Pub Date : 2023-07-27DOI: 10.46563/1560-9561-2023-26-3-187-193
E. Sibirskaya, I. Karachentsova, S. Sharkov, I.A. Melenchuk, Tamara N. Ivanovskaya, Y. Kirillova, Aina S. Annakilieva, L. Pivazyan, Eva D. Nakhapetyan
Background. Vulvar lichen sclerosus (VLS) is a chronic inflammatory dermatosis, which is characterized by focal skin atrophy of the vulva and mucous membranes. It leads to deformation of the perineum. The prevalence of VLS in children is up to 0.1%, among the adults — 3%. The average age of the first symptoms appearance in girls is 5.4 years, in women — 55.1 years. The diagnosis of VLS is made on the base of anamnesis, assessment of pubertal development, physical examination of the external genitalia, advanced vulvoscopy and vaginoscopy, bacteriological, bacterioscopic and cytological studies of vaginal wet mount, assessment of the hormonal profile. The treatment methods include high-potency steroids as the initial treatment, usage of diode, fractional CO2 and erbium lasers with different wavelengths and injections of platelet-rich plasma to improve the regeneration processes of the affected tissue by improving collagenogenesis, vasculogenesis, production of proteoglycans and glycoproteins. The aim of the study is to compare the effectiveness of photodestruction with a manual diode laser of the Dornier Medilas D LiteBeam, wavelength of 940 nm, minimum power in pulsed mode on the girls’ vulva mucosa with various types of VLS during neutral and preadolescent age. To obtain the results there were analyzed criteria including the size of the lesion after photodestruction therapy, the disappearance of cracks and angiomatosis in the LS area. The objectives of the complex therapy of VLS in girls were elimination of itching, decrease of the inflammatory changes, improvement of the skin and mucosa blood supply, and stimulation of local immunological mechanisms. Materials and methods. The study was conducted on the base of the gynecological department of Russian Children’s Clinical Hospital. The effectiveness of the laser-induced thermotherapy with a wavelength of 940 nm, minimum power in pulsed mode on the vulva of VLS girls of neutral and preadolescent periods as a part of the complex therapy was evaluated. Ninety 5 to 13 year VLS patients took part in the trial. They were divided into 3 groups. The first group included 30 patients (average age 7.5 ± 1.1) with atrophic form of lichen treated with a laser with a wavelength of 940 nm. The second group consisted of 30 patients (average age 6.8 ± 1.3) with erythematous-edematous lichen, who were treated with a laser with a wavelength of 635 nm. The third group included 30 patients (average age 7.3 ± 1.2) with erosive and ulcerative form of VLS, who received high-potency corticosteroids therapy. Results. The treatment results in the three subgroups of VLS patients show a diode laser with a wavelength of 940 nm therapy to be the most effective. After the first treatment course 27 (90%) patients in the first group showed a positive trend with a decrease in itching and burning, pink-color presence in vulva mucousа, a decrease of the skin pigmentation area and an increase in the elasticity of the skin. After 2
