Introduction: Nutrition is of great importance in the treatment of malignant diseases because the nutritional status affects the outcome of the disease itself, the tolerance of chemotherapy and the overall survival and quality of life. This is especially important in childhood when, in addition to the treatment of the underlying disease, it is necessary to ensure the normal growth and development of the child. Objective: The aim of this paper was, based on a review of the literature, to point out the importance and show the basics of nutritional assessment and monitoring, as well as nutritional interventions in children suffering from malignant diseases. Methods: The available scientific and professional literature (PubMed) for the last 10 years was searched, and the following keywords were used: nutrition, malignant disease, diet, nutritional assessment, and nutritional intervention. All received abstracts were screened and selected were publications that included above keywords as a main topic. Results: Based on the reviewed literature, presented are the basics of nutritional assessment and when to perform it, risks for nutritional disorders and available nutritional interventions. Conclusion: In pediatric oncology patients, it is important to carry out a nutritional assessment not only at the time of diagnosis, but also during the further course of treatment and follow-up of the patient, to determine individual nutritional interventions that will prevent or treat an already occurring nutritional disorder, and to ensure the growth of the child in accordance with the genetic potential.
{"title":"Nutritional interventions in children treated for malignant diseases","authors":"Z. Mišak, Sara Sila","doi":"10.13112/pc.2024.13","DOIUrl":"https://doi.org/10.13112/pc.2024.13","url":null,"abstract":"Introduction: Nutrition is of great importance in the treatment of malignant diseases because the nutritional status affects the outcome of the disease itself, the tolerance of chemotherapy and the overall survival and quality of life. This is especially important in childhood when, in addition to the treatment of the underlying disease, it is necessary to ensure the normal growth and development of the child.\u0000Objective: The aim of this paper was, based on a review of the literature, to point out the importance and show the basics of nutritional assessment and monitoring, as well as nutritional interventions in children suffering from malignant diseases.\u0000Methods: The available scientific and professional literature (PubMed) for the last 10 years was searched, and the following keywords were used: nutrition, malignant disease, diet, nutritional assessment, and nutritional intervention. All received abstracts were screened and selected were publications that included above keywords as a main topic.\u0000Results: Based on the reviewed literature, presented are the basics of nutritional assessment and when to perform it, risks for nutritional disorders and available nutritional interventions.\u0000Conclusion: In pediatric oncology patients, it is important to carry out a nutritional assessment not only at the time of diagnosis, but also during the further course of treatment and follow-up of the patient, to determine individual nutritional interventions that will prevent or treat an already occurring nutritional disorder, and to ensure the growth of the child in accordance with the genetic potential.","PeriodicalId":49715,"journal":{"name":"Paediatria Croatica","volume":"143 11","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141810836","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}
Izabela Kranjčec, Arnes Rešić, Domagoj Buljan, Nada Rajačić, Maja Pavlović, Nuša Matijašić Stjepović, Filip Jadrijević Cvrlje, Aleksandra Bonevski, Gordana Jakovljević, Jasminka Stepan Giljević
Aim: As the results of pharmacogenetic studies are increasingly translated into clinical practice, the ultimate goal of personalising treatment for children with cancer seems achievable in the future. Our survey aimed to establish to what extent pharmacogenetics has already been utilised in everyday work.Methods: A retrospective survey on pharmacogenetic testing in children treated for malignancies at the Department of Oncology and Haematology, Children’s Hospital Zagreb, from 2021 to 2023 was carried out.Results: Pharmacogenetic testing was performed in 17.2% of the 180 children (53.3% female, median age 7.0 years), the greatest number of tests obtained in 2023. Preemptive testing included thiopurine S-methyltransferase polymorphisms (in 94.4% of children with acute lymphoblastic leukaemia and 33.3% with eosinophilic granuloma) and methylenetetrahydrofolate reductase gene poly-morphisms assaying (in 55.6% of acute lymphoblastic leukaemia and 23.1% of osteosarcoma patients). In 8 children, pharmacogenetic testing was made due to adverse events (25% lung and 75% liver injury, all grade 4), in the majority of cases presumably related to vincristine. Pharmacogenetic testing results were pathological in all reactively tested patients, requiring dose modification/chemotherapeutics omission in 87.5% of cases.Conclusion: The number of pharmacogenetic assays performed due to high-grade adverse events in children with cancer has been continuously rising, steering otherwise standardised treatment towards a more individualised approach. Preemptive thiopurine Methyltransferase Polymorphism testing has been routinely done in almost all patients planned to receive thiopurines. However, more research is needed on drug-gene pairs in the field of paediatric oncology to minimise treatment-related toxicity and optimise treatment outcomes.
