%) subjects there are no children›s oncological beds and doctors-children›s oncologists. The incidence of malignant tumors was 13.2 (100,000 thousand) prevalence — 91.3, death rate — 2.5, a one-year lethality — 6.5%. Actually revealed 8.3% of patients. 1385 (37.4%) of primary patients were sent to medical organizations of federal subordination, 61 (1.6%) primary patients left the territory of the Russian Federation. Conclusion. It is necessary to take measures aimed at modernizing the system of rendering medical care to children with cancer, namely: to increase the reliability of statistical data, to analyze the needs of subjects in the number of children’s oncological beds and pediatric oncologists, to introduce criteria for selecting patients for children’s oncological beds as part of medical organizations of various levels, to minimize the deficit of doctors and children’s oncologists, to systematically increase the number of children’s cancer beds simultaneously dividing existing in accordance with the needs of the population and the level of congestion, as well as ensure the routing of patients in accordance with a three-tier model.","PeriodicalId":37249,"journal":{"name":"Onkopediatria","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67157561","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
L. Olkhova, O. Zheludkova, M. Ryzhova, Y. Kushel, Alexander M. Kryanev, V. Ozerova
Background. Atypical teratoid rhabdoid tumor (AT/RT) of the central nervous system (CNS) is an aggressive malignant tumor, mostly affecting children younger than 3 years old. AT/RT is characterized by the deletion and/or mutation of gene SMARCB1/INI1, located in the long arm 22q11, leading to a loss of the nuclear expression of protein INI1, which may be detected by immunohistochemical assay with the anti-INI1 antibody. These tumors are mostly located in the brain, and their incidence in the spinal cord is very rare, with just a few publications on their clinical observations.Description of a Clinical Case. A clinical example of AT/RT in a two-year patient with the tumor located in the thoracic and lumbar spinal cord is described. The patient underwent surgical treatment consisting in the tumor resection and also chemoradiotherapy. Evaluation showed complete response. By the moment this paper was written, the duration of event-free survival of this patient was 27 months.Conclusion. This paper reviews the earlier published cases of spinal cord AT/RT, in which the most common location of the tumor was the cervical portion of the spinal cord. The maximum overall survival was 18 months, and the average overall survival was 9 months. In our clinical example, we demonstrate a long-term complete response to the multimodal treatment for AT/RT.
{"title":"Atypical Teratoid Rhabdoid Tumor of Spinal Cord: A Clinical Case","authors":"L. Olkhova, O. Zheludkova, M. Ryzhova, Y. Kushel, Alexander M. Kryanev, V. Ozerova","doi":"10.15690/onco.v5i4.1970","DOIUrl":"https://doi.org/10.15690/onco.v5i4.1970","url":null,"abstract":"Background. Atypical teratoid rhabdoid tumor (AT/RT) of the central nervous system (CNS) is an aggressive malignant tumor, mostly affecting children younger than 3 years old. AT/RT is characterized by the deletion and/or mutation of gene SMARCB1/INI1, located in the long arm 22q11, leading to a loss of the nuclear expression of protein INI1, which may be detected by immunohistochemical assay with the anti-INI1 antibody. These tumors are mostly located in the brain, and their incidence in the spinal cord is very rare, with just a few publications on their clinical observations.Description of a Clinical Case. A clinical example of AT/RT in a two-year patient with the tumor located in the thoracic and lumbar spinal cord is described. The patient underwent surgical treatment consisting in the tumor resection and also chemoradiotherapy. Evaluation showed complete response. By the moment this paper was written, the duration of event-free survival of this patient was 27 months.Conclusion. This paper reviews the earlier published cases of spinal cord AT/RT, in which the most common location of the tumor was the cervical portion of the spinal cord. The maximum overall survival was 18 months, and the average overall survival was 9 months. In our clinical example, we demonstrate a long-term complete response to the multimodal treatment for AT/RT.","PeriodicalId":37249,"journal":{"name":"Onkopediatria","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2018-12-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49664390","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
N. Samburova, S. Kalinin, T. Zhevak, P. F. Litvitsky
The article provides the characteristic of a tumor from cells of cartilaginous tissue — chondroblastoma. Current data on etiology, key links of pathogenesis, types of tumor, General clinical manifestations, approaches to diagnosis and treatment in children are analyzed.
{"title":"Chondroblastoma: Etiology, Pathogenesis, Methods of Diagnosis and Treatment","authors":"N. Samburova, S. Kalinin, T. Zhevak, P. F. Litvitsky","doi":"10.15690/onco.v5i4.1968","DOIUrl":"https://doi.org/10.15690/onco.v5i4.1968","url":null,"abstract":"The article provides the characteristic of a tumor from cells of cartilaginous tissue — chondroblastoma. Current data on etiology, key links of pathogenesis, types of tumor, General clinical manifestations, approaches to diagnosis and treatment in children are analyzed.","PeriodicalId":37249,"journal":{"name":"Onkopediatria","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2018-12-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48991680","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
М. А. Рубанский, Анатолий Петрович Казанцев, П. А. Керимов, О. А. Кириллова, Светлана Николаевна Михайлова, А. С. Тёмный, Е. В. Шарапова, М. В. Рубанская
Background . According to the data of the world literature and own data of observations renal cell carcinoma (RCC) in children is a rare pathology. There are mainly localized and locally common forms of the disease, characterized by a good prognosis and rare reccurence. The tactic of the treatment in this group of patients based on radical surgery. If there are distant metastases, adjuvant chemotherapy is performed. The drug of choice in metastatic forms is a tyrosine kinase ingibitor. Description of a Clinical Case . A review of the world literature on the problem of RCC in children and also the clinical observation of RCC in a 6 years old patient, who treated in N.N. Blokhin National Medical Research Center of Oncology (Moscow), are presented. Now the patient is healthy and is under the dynamic observation. Conclusion . The rarity of RCС requires careful examination and observation of patients, morphological and molecular-genetic study of the tumor for features of biology and develop of «adult» cancer in children.
