Ewing's sarcoma (ES) is a highly malignant tumor of children and adolescents that affects bones and soft tissues with the same frequency. The origins of ES are the subject to many discussions. Differential diagnosis is complicated and requires a full range of immunohistochemical and molecular genetic studies. The prognostic value for extraskeletal and bone ES under current chemotherapy protocols is unknown and requires further analysis. The purposes of this research were a comparative analysis of clinical characteristics, therapeutic approaches and outcomes of the disease in patients with extraskeletal and bone localizations of ES. Materials and methods used: a single-center retrospective cohort study was conducted, which included 330 patients (237 (71.8%) boys/93 (28.2%) girls) aged 0 to 18 y/o (median 11 [7; 14] y/o) with confirmed diagnosis of ES who received treatment in 2008-2022, of which 280 (84.85%) with primary bone localization (group of bone ES - BES), and 50 (15.15%) with soft tissue localization (group of extraskeletal ES - ESES). Comparative analysis of survival rates for primary tumor localization in the area of bones and soft tissues was performed. The median follow-up time for all patients was 35.5 [18.2; 68.5] months, 37.0 [18.0; 71.0] months with BES, and 29.5 [16.8; 65.5] months with ESES. All patients received treatment according to the protocols adopted at the Research Institute of Pediatric Oncology and Hematology named after Academician L.A. Durnov with the N.N. Blokhin Russian Cancer Research Center (Moscow, Russia): MMES-99, ES-2017. Overall survival (OS) was calculated with the Kaplan-Meier estimator. Results: the selected groups differed statistically significantly by gender (74% of boys in the BES group, and 60% of boys the ESES group, p=0.035) and age (10.5 [8; 15] years in the BES group, and 8.5 [4; 12] y/o in the ESES group, p=0.001). BES was diagnosed statistically significantly more often in older age groups than ESES (p=0.004). Compared with BES, in ESES the tumor was statistically significantly more often located in the region of the axial skeleton and visceral organs (24.0% vs. 56%, p<0.001). Disseminated form of the disease in the BES group was recorded in 110 (39.3%) patients, and in 15 (30.0%) in the ESES group. Authors did not find statistically significant differences in overall 5-year OS for localized forms of BES and ESES (79% and 78.5%, respectively), the median OS in these groups was not reached. The OS of patients with disseminated stages of BES and ESES was statistically significantly lower than in the group of localized forms. At the same time, the 5-year OS was 41.2% and 40.6%, the median OS was 46.9 and 28.4 months (p=0.001, respectively). Differences in 5-year progression-free survival (PFS) for localized forms were 71.6% for BES and 75.6% for ESES (p=0.001), for disseminated forms - 32.4% vs. 44.9% (p=0.036, respectively). In the disseminated stage of BES, progression/relapse was detected in 50
{"title":"BONE AND EXTRASKELETAL EWING’S SARCOMA: COMPARATIVE CHARACTERISTICS OF THE COURSE AND OUTCOMES OF THE DISEASE. EXPERIENCE OF THE RESEARCH INSTITUTE OF PEDIATRIC ONCOLOGY AND HEMATOLOGY NAMED AFTER ACADEMICIAN L.A. DURNOV WITH THE N.N. BLOKHIN RUSSIAN CANCER RESEARCH CENTER (MOSCOW, RUSSIA)","authors":"O.M. Romantsova, D.B. Khestanov, A.Z. Dzampaev, V.V. Khairullova, M.M. Efimova, T.V. Gorbunova, K.I. Kirgizov, S.R. Varfolomeeva","doi":"10.24110/0031-403x-2023-102-3-41-50","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-3-41-50","url":null,"abstract":"Ewing's sarcoma (ES) is a highly malignant tumor of children and adolescents that affects bones and soft tissues with the same frequency. The origins of ES are the subject to many discussions. Differential diagnosis is complicated and requires a full range of immunohistochemical and molecular genetic studies. The prognostic value for extraskeletal and bone ES under current chemotherapy protocols is unknown and requires further analysis. The purposes of this research were a comparative analysis of clinical characteristics, therapeutic approaches and outcomes of the disease in patients with extraskeletal and bone localizations of ES. Materials and methods used: a single-center retrospective cohort study was conducted, which included 330 patients (237 (71.8%) boys/93 (28.2%) girls) aged 0 to 18 y/o (median 11 [7; 14] y/o) with confirmed diagnosis of ES who received treatment in 2008-2022, of which 280 (84.85%) with primary bone localization (group of bone ES - BES), and 50 (15.15%) with soft tissue localization (group of extraskeletal ES - ESES). Comparative analysis of survival rates for primary tumor localization in the area of bones and soft tissues was performed. The median follow-up time for all patients was 35.5 [18.2; 68.5] months, 37.0 [18.0; 71.0] months with BES, and 