{"title":"Farmakogenetsko profiliranje pedijatrijskih onkoloških bolesnika: iskustvo jednog centra","authors":"Izabela Kranjčec, Arnes Rešić, Domagoj Buljan, Nada Rajačić, Maja Pavlović, Nuša Matijašić Stjepović, Filip Jadrijević Cvrlje, Aleksandra Bonevski, Gordana Jakovljević, Jasminka Stepan Giljević","doi":"10.13112/pc.2024.8","DOIUrl":"https://doi.org/10.13112/pc.2024.8","url":null,"abstract":"Aim: As the results of pharmacogenetic studies are increasingly translated into clinical practice, the ultimate goal of personalising treatment for children with cancer seems achievable in the future. Our survey aimed to establish to what extent pharmacogenetics has already been utilised in everyday work.Methods: A retrospective survey on pharmacogenetic testing in children treated for malignancies at the Department of Oncology and Haematology, Children’s Hospital Zagreb, from 2021 to 2023 was carried out.Results: Pharmacogenetic testing was performed in 17.2% of the 180 children (53.3% female, median age 7.0 years), the greatest number of tests obtained in 2023. Preemptive testing included thiopurine S-methyltransferase polymorphisms (in 94.4% of children with acute lymphoblastic leukaemia and 33.3% with eosinophilic granuloma) and methylenetetrahydrofolate reductase gene poly-morphisms assaying (in 55.6% of acute lymphoblastic leukaemia and 23.1% of osteosarcoma patients). In 8 children, pharmacogenetic testing was made due to adverse events (25% lung and 75% liver injury, all grade 4), in the majority of cases presumably related to vincristine. Pharmacogenetic testing results were pathological in all reactively tested patients, requiring dose modification/chemotherapeutics omission in 87.5% of cases.Conclusion: The number of pharmacogenetic assays performed due to high-grade adverse events in children with cancer has been continuously rising, steering otherwise standardised treatment towards a more individualised approach. Preemptive thiopurine Methyltransferase Polymorphism testing has been routinely done in almost all patients planned to receive thiopurines. However, more research is needed on drug-gene pairs in the field of paediatric oncology to minimise treatment-related toxicity and optimise treatment outcomes.","PeriodicalId":49715,"journal":{"name":"Paediatria Croatica","volume":"9 9","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141813514","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}
D. Keretić, A. Car, Marko Mesić, R. Kralj, I. Petračić, Veronika Miljanović Vrđuka, S. Višnjić
Objective: The objective of this paper is to present the spectrum of surgical procedures that we perform for the purpose of treating oncological patients and the role of surgeons in oncological treatment.Methods: The PubMed medical database was searched with reference to the basic literature in pediatric surgery.Results: By searching and excluding, 18 recent works were considered along with 2 pediatric surgery books on the topic of surgical treatment of solid tumors in childhood. There were no exclusion parameters.Conclusion: The role of surgery in pediatric oncology during the 20th century changed it is position starting from the first and only option for small patients to it is place in the worldwide accepted treatment protocols. The surgical techniques we use in the diagno-sis and treatment of childhood tumors continuously evolve according to a specific type of tumor. Methods that were also previously acceptable are now becoming more and more meticulous to preserve the patient’s quality of life and fertility. Surgical techniques include tumor biopsy methods, surgery of tumor`s primary site (extirpation of tumors, resection of organs or organ systems), staging of tumors according to anatomic extension, operations for disease relapse and metastases, and palliative and supportive procedures. The role of surgery in the prophylaxis of oncological disease is also well defined for various predisposing syndromes and diseases. The most important aspect of surgical oncological treatment is strict adherence to and familiarity with the protocol for a certain type of tumor, as well as a multidisciplinary and individual approach to the pediatric oncologic patient.