背景。外阴硬化苔藓(VLS)是一种慢性炎症性皮肤病，其特征是外阴和粘膜局灶性皮肤萎缩。它会导致会阴变形。VLS在儿童中的患病率为0.1%，在成人中为3%。女孩首次出现症状的平均年龄为5.4岁，妇女为55.1岁。VLS的诊断是在记忆、青春期发育评估、外生殖器体格检查、高级外阴镜和阴道镜检查、阴道湿疣的细菌学、细菌学和细胞学研究、激素谱评估的基础上做出的。治疗方法包括高效类固醇作为初始治疗，使用二极管、不同波长的CO2和铒激光器，以及注射富含血小板的血浆，通过改善胶原生成、血管生成、蛋白聚糖和糖蛋白的产生来改善受影响组织的再生过程。本研究的目的是比较波长为940 nm、最小功率为脉冲模式的Dornier Medilas D LiteBeam手动二极管激光器对中性期和青春期前不同类型VLS女孩外阴黏膜的光破坏效果。为了得到结果，我们分析了光破坏治疗后病变的大小、裂缝的消失和LS区域的血管瘤病。女生VLS综合治疗的目的是消除瘙痒，减少炎症变化，改善皮肤和粘膜血供，刺激局部免疫机制。材料和方法。本研究以俄罗斯儿童临床医院妇科为基础进行。以940 nm波长、最小功率的脉冲激光诱导热疗作为综合治疗的一部分，对中性期和青春期前的VLS女生外阴进行了疗效评价。95至13岁的VLS患者参加了试验。他们被分成3组。第一组采用940nm波长激光治疗萎缩性地衣患者30例(平均年龄7.5±1.1岁)。第二组患者30例，平均年龄6.8±1.3岁，采用波长为635 nm的激光治疗。第三组包括30例(平均年龄7.3±1.2岁)糜烂和溃疡型VLS患者，接受高效糖皮质激素治疗。结果。三组VLS患者的治疗结果显示，波长为940 nm的二极管激光治疗最有效。第一个疗程结束后，第一组27例(90%)患者瘙痒、灼烧症状减轻，外阴黏膜呈粉红色，皮肤色素沉着面积减少，皮肤弹性增加，呈阳性趋势。2个疗程后，阳性趋势29例(96.7%)。第二种最有效的治疗方法是635纳米波长激光的光破坏。经过4个疗程(两次住院)，25例(83.3%)患者注意到瘙痒和“紧绷”感减轻。24例(80%)患者的外阴粘膜状况得到改善。23例(76.7%)患者在末次疗程后3周复查时水肿减轻。然而，在本组患者中，不能完全恢复外阴粘膜。最无效的药物治疗是使用高效类固醇。8例(26.7%)患者仅在第4天治疗时出现积极动态，11例(36.7%)患者在首次使用软膏后3周时出现积极动态，12例(40%)患者在第2个月时出现积极动态。5例(16.7%)患者粘膜的可见状况没有改变，出血性溃疡的数量减少最小。结论。试验结果表明，激光治疗儿童VLS的效率高于局部糖皮质激素治疗。治疗的持续时间和治疗后外生殖器的状况取决于VLS的组织学形式和分期。根据我们的研究，激光治疗有助于不同类型VLS的儿童患者在光破坏手术后尽可能短的时间内缓解发作。
{"title":"Optimization of treatment of vulvar scleroatrophic lichen with using modern laser technologies","authors":"E. Sibirskaya, I. Karachentsova, S. Sharkov, I.A. Melenchuk, Tamara N. Ivanovskaya, Y. Kirillova, Aina S. Annakilieva, L. Pivazyan, Eva D. Nakhapetyan","doi":"10.46563/1560-9561-2023-26-3-187-193","DOIUrl":"https://doi.org/10.46563/1560-9561-2023-26-3-187-193","url":null,"abstract":"Background. Vulvar lichen sclerosus (VLS) is a chronic inflammatory dermatosis, which is characterized by focal skin atrophy of the vulva and mucous membranes. It leads to deformation of the perineum. The prevalence of VLS in children is up to 0.1%, among the adults — 3%. The average age of the first symptoms appearance in girls is 5.4 years, in women — 55.1 years. The diagnosis of VLS is made on the base of anamnesis, assessment of pubertal development, physical examination of the external genitalia, advanced vulvoscopy and vaginoscopy, bacteriological, bacterioscopic and cytological studies of vaginal wet mount, assessment of the hormonal profile. The treatment methods include high-potency steroids as the initial treatment, usage of diode, fractional CO2 and erbium lasers with different wavelengths and injections of platelet-rich plasma to improve the regeneration processes of the affected tissue by improving collagenogenesis, vasculogenesis, production of proteoglycans and glycoproteins. \u0000The aim of the study is to compare the effectiveness of photodestruction with a manual diode laser of the Dornier Medilas D LiteBeam, wavelength of 940 nm, minimum power in pulsed mode on the girls’ vulva mucosa with various types of VLS during neutral and preadolescent age. To obtain the results there were analyzed criteria including the size of the lesion after photodestruction therapy, the disappearance of cracks and angiomatosis in the LS area. The objectives of the complex therapy of VLS in girls were elimination of itching, decrease of the inflammatory changes, improvement of the skin and mucosa blood supply, and stimulation of local immunological mechanisms. \u0000Materials and methods. The study was conducted on the base of the gynecological department of Russian Children’s Clinical Hospital. The effectiveness of the laser-induced thermotherapy with a wavelength of 940 nm, minimum power in pulsed mode on the vulva of VLS girls of neutral and preadolescent periods as a part of the complex therapy was evaluated. Ninety 5 to 13 year VLS patients took part in the trial. They were divided into 3 groups. The first group included 30 patients (average age 7.5 ± 1.1) with atrophic form of lichen treated with a laser with a wavelength of 940 nm. The second group consisted