29.5 [16.8; 65.5] months with ESES. All patients received treatment according to the protocols adopted at the Research Institute of Pediatric Oncology and Hematology named after Academician L.A. Durnov with the N.N. Blokhin Russian Cancer Research Center (Moscow, Russia): MMES-99, ES-2017. Overall survival (OS) was calculated with the Kaplan-Meier estimator. Results: the selected groups differed statistically significantly by gender (74% of boys in the BES group, and 60% of boys the ESES group, p=0.035) and age (10.5 [8; 15] years in the BES group, and 8.5 [4; 12] y/o in the ESES group, p=0.001). BES was diagnosed statistically significantly more often in older age groups than ESES (p=0.004). Compared with BES, in ESES the tumor was statistically significantly more often located in the region of the axial skeleton and visceral organs (24.0% vs. 56%, p<0.001). Disseminated form of the disease in the BES group was recorded in 110 (39.3%) patients, and in 15 (30.0%) in the ESES group. Authors did not find statistically significant differences in overall 5-year OS for localized forms of BES and ESES (79% and 78.5%, respectively), the median OS in these groups was not reached. The OS of patients with disseminated stages of BES and ESES was statistically significantly lower than in the group of localized forms. At the same time, the 5-year OS was 41.2% and 40.6%, the median OS was 46.9 and 28.4 months (p=0.001, respectively). Differences in 5-year progression-free survival (PFS) for localized forms were 71.6% for BES and 75.6% for ESES (p=0.001), for disseminated forms - 32.4% vs. 44.9% (p=0.036, respectively). In the disseminated stage of BES, progression/relapse was detected in 50","PeriodicalId":39654,"journal":{"name":"Pediatriya - Zhurnal im G.N. Speranskogo","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135626577","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}
Langerhans cell histiocytosis (LCH) is the most common histiocytic disorder in children and is characterized by clonal expansion of myeloid precursors. The pathological cells are morphologically and phenotypically similar to Langerhans cells. LCH is considered as a sporadic disease as yet with undescribed hereditary factors for its development. However, hereditary predisposition to the development of this pathology cannot be excluded. There are cases of the LCH development in twins described, both in monozygotic and dizygotic. Article represents bibliographical review on the familial forms of Langerhans cell histiocytosis and clinical case of the Langerhans cell histiocytosis development in twin girls.
{"title":"LANGERHANS CELL HISTIOCYTOSIS IN TWINS: BIBLIOGRAPHICAL REVIEW AND CLINICAL CASE","authors":"V.S. Fominykh, N.A. Batmanova, T.T. Valiev, I.A. Nazarenko, K.I. Kirgizov, S.R. Varfolomeeva","doi":"10.24110/0031-403x-2023-102-3-196-202","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-3-196-202","url":null,"abstract":"Langerhans cell histiocytosis (LCH) is the most common histiocytic disorder in children and is characterized by clonal expansion of myeloid precursors. The pathological cells are morphologically and phenotypically similar to Langerhans cells. LCH is considered as a sporadic disease as yet with undescribed hereditary factors for its development. However, hereditary predisposition to the development of this pathology cannot be excluded. There are cases of the LCH development in twins described, both in monozygotic and dizygotic. Article represents bibliographical review on the familial forms of Langerhans cell histiocytosis and clinical case of the Langerhans cell histiocytosis development in twin girls.","PeriodicalId":39654,"journal":{"name":"Pediatriya - Zhurnal im G.N. Speranskogo","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135626599","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}
Pub Date : 2023-06-01DOI: 10.24110/0031-403x-2023-102-3-151-157
A. Asmanov, N. Vashakmadze, V. Vilensky, V. Voinova, I. Vorontsova, I. Dantsev, N. Zhurkova, T.N. Kekeyeva, V. Kenis, T. T. Knyazeva, T. Markova, L. K. Mikhailova, E. Nagaeva, P. Ochirova, E. E. Petryaykina, A. V. Polyakov, D. Popkov, E. Putilina, D. A. Reshchikov, I. G. Rybkina, S. Ryabykh, M. Saghatelyan, L. Toropchina, A. Tiulpakov, S.V. Kazyukov
For citation: A.I. Asmanov, N.D. Vashakmadze, V.A. Vilensky, V.Yu. Voinova, I.G. Vorontsova, I.S. Dantsev, N.V. Zhurkova, T.N. Kekeyeva, V.M. Kenis, T.T. Knyazeva, T.V. Markova, L.K. Mikhailova, E.V. Nagaeva, P.V. Ochirova, E.E. Petryaykina, A.V. Polyakov, D.A. Popkov, E.A. Putilina, D.A. Reshchikov, I.G. Rybkina, S.O. Ryabykh, M.O. Saghatelyan, L.V. Toropchina, A.N. Tiulpakov. Russian interdisciplinary consensus on achondroplasia (approved by the Expert Council in Jan. 2023). Pediatria n.a. G.N. Speransky. 2023; 102 (3): 151-157. DOI: 10.24110/0031-403X-2023-102-3-151-157.