{"title":"The role of surgery in the treatment of childhood solid tumors","authors":"D. Keretić, A. Car, Marko Mesić, R. Kralj, I. Petračić, Veronika Miljanović Vrđuka, S. Višnjić","doi":"10.13112/pc.2024.12","DOIUrl":"https://doi.org/10.13112/pc.2024.12","url":null,"abstract":"Objective: The objective of this paper is to present the spectrum of surgical procedures that we perform for the purpose of treating oncological patients and the role of surgeons in oncological treatment.Methods: The PubMed medical database was searched with reference to the basic literature in pediatric surgery.Results: By searching and excluding, 18 recent works were considered along with 2 pediatric surgery books on the topic of surgical treatment of solid tumors in childhood. There were no exclusion parameters.Conclusion: The role of surgery in pediatric oncology during the 20th century changed it is position starting from the first and only option for small patients to it is place in the worldwide accepted treatment protocols. The surgical techniques we use in the diagno-sis and treatment of childhood tumors continuously evolve according to a specific type of tumor. Methods that were also previously acceptable are now becoming more and more meticulous to preserve the patient’s quality of life and fertility. Surgical techniques include tumor biopsy methods, surgery of tumor`s primary site (extirpation of tumors, resection of organs or organ systems), staging of tumors according to anatomic extension, operations for disease relapse and metastases, and palliative and supportive procedures. The role of surgery in the prophylaxis of oncological disease is also well defined for various predisposing syndromes and diseases. The most important aspect of surgical oncological treatment is strict adherence to and familiarity with the protocol for a certain type of tumor, as well as a multidisciplinary and individual approach to the pediatric oncologic patient.","PeriodicalId":49715,"journal":{"name":"Paediatria Croatica","volume":"136 5","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141811232","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}
PET/CT is the most sensitive and highly specific imaging technique for determining the location of tumors, primary tumors of unknown location, determining the extent and activity of malignant disease, monitoring treatment effects, detecting local recurrence and distant malignancy, precise planning of various treatment modalities and planning/determining the radiation field. PET uses biologically active molecules labeled with short-lived radionuclides that emit positrons and are the product of nuclear reactions in a cyclotron. Fluorine-18 (18F) is the most commonly used radionuclide in nuclear medicine, and its relatively long half-life of 110 minutes allows adequate synthesis of radiopharmaceuticals and monitoring of biological processes, as well as delivery to PET machines in remote facilities. The most commonly used radiopharmaceutical, which is now used in clinical practice in more than 90% of PET/CT examinations, is 2-(18F)-fluoro-2-deoxy-D-glucose (18F FDG), a glucose derivative that reflects the accumulation and consumption of glucose in cells, i.e. glucose metabolism. PET/CT has an advantage over other diagnostic imaging techniques because by combining PET, which is the evaluation of the intensity of the metabolic activity of a specific radiopharmaceutical in the cells, and CT, which shows the anatomy and morphology of the organs, we simultaneously obtain information about pathological abnormalities in both the function and morphology of the lesions or organ and the presence of viable tumor or inflammatory tissue. It is important to mention that with the latest PET/CT equipment we have the possibility to visualize metabolic activity even in very small lesions (2 mm). The advantage of this diagnostic method is that the findings are not only analyzed visually, but also quantitatively by measuring the intensity of radiopharmaceutical accumulation in the lesions, thus achieving objectification of the findings and the possibility of adequately monitoring the effect of different forms of treatment in control imaging. PET/CT allows us to identify prognostically risky patients and