of 30 patients (average age 6.8 ± 1.3) with erythematous-edematous lichen, who were treated with a laser with a wavelength of 635 nm. The third group included 30 patients (average age 7.3 ± 1.2) with erosive and ulcerative form of VLS, who received high-potency corticosteroids therapy. \u0000Results. The treatment results in the three subgroups of VLS patients show a diode laser with a wavelength of 940 nm therapy to be the most effective. After the first treatment course 27 (90%) patients in the first group showed a positive trend with a decrease in itching and burning, pink-color presence in vulva mucousа, a decrease of the skin pigmentation area and an increase in the elasticity of the skin. After 2 ","PeriodicalId":52396,"journal":{"name":"Russian Journal of Pediatric Hematology and Oncology","volume":"18 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-07-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"73007355","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 : 2023-07-19DOI: 10.21682/2311-1267-2023-10-2-71-76
M. Ryzhova, L. V. Olkhova, L. Shishkina, A. Golanov, O. Zheludkova, E. Kumirova, A. Kislyakov, D. Skobeev, M. Belogurova, Y. Dinikina, Y. Zabrodskaya, V. A. Grigorenko, A. Druy, L. Papusha
Currently, the daily routine of a molecular pathologist is DNA methylation and RNA and DNA sequencing. The authors, from the standpoint of researchers and clinicians, explain the molecular methods used not only to diagnose brain tumors, but also to search for possible targets for therapy.
{"title":"The principles of therapy for modern diagnosis of brain tumors in children, which determine the prescription of the drugs","authors":"M. Ryzhova, L. V. Olkhova, L. Shishkina, A. Golanov, O. Zheludkova, E. Kumirova, A. Kislyakov, D. Skobeev, M. Belogurova, Y. Dinikina, Y. Zabrodskaya, V. A. Grigorenko, A. Druy, L. Papusha","doi":"10.21682/2311-1267-2023-10-2-71-76","DOIUrl":"https://doi.org/10.21682/2311-1267-2023-10-2-71-76","url":null,"abstract":"Currently, the daily routine of a molecular pathologist is DNA methylation and RNA and DNA sequencing. The authors, from the standpoint of researchers and clinicians, explain the molecular methods used not only to diagnose brain tumors, but also to search for possible targets for therapy.","PeriodicalId":52396,"journal":{"name":"Russian Journal of Pediatric Hematology and Oncology","volume":"7 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-07-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"77470032","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 : 2023-07-19DOI: 10.21682/2311-1267-2023-10-2-117-123
G. B. Sagoyan, N. Zhukov, V. Strelnikov, R. Khagurov, A. M. Suleymanova, Yu. M. Mareeva, R. V. Garbuzov, E. Imyanitov, Y. Dinikina, S. Kutsev, E. Donyush, K. Kirgizov, N. Semenova, Y. Polyaev, I. Kletskaya, A. Maschan, S. Varfolomeeva
PROS (PIK3CA-Related Overgrowth Spectrum) encompasses vascular malformations, lipomatosis and other multiple congenital anomalies resulting from activating somatic mutations in the PIK3CA gene. PROS includes macrodactyly, hemimegalencephaly, muscle hemihypertrophy, facial infiltrating lipomatosis CLOVES, megalencephaly, vascular malformations (capillary, venous, lymphatic, arteriovenous and combined vascular malformations), skin disorders, epidermal nevi, etc.The experts of the Russian Society of Pediatric Oncologists and Hematologists, Russian Association of Pediatric Surgeons and Russian Society of Medical Geneticists developed this consensus statement of diagnostics and treatment of PROS.