{"title":"Russian interdisciplinary consensus on achondroplasia (approved by the Expert Council in Jan. 2023)","authors":"A. Asmanov, N. Vashakmadze, V. Vilensky, V. Voinova, I. Vorontsova, I. Dantsev, N. Zhurkova, T.N. Kekeyeva, V. Kenis, T. T. Knyazeva, T. Markova, L. K. Mikhailova, E. Nagaeva, P. Ochirova, E. E. Petryaykina, A. V. Polyakov, D. Popkov, E. Putilina, D. A. Reshchikov, I. G. Rybkina, S. Ryabykh, M. Saghatelyan, L. Toropchina, A. Tiulpakov, S.V. Kazyukov","doi":"10.24110/0031-403x-2023-102-3-151-157","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-3-151-157","url":null,"abstract":"For citation: A.I. Asmanov, N.D. Vashakmadze, V.A. Vilensky, V.Yu. Voinova, I.G. Vorontsova, I.S. Dantsev, N.V. Zhurkova, T.N. Kekeyeva, V.M. Kenis, T.T. Knyazeva, T.V. Markova, L.K. Mikhailova, E.V. Nagaeva, P.V. Ochirova, E.E. Petryaykina, A.V. Polyakov, D.A. Popkov, E.A. Putilina, D.A. Reshchikov, I.G. Rybkina, S.O. Ryabykh, M.O. Saghatelyan, L.V. Toropchina, A.N. Tiulpakov. Russian interdisciplinary consensus on achondroplasia (approved by the Expert Council in Jan. 2023). Pediatria n.a. G.N. Speransky. 2023; 102 (3): 151-157. DOI: 10.24110/0031-403X-2023-102-3-151-157.","PeriodicalId":39654,"journal":{"name":"Pediatriya - Zhurnal im G.N. Speranskogo","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"87449664","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}
Pub Date : 2023-06-01DOI: 10.24110/0031-403x-2023-102-3-65-70
D. V. Rybakova, P. A. Kerimov, D. Podluzhny, Y. Patyutko, M. Rubansky, A. S. Temnyy, E. A. Petrash, S. Varfolomeeva, I. Stilidi
The article is about the experience in treating of pediatric patients with tumors of the pancreas who have undergone the gastro-pancreatoduodenal resection (GPDR) and pancreatoduodenal resection (PDR) at the Research Institute of Pediatric Oncology and Hematology named after Academician L.A. Durnov with the N.N. Blokhin Russian Cancer Research Center (Moscow, Russia). These kinds of surgical interventions are performed both with local neoplasms of the pancreatic head and with the spread of the tumor process to the duodenum, distal part of the stomach, and parapancreatic tissue. Materials and methods used: 13 GPDR, PDR surgical interventions were performed in children aged 5 to 16 y/o in 2010-2022. The results of treatment of these patients were assessed as satisfactory despite the complications that arose in some patients in the early and late postoperative periods. All patients are alive. GPDR surgical interventions were performed in 6 patients, and PDR in 7. The most frequently verified were solid pseudopapillary tumor of the pancreatic head (in 10 cases), neuroendocrine tumor (in 2 cases) and paraganglioma in a single case. Discussion and conclusions: bleeding from the pancreatic branch of the splenic artery and acute pancreatitis were noted among the early postoperative complications. Recurrent phenomenon of enzyme evasion and syndrome of excessive bacterial growth were revealed in the late postoperative period. Despite the complications arisen all patients are alive without signs of recurrence of the disease. The prognosis for this category of patients with radically performed surgical intervention is favorable.