to select the most appropriate forms of treatment for each individual patient. PET/CT is the most sensitive method for distinguishing treatment-related changes from residual or recurrent disease. A continuous decrease in metabolic activity in the tumor area indicates a positive effect of the treatment. PET/CT enables precise monitoring of the effect of all forms of treatment (surgical, chemotherapeutic, radiotherapeutic and radiosurgical procedures) and has the advantage that the entire body is captured and analyzed with one image in the field of view and not just a single segment. The radiation exposure in PET/CT results from the use of radiopharmaceuticals and CT imaging, and the ALARA principle (as low as reasonably achievable) is followed, whereby the dose is adjusted taking into account the weight and age of the child and the type of device, taking into account the
PET/CT 是最灵敏、特异性最高的成像技术，可用于确定肿瘤位置、位置不明的原发性肿瘤、确定恶性疾病的范围和活动性、监测治疗效果、检测局部复发和远处恶性肿瘤、精确规划各种治疗方式以及规划/确定辐射场。正电子发射计算机断层显像（PET）使用短寿命放射性核素标记的生物活性分子，这些分子发射正电子，是回旋加速器中核反应的产物。氟-18（18F）是核医学中最常用的放射性核素，它的半衰期相对较长，为 110 分钟，可用于合成放射性药物和监测生物过程，也可输送到远程设施中的 PET 机。最常用的放射性药物是 2-(18F)-氟-2-脱氧-D-葡萄糖（18F FDG），它是一种葡萄糖衍生物，可反映细胞中葡萄糖的积累和消耗，即葡萄糖代谢。PET/CT 与其他诊断成像技术相比具有优势，因为通过将 PET（评估特定放射性药物在细胞中的代谢活动强度）与 CT（显示器官的解剖和形态）相结合，我们可以同时获得病变或器官功能和形态的病理异常信息，以及是否存在有活力的肿瘤或炎症组织的信息。值得一提的是，利用最新的 PET/CT 设备，我们甚至可以观察到非常小的病灶（2 毫米）的代谢活动。这种诊断方法的优势在于，不仅可以对检查结果进行直观分析，还可以通过测量病灶中放射性药物的蓄积强度进行定量分析，从而实现检查结果的客观化，并能在对照成像中充分监测不同形式治疗的效果。PET/CT 使我们能够识别预后有风险的病人，并为每个病人选择最合适的治疗方式。PET/CT 是区分治疗相关变化与残留或复发疾病的最灵敏方法。肿瘤区域代谢活动的持续下降表明治疗效果良好。PET/CT 可以精确监测各种形式治疗（手术、化疗、放疗和放射外科手术）的效果，其优势在于通过视野中的一幅图像而不仅仅是单个部分来捕捉和分析整个身体。PET/CT 的辐射量来自于放射性药物的使用和 CT 成像，并遵循 ALARA 原则（尽可能低），即根据儿童的体重、年龄和设备类型调整剂量，同时考虑到儿童机体的寿命较长和对辐射的敏感性。为了评估治疗效果，采用 RECIST 标准（实体瘤反应评估标准）评估病灶的大小，并采用 PERCIST 标准（实体瘤 PET 反应标准）评估病灶的代谢活动。纳入时还应考虑到儿科人群的特点，如检查是否需要家长或监护人同意、检查前的饥饿期等、在建立静脉通路方面的困难、由于成像时需要休息而对幼儿进行镇静/麻醉，以及放射性药物在身体不同部位的明显生理性蓄积。与成人相比，放射性药物在大脑中的总蓄积量比例略高，尿液中的放射性活度排泄量较低，骨髓中的蓄积量因造血功能较强而较高，淋巴组织区域的蓄积量较高、而使用造血生长促进因子会导致活性在脾脏和骨髓中明显弥漫性积累。与其他影像诊断技术相比，PET/MR 能更安全、更具体、更有效地评估儿童的疾病程度。与其他混合诊断程序相比，PET/MR 的优势在于可实现高分辨率以及病变组织与健康组织之间的高对比度，同时显著减少电离辐射暴露。
{"title":"PET/CT in diagnosis and monitoring the effect of treatment in children with malignant tumors","authors":"Sunčana Divošević","doi":"10.13112/pc.2024.18","DOIUrl":"https://doi.org/10.13112/pc.2024.18","url":null,"abstract":"PET/CT is the most sensitive and highly specific imaging technique for determining the location of tumors, primary tumors of unknown location, determining the extent and activity of malignant disease, monitoring treatment effects, detecting local recurrence and distant malignancy, precise planning of various treatment modalities and planning/determining the radiation field. PET uses biologically active molecules labeled with short-lived radionuclides that emit positrons and are the product of nuclear reactions in a cyclotron. Fluorine-18 (18F) is the most commonly used radionuclide in nuclear medicine, and its relatively long half-life of 110 minutes allows adequate synthesis of radiopharmaceuticals and monitoring of biological processes, as well as delivery to PET machines in remote facilities. The most commonly used radiopharmaceutical, which is now used in clinical practice in more than 90% of PET/CT examinations, is 2-(18F)-fluoro-2-deoxy-D-glucose (18F FDG), a glucose derivative that reflects the accumulation and consumption of glucose in cells, i.e. glucose metabolism. PET/CT has an advantage over other diagnostic imaging techniques because by combining PET, which is the evaluation of the intensity of the metabolic activity of a specific radiopharmaceutical in the cells, and CT, which shows the anatomy and morphology of the organs, we simultaneously obtain information about pathological abnormalities in both the function and morphology of the lesions or organ and the presence of viable tumor or inflammatory tissue. It is important to mention that with the latest PET/CT equipment we have the possibility to visualize metabolic activity even in very small lesions (2 mm). The advantage of this diagnostic method is that the findings are not only analyzed visually, but also quantitatively by measuring the intensity of radiopharmaceutical accumulation in the lesions, thus achieving objectification of the findings