{"title":"Consensus on the diagnosis and treatment of PROS (PIK3CA-related overgrowth spectrum). Russian version","authors":"G. B. Sagoyan, N. Zhukov, V. Strelnikov, R. Khagurov, A. M. Suleymanova, Yu. M. Mareeva, R. V. Garbuzov, E. Imyanitov, Y. Dinikina, S. Kutsev, E. Donyush, K. Kirgizov, N. Semenova, Y. Polyaev, I. Kletskaya, A. Maschan, S. Varfolomeeva","doi":"10.21682/2311-1267-2023-10-2-117-123","DOIUrl":"https://doi.org/10.21682/2311-1267-2023-10-2-117-123","url":null,"abstract":"PROS (PIK3CA-Related Overgrowth Spectrum) encompasses vascular malformations, lipomatosis and other multiple congenital anomalies resulting from activating somatic mutations in the PIK3CA gene. PROS includes macrodactyly, hemimegalencephaly, muscle hemihypertrophy, facial infiltrating lipomatosis CLOVES, megalencephaly, vascular malformations (capillary, venous, lymphatic, arteriovenous and combined vascular malformations), skin disorders, epidermal nevi, etc.The experts of the Russian Society of Pediatric Oncologists and Hematologists, Russian Association of Pediatric Surgeons and Russian Society of Medical Geneticists developed this consensus statement of diagnostics and treatment of PROS.","PeriodicalId":52396,"journal":{"name":"Russian Journal of Pediatric Hematology and Oncology","volume":"48 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-07-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"76346336","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 : 2023-07-19DOI: 10.21682/2311-1267-2023-10-2-99-106
V. Lozovaya, O. Gusarova, O. Malikhova, N. Besova, A. M. Suleymanova, Yu.V. Sinyagina, A. Tumanyan
Malignant neoplasms (MNP) of the gastrointestinal tract in children are extremely rare and account for 1.2 % of all MNP. According to world literature, the incidence of gastric cancer is no more than 0.05 % of all cases of MNP of the gastrointestinal tract in children. In national and foreign literature, only isolated cases of stomach cancer in children and adolescents aged 10 to 18 years are described. Clinical manifestations of the disease, as in patients over the age of 18, are non-specific – epigastric pain, loss of appetite, weight loss, ascites, anemia, melena and others. In this regard, the diagnosis occurs at the late stages of the disease and, therefore, is characterized by an unfavorable prognosis. Gastric signet ring cell carcinoma (SRCC) is an uncommon histologic subtype of adenocarcinoma and usually occurs in people aged 20 to 40 years. We would like to report an extremely rare case of SRCC in a 14-year-old child.
{"title":"A clinical case of signet ring cell carcinoma of stomach in a child. Endoscopic diagnostics","authors":"V. Lozovaya, O. Gusarova, O. Malikhova, N. Besova, A. M. Suleymanova, Yu.V. Sinyagina, A. Tumanyan","doi":"10.21682/2311-1267-2023-10-2-99-106","DOIUrl":"https://doi.org/10.21682/2311-1267-2023-10-2-99-106","url":null,"abstract":"Malignant neoplasms (MNP) of the gastrointestinal tract in children are extremely rare and account for 1.2 % of all MNP. According to world literature, the incidence of gastric cancer is no more than 0.05 % of all cases of MNP of the gastrointestinal tract in children. In national and foreign literature, only isolated cases of stomach cancer in children and adolescents aged 10 to 18 years are described. Clinical manifestations of the disease, as in patients over the age of 18, are non-specific – epigastric pain, loss of appetite, weight loss, ascites, anemia, melena and others. In this regard, the diagnosis occurs at the late stages of the disease and, therefore, is characterized by an unfavorable prognosis. Gastric signet ring cell carcinoma (SRCC) is an uncommon histologic subtype of adenocarcinoma and usually occurs in people aged 20 to 40 years. We would like to report an extremely rare case of SRCC in a 14-year-old child.","PeriodicalId":52396,"journal":{"name":"Russian Journal of Pediatric Hematology and Oncology","volume":"42 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-07-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"86771839","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 : 2023-07-19DOI: 10.21682/2311-1267-2023-10-2-77-91
N. Ivanov, D. Kachanov, T. Shamanskaya
Neuroblastoma (NB) is the most common extracranial solid tumor of childhood affecting children from 0 to 14 years old. Despite the achievements of modern multimodal risk-adapted therapy, the prognosis in patients with high-risk NB remains unfavorable. Numerous research groups have shown that a good response achieved at the time of completion of the induction stage of therapy in this subgroup of patients correlates with survival rates. Thus, improving the response to induction therapy may be a potential mechanism for improving long-term survival rates. Over the past few decades, traditional approaches to cancer therapy have undergone a radical revolution, largely due to the development and implementation of the immunotherapy method. It is known that combined antitumor therapy is superior to monotherapy and is one of the tools for overcoming heterogeneous drug resistance. A vast number of preclinical studies has shown that GD2-directed monoclonal antibodies (mAbs) are able to enhance the cytostatic effects of chemotherapeutic drugs, which has become a promising model for clinical studies of various chemoimmunotherapy regimens, which have demonstrated convincing evidence of safety and an acceptable toxicity profile with an encouraging effect on objective response rates, overall and event-free survival in both patients with recurrent, refractory NB, and primary patients of the high-risk group.The article discusses fundamental ideas about the synergistic interaction of GD2-directed mAbs in combination with cytostatic agents, the role of response to the induction stage of therapy and prospects for the use of induction chemoimmunotherapy as a method of improving postinduction response, event-free and overall survival in patients with NB.