本文是关于N.N. Blokhin俄罗斯癌症研究中心(Moscow, Russia)以L.A. Durnov院士命名的儿童肿瘤学和血液学研究所(academy of L.A. Durnov)对行胃胰十二指肠切除术(GPDR)和胰十二指肠切除术(PDR)的儿童胰腺肿瘤患者的治疗经验。这些类型的手术干预既适用于胰腺头部的局部肿瘤，也适用于肿瘤扩散到十二指肠、胃远端和胰腺旁组织的肿瘤。采用的材料和方法:2010-2022年对5 ~ 16岁儿童进行了13例GPDR、PDR手术干预。尽管一些患者在术后早期和后期出现并发症，但这些患者的治疗结果令人满意。所有病人都还活着。GPDR手术干预6例，PDR手术干预7例。最常见的是胰头实性假乳头状瘤(10例)、神经内分泌瘤(2例)和副神经节瘤(1例)。讨论与结论:术后早期并发症以脾动脉胰支出血和急性胰腺炎为主。术后后期出现酶逃避和细菌过度生长综合征反复出现。尽管出现了并发症，但所有患者都存活，没有疾病复发的迹象。这类患者经根治性手术干预后预后良好。
{"title":"Possibility for gastro-pancreatoduodenal and pancreatoduodenal surgical resections in children with pancreatic tumors","authors":"D. V. Rybakova, P. A. Kerimov, D. Podluzhny, Y. Patyutko, M. Rubansky, A. S. Temnyy, E. A. Petrash, S. Varfolomeeva, I. Stilidi","doi":"10.24110/0031-403x-2023-102-3-65-70","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-3-65-70","url":null,"abstract":"The article is about the experience in treating of pediatric patients with tumors of the pancreas who have undergone the gastro-pancreatoduodenal resection (GPDR) and pancreatoduodenal resection (PDR) at the Research Institute of Pediatric Oncology and Hematology named after Academician L.A. Durnov with the N.N. Blokhin Russian Cancer Research Center (Moscow, Russia). These kinds of surgical interventions are performed both with local neoplasms of the pancreatic head and with the spread of the tumor process to the duodenum, distal part of the stomach, and parapancreatic tissue. Materials and methods used: 13 GPDR, PDR surgical interventions were performed in children aged 5 to 16 y/o in 2010-2022. The results of treatment of these patients were assessed as satisfactory despite the complications that arose in some patients in the early and late postoperative periods. All patients are alive. GPDR surgical interventions were performed in 6 patients, and PDR in 7. The most frequently verified were solid pseudopapillary tumor of the pancreatic head (in 10 cases), neuroendocrine tumor (in 2 cases) and paraganglioma in a single case. Discussion and conclusions: bleeding from the pancreatic branch of the splenic artery and acute pancreatitis were noted among the early postoperative complications. Recurrent phenomenon of enzyme evasion and syndrome of excessive bacterial growth were revealed in the late postoperative period. Despite the complications arisen all patients are alive without signs of recurrence of the disease. The prognosis for this category of patients with radically performed surgical intervention is favorable.","PeriodicalId":39654,"journal":{"name":"Pediatriya - Zhurnal im G.N. Speranskogo","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"89160957","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}
Pub Date : 2023-06-01DOI: 10.24110/0031-403x-2023-102-3-183-188
E.O. Bezdolnova, M.S. Kubirov, A.A. Kirillov, V.V. Gorev, E.V. Kumirova
Juvenile granulosa cell tumor of the ovaries is a rare disease in pediatric oncology. Mostly seen in girls and young women in premenarchal age (median age 10 y/o) and is more aggressive than the adult type, but has a favorable prognosis, especially in its early stages. Large reviews and studies include patients with bot adult and juvenile types granulosa cell tumor. Due to the limited cohort of pediatric patients, there are no standards for the treatment of juvenile granulosa cell tumor in pediatric practice. Therefore, diagnosis, treatment, and long-term follow-up for this disease are based on recommendations for an adult cohort. Adjuvant chemotherapy as a curative option is used in advanced stages above the FIGO IC. For the IC stage, the issue of prescribing adjuvant chemotherapy remains debatable. Article represents analysis of the bibliographical data of the largest studies on the treatment of this disease as well as clinical case of a 15 y/o patient with juvenile granulosa cell tumor of the right ovary who had received complex treatment in the Oncological Department of the Morozov Children’s City Clinical Hospital (Moscow, Russia).