and the possibility of adequately monitoring the effect of different forms of treatment in control imaging. PET/CT allows us to identify prognostically risky patients and to select the most appropriate forms of treatment for each individual patient. PET/CT is the most sensitive method for distinguishing treatment-related changes from residual or recurrent disease. A continuous decrease in metabolic activity in the tumor area indicates a positive effect of the treatment. PET/CT enables precise monitoring of the effect of all forms of treatment (surgical, chemotherapeutic, radiotherapeutic and radiosurgical procedures) and has the advantage that the entire body is captured and analyzed with one image in the field of view and not just a single segment. The radiation exposure in PET/CT results from the use of radiopharmaceuticals and CT imaging, and the ALARA principle (as low as reasonably achievable) is followed, whereby the dose is adjusted taking into account the weight and age of the child and the type of device, taking into account the ","PeriodicalId":49715,"journal":{"name":"Paediatria Croatica","volume":"123 12","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141811832","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}
Osteosarcoma is the most common primary tumor of the bone with the highest incidence in the first two decades of life. The incidence rate with 95% confidence is 4 for the range of 0-14 and 5 for the range of 0-19 cases pre million per year. Osteosarcoma is a rare malignant mesenchymal tumor with presence of mesenchymal cells and production of osteoid matrix (1). Distinct histological subtypes have been defined, but the biological behavior and the conventional approach to treatment have been similar for last couple of decades without improvement in outcome. The biology of osteosarcoma is characterized with a high rate of lung metastasis. Disorganized genome seems to be the best description of genetic aberrations and changes in gene expression in osteosarcoma, with the most consistent finding, beside the p53 and RB dysregulation,significant aneuploidy and some evidence of massive disruption in the chromosomal structure (2). The metastatic cascade represents a process where cell leaves primary tumor and invades the surrounding tumormicroenvironment with intravascular and also extravascular invasion to the distant sites enabling the blood supply and growth to the secondary site and reengage to the new microenvironment. Meanwhile, metastaticcell could be dormant in the „protective“ environment and then move to the secondary distant sites (3). The microscopic metastases are usually responsible for disease progression, so targeting geneticand epigenetic alterations will certainly improve the outcome (2). Recent studies showed that metastatic clones often do not correspond to the dominant clones in the primary tumor, but may evolve monoclonal and polyclonal, showing the clonal/subclonal heterogeneity of osteosarcoma. At the same time, immune response is a complex process that combines recognition of tumor cells, and response of effector and regulatory immune cells. Tumor cells by soluble factors secretion lead to downmodulation of the immune system. The well known immune „escape“ is the hallmark of cancer when immune system play a dual function, slowing down the tumor progression at first and then facilitating the tumor growth after the modelling phase of tumor cells. How to switch immunotolerance which contribute to permissive microenvironment beneficial for tumor cells to immunocompetent environment, is the challenge of immunotherapy (4). Two mains immunotherapeutic approaches are proposed for bone sarcomas. The first one is based on targeting the pro-tumoral effectors including M2 macrophages, i.e. muramyl tripeptide phosphatidylethanolamine (MTP-PE), interferon gamma (IFNγ) and the molecules associated with immune checkpoints; programmed death-ligand 1 (PD-L1) with it's receptor, programmed cell death protein (PD1) and cytotoxic T-lymphocyte-associated antigen 4 (CTLA-4). The other one is based on the stimulation of anti-tumoral effectors; dendritic cells (DC), nitrogen containing bisphosphonates (N-BPs) and natural killer cells (NK). MTP-PE is a dr