{"title":"Neuroblastoma chemoimmunotherapy: current results and application prospects","authors":"N. Ivanov, D. Kachanov, T. Shamanskaya","doi":"10.21682/2311-1267-2023-10-2-77-91","DOIUrl":"https://doi.org/10.21682/2311-1267-2023-10-2-77-91","url":null,"abstract":"Neuroblastoma (NB) is the most common extracranial solid tumor of childhood affecting children from 0 to 14 years old. Despite the achievements of modern multimodal risk-adapted therapy, the prognosis in patients with high-risk NB remains unfavorable. Numerous research groups have shown that a good response achieved at the time of completion of the induction stage of therapy in this subgroup of patients correlates with survival rates. Thus, improving the response to induction therapy may be a potential mechanism for improving long-term survival rates. Over the past few decades, traditional approaches to cancer therapy have undergone a radical revolution, largely due to the development and implementation of the immunotherapy method. It is known that combined antitumor therapy is superior to monotherapy and is one of the tools for overcoming heterogeneous drug resistance. A vast number of preclinical studies has shown that GD2-directed monoclonal antibodies (mAbs) are able to enhance the cytostatic effects of chemotherapeutic drugs, which has become a promising model for clinical studies of various chemoimmunotherapy regimens, which have demonstrated convincing evidence of safety and an acceptable toxicity profile with an encouraging effect on objective response rates, overall and event-free survival in both patients with recurrent, refractory NB, and primary patients of the high-risk group.The article discusses fundamental ideas about the synergistic interaction of GD2-directed mAbs in combination with cytostatic agents, the role of response to the induction stage of therapy and prospects for the use of induction chemoimmunotherapy as a method of improving postinduction response, event-free and overall survival in patients with NB.","PeriodicalId":52396,"journal":{"name":"Russian Journal of Pediatric Hematology and Oncology","volume":"142 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-07-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"86744671","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 : 2023-07-19DOI: 10.21682/2311-1267-2023-10-2-107-111
A. Editorial
.
．
{"title":"Resolution of the Scientific and Practical Conference with international participation “Targeted and immunotherapy of tumors of the central nervous system in children” (St. Petersburg, March 31, 2023, Almazov National Medical Research Center of the Ministry of Health of Russia)","authors":"A. Editorial","doi":"10.21682/2311-1267-2023-10-2-107-111","DOIUrl":"https://doi.org/10.21682/2311-1267-2023-10-2-107-111","url":null,"abstract":"<jats:p>.</jats:p>","PeriodicalId":52396,"journal":{"name":"Russian Journal of Pediatric Hematology and Oncology","volume":"108 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-07-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"73518922","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 : 2023-07-19DOI: 10.21682/2311-1267-2023-10-2-112-116
A. Editorial
.
．
{"title":"Resolution on the results of the round table “NTRK-positive tumors: diagnosis and treatment”","authors":"A. Editorial","doi":"10.21682/2311-1267-2023-10-2-112-116","DOIUrl":"https://doi.org/10.21682/2311-1267-2023-10-2-112-116","url":null,"abstract":"<jats:p>.</jats:p>","PeriodicalId":52396,"journal":{"name":"Russian Journal of Pediatric Hematology and Oncology","volume":"22 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-07-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"85713404","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 : 2023-07-19DOI: 10.21682/2311-1267-2023-10-2-92-98
M. S. Korneeva, N. Batmanova, T. Valiev, K. Kirgizov
Despite of good outcomes in treatment of patients with newly diagnosed Langerhans cell histiocytosis (LCH), recurrences have been reported in 29.9 % of patients. There are currently no generally accepted standards for the treatment of recurrences and refractory forms of LCH. The prognosis of patients, suffering from this pathology, remains unfavorable. Current treatment approaches of recurrences and refractory forms of LCH include using of BRAF- and MEK-inhibitors and cellular treatment technologies. The article provides a literature review of current approaches to the treatment of recurrences and refractory forms of LCH and identifies the prospects for further research.