{"title":"JUVENILE GRANULOSA CELL TUMOR OF THE OVARY IN A CHILD: CLINICAL CASE AND BIBLIOGRAPHICAL REVIEW","authors":"E.O. Bezdolnova, M.S. Kubirov, A.A. Kirillov, V.V. Gorev, E.V. Kumirova","doi":"10.24110/0031-403x-2023-102-3-183-188","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-3-183-188","url":null,"abstract":"Juvenile granulosa cell tumor of the ovaries is a rare disease in pediatric oncology. Mostly seen in girls and young women in premenarchal age (median age 10 y/o) and is more aggressive than the adult type, but has a favorable prognosis, especially in its early stages. Large reviews and studies include patients with bot adult and juvenile types granulosa cell tumor. Due to the limited cohort of pediatric patients, there are no standards for the treatment of juvenile granulosa cell tumor in pediatric practice. Therefore, diagnosis, treatment, and long-term follow-up for this disease are based on recommendations for an adult cohort. Adjuvant chemotherapy as a curative option is used in advanced stages above the FIGO IC. For the IC stage, the issue of prescribing adjuvant chemotherapy remains debatable. Article represents analysis of the bibliographical data of the largest studies on the treatment of this disease as well as clinical case of a 15 y/o patient with juvenile granulosa cell tumor of the right ovary who had received complex treatment in the Oncological Department of the Morozov Children’s City Clinical Hospital (Moscow, Russia).","PeriodicalId":39654,"journal":{"name":"Pediatriya - Zhurnal im G.N. Speranskogo","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135626031","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}
Pub Date : 2023-06-01DOI: 10.24110/0031-403x-2023-102-3-33-40
Y. Kozel, D. Yurchenko, E. Ulyanova, A. Sagakyants, G. Mkrtchyan, E. E. Pak, V. V. Dmitrieva, M. V. Starzhetskaya, O. V. Kozyuk, A. I. Bespalova, O. P. Popovyan, V. Kovalenko, L. B. Kushtova
Despite processes of epithelial-mesenchymal transition (EMT) that underlie the development of malignant tumors are described widely, the mesenchymal-epithelial transition (MET), which in its turn promotes the metastasis of various tumors, including Ewing's sarcoma (ES), had been studied very little as yet. Within the framework of pediatric oncopathology, ES is one of the most aggressive and metastatic tumors of bones and soft tissues. The purpose of this research was to analyze the features of the expression of markers of the metastatic phenotype of tumor cells MMP2, MMP9 and FN1 in the tissue of the primary tumor in children and adolescents with various forms of ES prevalence. Materials and methods used: 67 patients with localized (n=26) and generalized (n=41) forms of ES aged 0 to 18 y/o, who have been treated at the Pediatric Oncology Department of the National Medical Research Center for Oncology of the Ministry of Healthcare of Russia (Rostov-on-Don, Russia) in Jan. 2009-Dec. 2019. MMP2, MMP9 and FN1 markers were studied by immunohistochemical method in the primary tumor tissue obtained at the stage of process verification and radical surgical treatment after multi-course polychemotherapy (PCT). Results: MMP2 expression in groups with localized and generalized ES before treatment was 4 (p=0.018) and 4.4 (p=0.001) times higher compared to those after treatment. MMP9 expression in the group with generalized ES before treatment prevailed 1.5 times (p=0.020) in comparison with the group after treatment. The median FN1 expression, on the contrary, was 1.2 times lower in the group with generalized ES before treatment than after (p=0.799). Conclusions: determining the expression level of the MMP2 marker in the primary tumor of ES patients can be used to predict the course of the disease and to evaluate the effect of treatment. Whilst markers MMP9 and FN1 require further expanded research.
{"title":"ASSESSMENT OF THE EXPRESSION OF MMР-2, MMР-9 AND FIBRONECTIN MARKERS AS PROGRESSION PREDICTORS IN THE PRIMARY TUMOR TISSUE OF LOCALIZED AND GENERALIZED FORMS OF EWING’S SARCOMA IN CHILDREN AND ADOLESCENTS","authors":"Y. Kozel, D. Yurchenko, E. Ulyanova, A. Sagakyants, G. Mkrtchyan, E. E. Pak, V. V. Dmitrieva, M. V. Starzhetskaya, O. V. Kozyuk, A. I. Bespalova, O. P. Popovyan, V. Kovalenko, L. B. Kushtova","doi":"10.24110/0031-403x-2023-102-3-33-40","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-3-33-40","url":null,"abstract":"Despite processes of epithelial-mesenchymal transition (EMT) that underlie the development of malignant tumors are described widely, the mesenchymal-epithelial transition (MET), which in its turn promotes the metastasis of various tumors, including Ewing's sarcoma (ES), had been studied very little as yet. Within the framework of pediatric oncopathology, ES is one of the most aggressive and metastatic tumors of bones and soft tissues. The purpose of this research was to analyze the features of the expression of markers of the metastatic phenotype of tumor cells MMP2, MMP9 and FN1 in the tissue of the primary tumor in children and adolescents with various forms of ES prevalence. Materials and methods used: 67 patients with localized (n=26) and generalized (n=41) forms of ES aged 0 to 18 y/o, who have been treated at the Pediatric Oncology Department of the National Medical Research Center for Oncology of the Ministry of Healthcare of Russia (Rostov-on-Don, Russia) in Jan. 2009-Dec. 2019. MMP2, MMP9 and FN1 markers