{"title":"Insights to biology and immunotherapy of osteosarcoma","authors":"A. Bonevski, M. Milavić, Sven Seiwerth","doi":"10.13112/pc.2024.17","DOIUrl":"https://doi.org/10.13112/pc.2024.17","url":null,"abstract":"Osteosarcoma is the most common primary tumor of the bone with the highest incidence in the first two decades of life. The incidence rate with 95% confidence is 4 for the range of 0-14 and 5 for the range of 0-19 cases pre million per year. Osteosarcoma is a rare malignant mesenchymal tumor with presence of mesenchymal cells and production of osteoid matrix (1). Distinct histological subtypes have been defined, but the biological behavior and the conventional approach to treatment have been similar for last couple of decades without improvement in outcome. The biology of osteosarcoma is characterized with a high rate of lung metastasis. Disorganized genome seems to be the best description of genetic aberrations and changes in gene expression in osteosarcoma, with the most consistent finding, beside the p53 and RB dysregulation,significant aneuploidy and some evidence of massive disruption in the chromosomal structure (2). The metastatic cascade represents a process where cell leaves primary tumor and invades the surrounding tumormicroenvironment with intravascular and also extravascular invasion to the distant sites enabling the blood supply and growth to the secondary site and reengage to the new microenvironment. Meanwhile, metastaticcell could be dormant in the „protective“ environment and then move to the secondary distant sites (3). The microscopic metastases are usually responsible for disease progression, so targeting geneticand epigenetic alterations will certainly improve the outcome (2). Recent studies showed that metastatic clones often do not correspond to the dominant clones in the primary tumor, but may evolve monoclonal and polyclonal, showing the clonal/subclonal heterogeneity of osteosarcoma. At the same time, immune response is a complex process that combines recognition of tumor cells, and response of effector and regulatory immune cells. Tumor cells by soluble factors secretion lead to downmodulation of the immune system. The well known immune „escape“ is the hallmark of cancer when immune system play a dual function, slowing down the tumor progression at first and then facilitating the tumor growth after the modelling phase of tumor cells. How to switch immunotolerance which contribute to permissive microenvironment beneficial for tumor cells to immunocompetent environment, is the challenge of immunotherapy (4). Two mains immunotherapeutic approaches are proposed for bone sarcomas. The first one is based on targeting the pro-tumoral effectors including M2 macrophages, i.e. muramyl tripeptide phosphatidylethanolamine (MTP-PE), interferon gamma (IFNγ) and the molecules associated with immune checkpoints; programmed death-ligand 1 (PD-L1) with it's receptor, programmed cell death protein (PD1) and cytotoxic T-lymphocyte-associated antigen 4 (CTLA-4). The other one is based on the stimulation of anti-tumoral effectors; dendritic cells (DC), nitrogen containing bisphosphonates (N-BPs) and natural killer cells (NK). MTP-PE is a dr","PeriodicalId":49715,"journal":{"name":"Paediatria Croatica","volume":"99 25","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141812206","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}
Maja Pavlović, Domagoj Buljan, Aleksandra Bonevski
Non-rhabdomyosarcoma soft-tissue sarcoma (“RMS-like” and “non-RMS-like” tumours- NRSTS) of childhood is a highly heterogeneous group of tumours. Within the subgroup of undifferentiated sarcomas (UDS), new entities have been described based on molecular markers found through increasingly available genetic analysis in the new 2020 classification of the World Health Organization (WHO). In recent years, the importance of genetic analysis and the detection of molecular tumour markers has been growing due to the possibility of targeted therapy application and the determination of prognosis or disease course. We will present two patients with soft tissue sarcoma and neurotrophic tyrosine receptor kinase (NTRK) gene rearrangement, focusing on excellent therapeutic response to NTRK inhibitor targeted therapy with good drug tolerance.