{"title":"Modern approaches to the treatment of relapses and refractory forms of Langerhans cell histiocytosis. Literature review","authors":"M. S. Korneeva, N. Batmanova, T. Valiev, K. Kirgizov","doi":"10.21682/2311-1267-2023-10-2-92-98","DOIUrl":"https://doi.org/10.21682/2311-1267-2023-10-2-92-98","url":null,"abstract":"Despite of good outcomes in treatment of patients with newly diagnosed Langerhans cell histiocytosis (LCH), recurrences have been reported in 29.9 % of patients. There are currently no generally accepted standards for the treatment of recurrences and refractory forms of LCH. The prognosis of patients, suffering from this pathology, remains unfavorable. Current treatment approaches of recurrences and refractory forms of LCH include using of BRAF- and MEK-inhibitors and cellular treatment technologies. The article provides a literature review of current approaches to the treatment of recurrences and refractory forms of LCH and identifies the prospects for further research.","PeriodicalId":52396,"journal":{"name":"Russian Journal of Pediatric Hematology and Oncology","volume":"6 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-07-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"79685679","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 : 2023-07-18DOI: 10.21682/2311-1267-2023-10-2-63-70
E. A. Petrash, M. A. Shorikov, E. Mikhailova, T. R. Panferova, A. Nikulina, A. Petrash
Introduction. Liver tumors account for 1.1 % of all newly diagnosed neoplasms in children. The rarity of this pathology causes difficulties in differential diagnosis. Currently, magnetic resonance imaging (MRI) is the main and most promising method for diagnosing liver diseases. In our work, we decided to quantify the data from this study.Purpose of the study – determination of the possibilities of quantitative assessment of multiparametric MRI data in the differential diagnosis of benign and malignant liver tumors in children.Material and methods. 133 patients with 307 liver lesions aged from 5 months to 20 years were examined. All patients underwent MRI on high-field MRI machines using an extracellular contrast agent, which included T2 weighted images with and without suppression of the signal from adipose tissue, diffusion-weighted images with automatic calculation of maps of the apparent diffusion coefficient (ADC), T1 weighted images with suppression of the signal from adipose tissue before and after the introduction of a contrast agent (in the arterial, portal, venous and delayed phases). Quantitative characteristics of changes in signal intensity in the lesion, intact liver parenchyma, spleen, kidney, aorta, and inferior vena cava (IVC) were obtained. To level the influence of external factors, we used not the absolute values of the signal intensity, but the ratios: lesion/intact liver parenchyma, lesion/kidney, lesion/aorta, lesion/spleen, lesion/IVC. For each lesion, 5 coefficients were calculated in each of the sequences, with the exception of patients (n = 4) after splenectomy, in whom 4 coefficients were calculated. In addition, for images obtained after the injection of a contrast agent, the ratios of the signal on post-contrast images to the native phase were calculated. Quantitative parameters such as the maximum size of the tumor, its volume and the age of the patient were included in the calculation. Tumors were represented by benign (n = 139) and malignant (n = 169) formations. The diagnosis of all malignant neoplasms and some benign ones was confirmed morphologically, benign ones – using MRI with intravenous contrast and dynamic observation.Results. A mathematical model was built:A = 1/(1+e-Z),where Z = 6,25019 + 1,03132 × S + 1,30077 × P2le/li – 0,00459 × DCle + 4,01375 × P1le/a – 2,05533 × Part le/li – 2,55823 × Pport le/k + 7,56980 × Pdel5 le/k – 15,91047 × Pdel5 le/a.The model is informative and statistically significant (p < 0.001). If A > 0.5, it should be considered that the studied focus is of a malignant nature, if A ≤ 0.5, the formation is benign. Model sensitivity and specificity were, respectively, 0.947 and 0.917.Conclusion. The mathematical model makes it possible to differentiate between malignant and benign formations with a high degree of informativeness, which is a priority task in detecting a mass formation in the liver.