were studied by immunohistochemical method in the primary tumor tissue obtained at the stage of process verification and radical surgical treatment after multi-course polychemotherapy (PCT). Results: MMP2 expression in groups with localized and generalized ES before treatment was 4 (p=0.018) and 4.4 (p=0.001) times higher compared to those after treatment. MMP9 expression in the group with generalized ES before treatment prevailed 1.5 times (p=0.020) in comparison with the group after treatment. The median FN1 expression, on the contrary, was 1.2 times lower in the group with generalized ES before treatment than after (p=0.799). Conclusions: determining the expression level of the MMP2 marker in the primary tumor of ES patients can be used to predict the course of the disease and to evaluate the effect of treatment. Whilst markers MMP9 and FN1 require further expanded research.","PeriodicalId":39654,"journal":{"name":"Pediatriya - Zhurnal im G.N. Speranskogo","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"90209172","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}
Pub Date : 2023-06-01DOI: 10.24110/0031-403x-2023-102-3-145-150
P. Svirin, L. Larina, I.N. Lavrentyevа
Thromboses in children represent the serious pathology that can lead to disability and even death of a pediatric patient. At certain localizations of thrombosis, the most rapid restoration of vascular patency (reperfusion) becomes prognostically significant. The most accessible method for reperfusion is drug therapy, in which a drug is injected into the patient's blood, which in its turn significantly accelerates the process of fibrinolysis, i.e., thrombolytic therapy (TT). The purpose of this bibliographical review was to familiarize physicians, especially pediatricians with the place of TT in modern clinical practice. The article represents bibliographical review of published works for over 50 years (since 1966). Authors have used both domestic Russian resources and the PUBMED database. Articles were selected based on the relevance. Statistical analysis was not performed. A review and analysis of bibliographical data on the modern use of TT in pediatric practice is presented. Preference was given to the most modern publications containing analytical information with full text available both pro bono and online. Authors emphasize pediatric practitioners’ attention on the fact that TT at the current level of knowledge is a fairly effective and predictable method for rapid reperfusion.
{"title":"THROMBOLYTIC THERAPY IN PEDIATRIC PRACTICE (PART 1)","authors":"P. Svirin, L. Larina, I.N. Lavrentyevа","doi":"10.24110/0031-403x-2023-102-3-145-150","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-3-145-150","url":null,"abstract":"Thromboses in children represent the serious pathology that can lead to disability and even death of a pediatric patient. At certain localizations of thrombosis, the most rapid restoration of vascular patency (reperfusion) becomes prognostically significant. The most accessible method for reperfusion is drug therapy, in which a drug is injected into the patient's blood, which in its turn significantly accelerates the process of fibrinolysis, i.e., thrombolytic therapy (TT). The purpose of this bibliographical review was to familiarize physicians, especially pediatricians with the place of TT in modern clinical practice. The article represents bibliographical review of published works for over 50 years (since 1966). Authors have used both domestic Russian resources and the PUBMED database. Articles were selected based on the relevance. Statistical analysis was not performed. A review and analysis of bibliographical data on the modern use of TT in pediatric practice is presented. Preference was given to the most modern publications containing analytical information with full text available both pro bono and online. Authors emphasize pediatric practitioners’ attention on the fact that TT at the current level of knowledge is a fairly effective and predictable method for rapid reperfusion.","PeriodicalId":39654,"journal":{"name":"Pediatriya - Zhurnal im G.N. Speranskogo","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"85307519","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}
Pub Date : 2023-06-01DOI: 10.24110/0031-403x-2023-102-3-194-201
A.S. Klyut, T. Valiev, T. Belysheva, O. Malikhova, T. Nasedkina, O. Gusarova, K. Kirgizov, S. Varfolomeeva
Reactive blood changes are a heterogeneous group of pathological conditions that can occur in response to various conditions: viral, bacterial infections, helminth toxins, allergic agents. These reactions often require differential diagnosis with malignant neoplasms (MN) of the blood and hematopoietic organs (leukemias, lymphomas). It is important to note that only with a comprehensive examination of the patient with the involvement of a pediatric oncologist and hematologist, it is possible to exclude MN and carry out etiotropic treatment that contributes to the normalization of hemogram parameters. Article represents current data on the etiopathogenesis of leukemoid reactions, reactive thrombocytosis, secondary erythrocytosis and a clinical case observation of the development of a pseudoblast leukemoid reaction and reactive thrombocytosis against the background of an infectious process in a patient with Peutz-Jeghers syndrome, which will be of use to a wide range of pediatric practitioners.