{"title":"Ciljana terapija entrektinibom u djece s mezenhimalnimneoplazmama s NTRK rearanžmanom","authors":"Maja Pavlović, Domagoj Buljan, Aleksandra Bonevski","doi":"10.13112/pc.2024.15","DOIUrl":"https://doi.org/10.13112/pc.2024.15","url":null,"abstract":"Non-rhabdomyosarcoma soft-tissue sarcoma (“RMS-like” and “non-RMS-like” tumours- NRSTS) of childhood is a highly heterogeneous group of tumours. Within the subgroup of undifferentiated sarcomas (UDS), new entities have been described based on molecular markers found through increasingly available genetic analysis in the new 2020 classification of the World Health Organization (WHO). In recent years, the importance of genetic analysis and the detection of molecular tumour markers has been growing due to the possibility of targeted therapy application and the determination of prognosis or disease course. We will present two patients with soft tissue sarcoma and neurotrophic tyrosine receptor kinase (NTRK) gene rearrangement, focusing on excellent therapeutic response to NTRK inhibitor targeted therapy with good drug tolerance.","PeriodicalId":49715,"journal":{"name":"Paediatria Croatica","volume":"88 24","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141812737","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}
Neuroblastoma is childhood’s most common extracranial solid tumor, with a broad spectrum of biological conduct. The high-risk disease encompasses a significant portion of childhood cancer mortality. Nuclear medicine methods, including disease risk categorization, are essential in the diagnostic process. However, nuclear medicine has its place as a segment of the multidisciplinary treatment of high-risk diseases, too. In this article, a patient diagnosed with high-risk neuroblastoma due to age and metastases is presented, with an accent on the role of nuclear medicine and a multidisciplinary approach in diagnostic and therapeutic procedures.
{"title":"Nuclear medicine methods in the diagnosis and treatmentof high-risk neuroblastoma - case report","authors":"Vedrana Gladić Nenadić, Nada Rajacic","doi":"10.13112/pc.2024.16","DOIUrl":"https://doi.org/10.13112/pc.2024.16","url":null,"abstract":"Neuroblastoma is childhood’s most common extracranial solid tumor, with a broad spectrum of biological conduct. The high-risk disease encompasses a significant portion of childhood cancer mortality. Nuclear medicine methods, including disease risk categorization, are essential in the diagnostic process. However, nuclear medicine has its place as a segment of the multidisciplinary treatment of high-risk diseases, too. In this article, a patient diagnosed with high-risk neuroblastoma due to age and metastases is presented, with an accent on the role of nuclear medicine and a multidisciplinary approach in diagnostic and therapeutic procedures.","PeriodicalId":49715,"journal":{"name":"Paediatria Croatica","volume":"33 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141813278","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}
Ista tumorska bolest kod različitih bolesnika može imati različitu prognozu, odnosno dužinu preživljenja. S ciljem individualizacije terapije i poboljšanja liječenja pedijatrijskih malignih tumora, intenzitet i vrsta liječenja iste tumorske bolesti kod razičitih bolesnika mo?e biti različita. Razlike u liječenju određene su stupnjem rizika, odnosno prognostičkim čimbenicima rizika. Prognostički čimbenici rizika utječu na ishod liječenja i preživljenje.