{"title":"Differential diagnosis of benign and malignant liver tumors in children using the method of quantitative assessment of multiparametric magnetic resonance imaging data","authors":"E. A. Petrash, M. A. Shorikov, E. Mikhailova, T. R. Panferova, A. Nikulina, A. Petrash","doi":"10.21682/2311-1267-2023-10-2-63-70","DOIUrl":"https://doi.org/10.21682/2311-1267-2023-10-2-63-70","url":null,"abstract":"Introduction. Liver tumors account for 1.1 % of all newly diagnosed neoplasms in children. The rarity of this pathology causes difficulties in differential diagnosis. Currently, magnetic resonance imaging (MRI) is the main and most promising method for diagnosing liver diseases. In our work, we decided to quantify the data from this study.Purpose of the study – determination of the possibilities of quantitative assessment of multiparametric MRI data in the differential diagnosis of benign and malignant liver tumors in children.Material and methods. 133 patients with 307 liver lesions aged from 5 months to 20 years were examined. All patients underwent MRI on high-field MRI machines using an extracellular contrast agent, which included T2 weighted images with and without suppression of the signal from adipose tissue, diffusion-weighted images with automatic calculation of maps of the apparent diffusion coefficient (ADC), T1 weighted images with suppression of the signal from adipose tissue before and after the introduction of a contrast agent (in the arterial, portal, venous and delayed phases). Quantitative characteristics of changes in signal intensity in the lesion, intact liver parenchyma, spleen, kidney, aorta, and inferior vena cava (IVC) were obtained. To level the influence of external factors, we used not the absolute values of the signal intensity, but the ratios: lesion/intact liver parenchyma, lesion/kidney, lesion/aorta, lesion/spleen, lesion/IVC. For each lesion, 5 coefficients were calculated in each of the sequences, with the exception of patients (n = 4) after splenectomy, in whom 4 coefficients were calculated. In addition, for images obtained after the injection of a contrast agent, the ratios of the signal on post-contrast images to the native phase were calculated. Quantitative parameters such as the maximum size of the tumor, its volume and the age of the patient were included in the calculation. Tumors were represented by benign (n = 139) and malignant (n = 169) formations. The diagnosis of all malignant neoplasms and some benign ones was confirmed morphologically, benign ones – using MRI with intravenous contrast and dynamic observation.Results. A mathematical model was built:A = 1/(1+e-Z),where Z = 6,25019 + 1,03132 × S + 1,30077 × P2le/li – 0,00459 × DCle + 4,01375 × P1le/a – 2,05533 × Part le/li – 2,55823 × Pport le/k + 7,56980 × Pdel5 le/k – 15,91047 × Pdel5 le/a.The model is informative and statistically significant (p < 0.001). If A > 0.5, it should be considered that the studied focus is of a malignant nature, if A ≤ 0.5, the formation is benign. Model sensitivity and specificity were, respectively, 0.947 and 0.917.Conclusion. The mathematical model makes it possible to differentiate between malignant and benign formations with a high degree of informativeness, which is a priority task in detecting a mass formation in the liver.","PeriodicalId":52396,"journal":{"name":"Russian Journal of Pediatric Hematology and Oncology","volume":"201 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-07-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"80154428","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 : 2023-07-17DOI: 10.21682/2311-1267-2023-10-2-54-62
V. Merabishvili
Relevance. Malignant tumors among the children’s population is a relatively rare phenomenon. In the USSR, the form of state reporting for the analysis of morbidity provided for the collection of data in the first age group totaling from 0 to 29 years. Indeed, in this age group, malignant tumors are rare 10–15 cases per 100,000 children. Then, after the 35-year-old group, the risk of cancer increases by 10 % every year, and a five-year period gives an increase in the incidence rate by 50–70 %. The probability of malignant tumors in younger age groups differs hundreds of times from those who have reached the age of 70. In accordance with World Health Organization – IARC recommendations, the data in IARC monographs are summarized for 5-year-old age groups 0–4, 5–9, 10–14, 15–19, etc. The last open interval is 85+.Prepared by the staff of the Laboratory of Oncological Statistics and the Children’s Department of the N.N. Petrov National Medical Research Center of Oncology together with Novel LLC, the completed topic of scientific development of the population children’s cancer registry at the federal district level, transmitted and accepted by the Ministry of Health of the Russian Federation, turned out to be unclaimed.In February 2019, we formed a unified database of the Northwestern Federal District with a volume of more than 1 million 350 thousand observations, which made it possible to carry out a more detailed development of data on rare localizations: cancer of the heart, eye, thymus, including rare tumors that occur among children population (0–14 years old) and adolescents (15–17 years old). The purpose of the study is to study the patterns of dynamics of morbidity, mortality, the reliability of accounting for sick children (0–14 years old) at the population level, to calculate the survival rates of patients with all tumor localizations, taking into account gender, age, stage of the disease, histological forms and other parameters included in the registration form card (in accordance with the international standard of the Eurocare program). Results. From 2000 to 2018, 4970 children (0–14 years old) with malignant tumors were registered in the Northwestern Federal District of the Russian Federation, of which 3011 are subject to the staging of the tumor process. It is important to note that among all children of patients with malignant tumors, the five-year survival rate of stageable and non-stageable patients was close to 80 %. There is a high level of staging of patients of this group. Thus, patients with stage I of the disease had a five-year survival rate (2010–2014) – 96.3 %, with II – 91.0 % and only with stage IV – 50.9 %.Conclusions. The creation of a children’s population cancer registry at the federal district level could significantly raise the level of primary registration of malignant tumors, eliminate losses and be able to calculate analytical indicators for rare childhood localization of malignant tumors.
{"title":"The state of oncology care in Russia: children (0–14 years old), morbidity, mortality, year-by-year lethality (populated study at the federal district level). Part I","authors":"V. Merabishvili","doi":"10.21682/2311-1267-2023-10-2-54-62","DOIUrl":"https://doi.org/10.21682/2311-1267-2023-10-2-54-62","url":null,"abstract":"Relevance. Malignant tumors among the children’s population is a relatively rare phenomenon. In the USSR, the form of state reporting for the analysis of morbidity provided for the collection of data in the first age group totaling from 0 to 29 years. Indeed, in this age group, malignant tumors are rare 10–15 cases per 100,000 children. Then, after the 35-year-old group, the risk of cancer increases by 10 % every year, and a five-year period gives an increase in the incidence rate by 50–70 %. The probability of malignant tumors in younger age groups differs hundreds of times from those who have reached the age of 70. In accordance with World Health Organization – IARC recommendations, the data in IARC monographs are summarized for 5-year-old age groups 0–4, 5–9, 10–14, 15–19, etc. The last open interval is 85+.Prepared by the staff of the Laboratory of Oncological Statistics and the Children’s Department of the N.N. Petrov National Medical Research Center of Oncology together with Novel LLC, the completed topic of scientific development of the population children’s cancer registry at the federal district level, transmitted and accepted by the Ministry of Health of the Russian Federation, turned out to be unclaimed.In February 2019, we formed a unified database of the Northwestern Federal District with a volume of more than 1 million 350 thousand observations, which made it possible to carry out a more detailed development of data on rare localizations: cancer of the heart, eye, thymus, including rare tumors that occur among children population (0–14 years old) and adolescents (15–17 years old). The purpose of the study is to study the patterns of dynamics of morbidity, mortality, the reliability of accounting for sick children (0–14 years old) at the population level, to calculate the survival rates of patients with all tumor localizations, taking into account gender, age, stage of the disease, histological forms and other parameters included in the registration form card (in accordance with the international standard of the Eurocare program). Results. From 2000 to 2018, 4970 children (0–14 years old) with malignant tumors were registered in the Northwestern Federal District of the Russian Federation, of which 3011 are subject to the staging of the tumor process. It is important to note that among all children of patients with malignant tumors, the five-year survival rate of stageable and non-stageable patients was close to 80 %. There is a high level of staging of patients of this group. Thus, patients with stage I of the disease had a five-year survival rate (2010–2014) – 96.3 %, with II – 91.0 % and only with stage IV – 50.9 %.Conclusions. The creation of a children’s population cancer registry at the federal district level could significantly raise the level of primary registration of malignant tumors, eliminate losses and be able to calculate analytical indicators for rare childhood localization of malignant tumors.","PeriodicalId":52396,"journal":{"name":"Russian Journal of Pediatric Hematology and Oncology","volume":"63 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-07-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"73143605","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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