{"title":"Reactive blood changes in the practice of pediatric physician, oncologist and hematologist: bibliographical review and clinical case","authors":"A.S. Klyut, T. Valiev, T. Belysheva, O. Malikhova, T. Nasedkina, O. Gusarova, K. Kirgizov, S. Varfolomeeva","doi":"10.24110/0031-403x-2023-102-3-194-201","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-3-194-201","url":null,"abstract":"Reactive blood changes are a heterogeneous group of pathological conditions that can occur in response to various conditions: viral, bacterial infections, helminth toxins, allergic agents. These reactions often require differential diagnosis with malignant neoplasms (MN) of the blood and hematopoietic organs (leukemias, lymphomas). It is important to note that only with a comprehensive examination of the patient with the involvement of a pediatric oncologist and hematologist, it is possible to exclude MN and carry out etiotropic treatment that contributes to the normalization of hemogram parameters. Article represents current data on the etiopathogenesis of leukemoid reactions, reactive thrombocytosis, secondary erythrocytosis and a clinical case observation of the development of a pseudoblast leukemoid reaction and reactive thrombocytosis against the background of an infectious process in a patient with Peutz-Jeghers syndrome, which will be of use to a wide range of pediatric practitioners.","PeriodicalId":39654,"journal":{"name":"Pediatriya - Zhurnal im G.N. Speranskogo","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"75140039","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}
Pub Date : 2023-06-01DOI: 10.24110/0031-403x-2023-102-3-107-114
Ya. A. Erdomaeva, K. Kirgizov, S. Kogan, G. B. Sagoyan, T. Valiev, V. Polyakov, S. Varfolomeeva
Late diagnostics of oncological diseases (OD) in children accounts for significant deaths in pediatric oncology. As a result of it the overall survival rate is decreasing and the level of complications is increasing. There are no effective screening programs for malignant neoplasms in children as yet. Programs intent to spread the early OD diagnostics require significant efforts in the fields of increasing of the oncological awareness among practitioners and optimization of routing. The Article provides up-to-date information on the importance of early diagnostics in pediatric oncology, various international campaigns to improve the early OD diagnostics and their effectiveness. The information about existing programs to improve the early OD diagnostics in children in Russia is provided as well.
{"title":"Importance of the early diagnostics in pediatric oncology and hematology","authors":"Ya. A. Erdomaeva, K. Kirgizov, S. Kogan, G. B. Sagoyan, T. Valiev, V. Polyakov, S. Varfolomeeva","doi":"10.24110/0031-403x-2023-102-3-107-114","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-3-107-114","url":null,"abstract":"Late diagnostics of oncological diseases (OD) in children accounts for significant deaths in pediatric oncology. As a result of it the overall survival rate is decreasing and the level of complications is increasing. There are no effective screening programs for malignant neoplasms in children as yet. Programs intent to spread the early OD diagnostics require significant efforts in the fields of increasing of the oncological awareness among practitioners and optimization of routing. The Article provides up-to-date information on the importance of early diagnostics in pediatric oncology, various international campaigns to improve the early OD diagnostics and their effectiveness. The information about existing programs to improve the early OD diagnostics in children in Russia is provided as well.","PeriodicalId":39654,"journal":{"name":"Pediatriya - Zhurnal im G.N. Speranskogo","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"78826163","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}
Pub Date : 2023-06-01DOI: 10.24110/0031-403x-2023-102-3-158-166
I. N. Lupan, A.Y. Pishchalnikov, A. Volyanskiy, Marina Zakharova, L. V. Glukhova, O.M. Ulanova, D. S. Vasilkova, O. A. Levashova, V. Turchina, M. V. Vinogradova, I.A. Khannanov
Atypical hemolytic-uremic syndrome (aHUS) is a rare systemic life-threatening disorder with an unfavorable prognosis and progressive course and is based on chronic uncontrolled overactivation of the alternative complement pathway of hereditary (gene mutations with loss of function of complement regulators/activators) or acquired nature (antibodies to factor H), which leads to the development of a systemic complement-mediated form of thrombotic microangiopathy (TMA). The pathomorphological essence of TMA is the development of endotheliosis, which in its turn triggers the activation of the coagulation cascade with the formation of platelet-fibrin thrombi, partially or completely occluding the lumen of the vessels of the microvasculature (small arteries and arterioles). The therapeutic approach to aHUS has been improved with the introduction of eculizumab, a humanized monoclonal antibody, into clinical practice. Eculizumab has a high affinity