{"title":"ODREĐIVANJE PROGNOSTIČKOG RIZIKA TUMORSKE BOLESTI KOD DJECE SA SOLIDNIM TUMORIMA","authors":"Gordana Jakovljević","doi":"10.13112/pc.2024.19","DOIUrl":"https://doi.org/10.13112/pc.2024.19","url":null,"abstract":"Ista tumorska bolest kod različitih bolesnika može imati različitu prognozu, odnosno dužinu preživljenja. S ciljem individualizacije terapije i poboljšanja liječenja pedijatrijskih malignih tumora, intenzitet i vrsta liječenja iste tumorske bolesti kod razičitih bolesnika mo?e biti različita. Razlike u liječenju određene su stupnjem rizika, odnosno prognostičkim čimbenicima rizika. Prognostički čimbenici rizika utječu na ishod liječenja i preživljenje.","PeriodicalId":49715,"journal":{"name":"Paediatria Croatica","volume":"19 2","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141809934","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}
Malignant diseases in the paediatric population are rare and account for only 0.5% of all newly diagnosed malignant diseases. In Croatia, 105 to 125 children are diagnosed with malignant diseases every year. As in most countries of the European Union, they are the second most common cause of death in this age group, after accidents. Treatment outcomes for children with malignant diseases in Croatia are comparable to those in other countries of the European Union. Due to the small number of patients, only slightly more than one hundred new patients per year, it would be necessary to centralize the treatment of all high-risk patients. Such children should be treated in two centers, one of which would be a center for solid tumors and the other a center for haematological neoplasms. Such an approach would probably allow an additional improvement in treatment outcomes with practically same resources.
{"title":"Mjesto zbrinjavanja djetetasa solidnim malignim tumorom u Hrvatskoj","authors":"E. Bilić, Matej Jelic","doi":"10.13112/pc.2024.9","DOIUrl":"https://doi.org/10.13112/pc.2024.9","url":null,"abstract":"Malignant diseases in the paediatric population are rare and account for only 0.5% of all newly diagnosed malignant diseases. In Croatia, 105 to 125 children are diagnosed with malignant diseases every year. As in most countries of the European Union, they are the second most common cause of death in this age group, after accidents. Treatment outcomes for children with malignant diseases in Croatia are comparable to those in other countries of the European Union. Due to the small number of patients, only slightly more than one hundred new patients per year, it would be necessary to centralize the treatment of all high-risk patients. Such children should be treated in two centers, one of which would be a center for solid tumors and the other a center for haematological neoplasms. Such an approach would probably allow an additional improvement in treatment outcomes with practically same resources.","PeriodicalId":49715,"journal":{"name":"Paediatria Croatica","volume":"32 25","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141813998","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}
With current treatment protocols, over 90% of children with acute lymphoblastic leukemia (ALL) are cured. Simultaneously with these excellent results, there is an increasing importance of the recognition of possible late effects of antileukemic treatment. The most frequent late effects of therapy for childhood ALL include endocrine abnormalities, obesity, growth disturbances, neurocognitive deficits, psychosocial adverse effects, cardiotoxicity, gonadotoxicity and reproductive changes, neurotoxicity, bone toxicity, secondary malignancies, and premature late mortality. Better recognition of late effects has resulted in the modifications of treatment regimens and development of guidelines for lifelong follow-up of survivors.
按照目前的治疗方案，90% 以上的急性淋巴细胞白血病（ALL）患儿都能治愈。在取得这些优异成绩的同时，认识到抗白血病治疗可能产生的晚期效应也变得越来越重要。儿童 ALL 治疗最常见的晚期效应包括内分泌异常、肥胖、生长障碍、神经认知障碍、社会心理不良反应、心脏毒性、性腺毒性和生殖系统变化、神经毒性、骨毒性、继发性恶性肿瘤和过早晚期死亡。由于对晚期效应有了更好的认识，因此对治疗方案进行了修改，并制定了对幸存者进行终生随访的指南。
{"title":"Kasne posljedice liječenja pedijatrijske akutne limfoblastične leukemije","authors":"Jelena Roganović","doi":"10.13112/pc.2024.10","DOIUrl":"https://doi.org/10.13112/pc.2024.10","url":null,"abstract":"With current treatment protocols, over 90% of children with acute lymphoblastic leukemia (ALL) are cured. Simultaneously with these excellent results, there is an increasing importance of the recognition of possible late effects of antileukemic treatment. The most frequent late effects of therapy for childhood ALL include endocrine abnormalities, obesity, growth disturbances, neurocognitive deficits, psychosocial adverse effects, cardiotoxicity, gonadotoxicity and reproductive changes, neurotoxicity, bone toxicity, secondary malignancies, and premature late mortality. Better recognition of late effects has resulted in the modifications of treatment regimens and development of guidelines for lifelong follow-up of survivors.","PeriodicalId":49715,"journal":{"name":"Paediatria Croatica","volume":"16 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141810662","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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