{"title":"Почечно-клеточный рак у пациентки в возрасте 6 лет: редкое клиническое наблюдение","authors":"М. А. Рубанский, Анатолий Петрович Казанцев, П. А. Керимов, О. А. Кириллова, Светлана Николаевна Михайлова, А. С. Тёмный, Е. В. Шарапова, М. В. Рубанская","doi":"10.15690/ONCO.V5I4.1971","DOIUrl":"https://doi.org/10.15690/ONCO.V5I4.1971","url":null,"abstract":"Background . According to the data of the world literature and own data of observations renal cell carcinoma (RCC) in children is a rare pathology. There are mainly localized and locally common forms of the disease, characterized by a good prognosis and rare reccurence. The tactic of the treatment in this group of patients based on radical surgery. If there are distant metastases, adjuvant chemotherapy is performed. The drug of choice in metastatic forms is a tyrosine kinase ingibitor. Description of a Clinical Case . A review of the world literature on the problem of RCC in children and also the clinical observation of RCC in a 6 years old patient, who treated in N.N. Blokhin National Medical Research Center of Oncology (Moscow), are presented. Now the patient is healthy and is under the dynamic observation. Conclusion . The rarity of RCС requires careful examination and observation of patients, morphological and molecular-genetic study of the tumor for features of biology and develop of «adult» cancer in children.","PeriodicalId":37249,"journal":{"name":"Onkopediatria","volume":"5 1","pages":"278-283"},"PeriodicalIF":0.0,"publicationDate":"2018-12-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67157512","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Background. Improving the quality of medical care is based on a coordinated policy at the federal, regional and municipal levels in the field of health. The implementation of this goal is based on a regular assessment of the current state of the healthcare system in the Russian Federation.Objective. Our aim was to analyse of the main indicators characterizing medical care for children with cancer in the South Federal District.Methods. The operative reports for 2017 of the executive authorities in the sphere of health protection of 8 subjects of the Russian Federation that are part of the South Federal District of the Russian Federation have been analyzed.Results. The number of children were 3 216 797 people (aged 0–17 years), the number of children’s oncological beds is 272 (0.8 per 10 thousand children aged 0–17 years), the average number of days of berth employment in the year is 289.9 bed days. In 4 (50%) subjects of the department of pediatric oncology are absent, in 1 (12.5%) — there are no children’s oncological beds. The number of doctors providing medical care to children with cancer is 50, of them 42 (84%, 0.1 per 10 thousand children aged 0–17 years) have a certificate of a pediatric oncologist. In 2 (25%) subjects, there are no pediatric oncologists. Incidence of malignant tumors was 13.9 (per 100,000 children aged 0–17 years), prevalence was 86.5 (per 100,000 children aged 0–17 years), mortality was 2.5 (per 100,000 children aged 0–17 years), a one-year mortality rate of 4.7%. 8% of patients were actively detected. 104 (23.2%) of primary patients were sent to medical organizations of federal subordination, 4 (0.9%) of primary patients left the territory of the Russian Federation.Conclusion. The analysis revealed a number of defects: incorrect information contained in the reports, low incidence (detectability) of patients, including during planned preventive examinations, staff shortages and non-rational use of bed facilities.
{"title":"Analysis of Some Indicators Characterizing the Quality of Medical Care for Children with Cancer in the South Federal District of the Russian Federation: An Ecological Study","authors":"M. Rykov","doi":"10.15690/onco.v5i4.1967","DOIUrl":"https://doi.org/10.15690/onco.v5i4.1967","url":null,"abstract":"Background. Improving the quality of medical care is based on a coordinated policy at the federal, regional and municipal levels in the field of health. The implementation of this goal is based on a regular assessment of the current state of the healthcare system in the Russian Federation.Objective. Our aim was to analyse of the main indicators characterizing medical care for children with cancer in the South Federal District.Methods. The operative reports for 2017 of the executive authorities in the sphere of health protection of 8 subjects of the Russian Federation that are part of the South Federal District of the Russian Federation have been analyzed.Results. The number of children were 3 216 797 people (aged 0–17 years), the number of children’s oncological beds is 272 (0.8 per 10 thousand children aged 0–17 years), the average number of days of berth employment in the year is 289.9 bed days. In 4 (50%) subjects of the department of pediatric oncology are absent, in 1 (12.5%) — there are no children’s oncological beds. The number of doctors providing medical care to children with cancer is 50, of them 42 (84%, 0.1 per 10 thousand children aged 0–17 years) have a certificate of a pediatric oncologist. In 2 (25%) subjects, there are no pediatric oncologists. Incidence of malignant tumors was 13.9 (per 100,000 children aged 0–17 years), prevalence was 86.5 (per 100,000 children aged 0–17 years), mortality was 2.5 (per 100,000 children aged 0–17 years), a one-year mortality rate of 4.7%. 8% of patients were actively detected. 104 (23.2%) of primary patients were sent to medical organizations of federal subordination, 4 (0.9%) of primary patients left the territory of the Russian Federation.Conclusion. The analysis revealed a number of defects: incorrect information contained in the reports, low incidence (detectability) of patients, including during planned preventive examinations, staff shortages and non-rational use of bed facilities.","PeriodicalId":37249,"journal":{"name":"Onkopediatria","volume":"242 ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2018-12-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41277666","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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