for the C5 component of complement and, by binding to it, completely blocks the splitting of C5 into C5a and C5b. As a result, the formation of pro-inflammatory cytokines is inhibited by blocking the formation of C5a and the membrane attack complex (MAC) by blocking the formation of C5b. This therapy improves the prognosis of the disease and reduces the risk for development of the life-threatening conditions, including end-stage chronic kidney disease (CKD). The Article represents the analysis of the Authors’ own clinical experience with aHUS in the region of the Chelyabinsk Oblast of Russia. The diagnosis of aHUS was based on a combination of microangiopathic non-immune hemolytic anemia, thrombocytopenia, and acute kidney injury, after excluding other forms of TMA. Most often, aHUS in the form of clinical manifestations of TMA was diagnosed in pediatric patients during the first 3 years of life (67%). Both pathogenic and possibly-pathogenic mutations associated with the development of aHUS were detected in 56% of cases. These included mutations in the CFH, CFI, C3 genes, heterozygous deletion of CFHR1/CFHR3. Eculizumab had demonstrated its high efficiency accompanied by the restoration of diuresis, a decrease in the severity of arterial hypertension, a rapid suppression of hemolysis activity and the signs of active TMA. Throughout the course of treatment, all patients maintained hematological remission coupled with the absence of necessity for continuing extracorporeal therapy. Conclusion: the onset of remission of aHUS against the background of complement blocking therapy with eculizumab confirmed the correctness of the established diagnosis and the chosen treatment tactics.
{"title":"Overview of the regional experience in the diagnostics and treatment of atypical-hemolytic uremic syndrome in the Chelyabinsk Oblast of Russia","authors":"I. N. Lupan, A.Y. Pishchalnikov, A. Volyanskiy, Marina Zakharova, L. V. Glukhova, O.M. Ulanova, D. S. Vasilkova, O. A. Levashova, V. Turchina, M. V. Vinogradova, I.A. Khannanov","doi":"10.24110/0031-403x-2023-102-3-158-166","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-3-158-166","url":null,"abstract":"Atypical hemolytic-uremic syndrome (aHUS) is a rare systemic life-threatening disorder with an unfavorable prognosis and progressive course and is based on chronic uncontrolled overactivation of the alternative complement pathway of hereditary (gene mutations with loss of function of complement regulators/activators) or acquired nature (antibodies to factor H), which leads to the development of a systemic complement-mediated form of thrombotic microangiopathy (TMA). The pathomorphological essence of TMA is the development of endotheliosis, which in its turn triggers the activation of the coagulation cascade with the formation of platelet-fibrin thrombi, partially or completely occluding the lumen of the vessels of the microvasculature (small arteries and arterioles). The therapeutic approach to aHUS has been improved with the introduction of eculizumab, a humanized monoclonal antibody, into clinical practice. Eculizumab has a high affinity for the C5 component of complement and, by binding to it, completely blocks the splitting of C5 into C5a and C5b. As a result, the formation of pro-inflammatory cytokines is inhibited by blocking the formation of C5a and the membrane attack complex (MAC) by blocking the formation of C5b. This therapy improves the prognosis of the disease and reduces the risk for development of the life-threatening conditions, including end-stage chronic kidney disease (CKD). The Article represents the analysis of the Authors’ own clinical experience with aHUS in the region of the Chelyabinsk Oblast of Russia. The diagnosis of aHUS was based on a combination of microangiopathic non-immune hemolytic anemia, thrombocytopenia, and acute kidney injury, after excluding other forms of TMA. Most often, aHUS in the form of clinical manifestations of TMA was diagnosed in pediatric patients during the first 3 years of life (67%). Both pathogenic and possibly-pathogenic mutations associated with the development of aHUS were detected in 56% of cases. These included mutations in the CFH, CFI, C3 genes, heterozygous deletion of CFHR1/CFHR3. Eculizumab had demonstrated its high efficiency accompanied by the restoration of diuresis, a decrease in the severity of arterial hypertension, a rapid suppression of hemolysis activity and the signs of active TMA. Throughout the course of treatment, all patients maintained hematological remission coupled with the absence of necessity for continuing extracorporeal therapy. Conclusion: the onset of remission of aHUS against the background of complement blocking therapy with eculizumab confirmed the correctness of the established diagnosis and the chosen treatment tactics.","PeriodicalId":39654,"journal":{"name":"Pediatriya - Zhurnal im G.N. Speranskogo","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"74185041","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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