Cognitive performance of medulloblastoma tumour survivors related to the area of cerebellum damage.

Background: The aim of the study was to determine the relationship between a damaged cerebellum area and the cognitive performance of medulloblastoma tumour survivors. Also, age-based differences in cognitive performance were tested. Materials and methods: Magnetic resonance imaging (MRI) technique was used to obtain brain images of survivors. The cognitive performance was tested using Wechsler Intelligence Scale for Children Revised (WISC-R) and Wechsler Adult Intelligence Scale (WAIS). Statistical analysis was performed with highly robust permutation tests. Results: There were two anatomical features strongly influencing the cognitive performance of survivors. The extension of the foramen of Luschka had a negative impact on the overall verbal IQ score and some non-verbal scales while the excision of the middle part of the vermis influenced scores in such scales as arithmetic and picture completing. Conclusions: Children with postoperative damages in the area of the middle part of the vermis are more likely to suffer from cognitive dysfunctions after the end of the treatment.

Quality of life in survivors of childhood brain tumour and the association of children's diseases on quality of their parents life.

OBJECTIVE: Evaluation of children's quality of life (QoL) after finished brain tumour treatment and the association of children's diseases on quality of their parents' life. METHODS: The study group was consisted of 46 children after brain tumour treatment (aged 4, 5, to 29 years old). The control group was composed of 104 students of primary, secondary, and high schools. One hundred fifty (104 + 46) parents were included in the study. Standardised QoL questionnaires (PEDsQL-4.0, WHOQOL-BREF) were used. Survivors' QoL was assessed from patients' and their parents' point of view, also the association of children's diseases on quality of their parents' life was estimated. RESULTS: QoL of children after brain tumour treatment was lower than in the control group according to the children (P < 0.001) and their parents (P < 0.001). The survivors worst rated their ability to social functioning (P < 0.0010) and physical functioning (P < 0.001) in comparison with self-assessment of healthy children. According to their parents, the functioning of children in all zones was worse than in the control group, mostly in social (P < 0.001) and physical sphere (P < 0.001), too. QoL of children with low-grade tumour was comparable with QoL of children with high-grade tumour). QoL of survivors' caregivers in study was higher than QoL of parents of control groups (P = 0.023). CONCLUSIONS: The quality of patients' life after brain tumour treatment is lower in comparison with healthy children. QoL of the parents of survivor is higher than the QoL of healthy children parents. The assessment of QoL of children after brain tumour treatment should be an inherent element of health monitoring.

Imatinib in the treatment of chronic myeloid leukemia in children and adolescents is effective and well tolerated: Report of the Polish Pediatric Study Group for the Treatment of Leukemias and Lymphomas.

BACKGROUND: Chronic myeloid leukemia (CML) constitutes only 2-3% of all leukemias in pediatric patients. Philapelphia chromosome and BCR-ABL fusion are genetic hallmarks of CML, and their presence is crucial for targeted molecular therapy with tyrosine kinase inhibitors (TKIs), which replaced hematopoietic stem cell transplantation (HSCT) as a standard first-line therapy. The disease in pediatric population is rare, and despite molecular and clinical similarities to CML in adults, different approach is needed, due to the long lifetime expectancy and distinct developmental characteristics of affected children. OBJECTIVES: The objective of this study is to evaluate treatment with imatinib in Polish pediatric patients with CML. MATERIAL AND METHODS: We analyzed the results of treatment with imatinib in 57 pediatric patients (June 2006 - January 2016) from 14 Polish pediatric hematology and oncology centers. RESULTS: In the study group, 40 patients continued imatinib (median follow-up: 23.4 months), while in 17 the treatment was terminated (median follow-up: 15.1 months) due to therapy failure. In the latter group, 13 patients underwent HSCT, while 4 switched to second-generation TKIs. The 5-year overall survival rate (OS) in the study group was 96%, and the 5-year event-free survival (EFS) was 81%. CONCLUSIONS: Our results confirm that the introduction of TKI therapy has revolutionized the treatment of CML in the pediatric population by replacing the previous method of treatment with HSCT and allowing a high percentage of OS and EFS.

PRES in the course of hemato-oncological treatment in children.

INTRODUCTION: Posterior reversible leukoencephalopathy syndrome (PRES) is a clinical syndrome of varying aetiologies, characterised by acute neurological symptoms of brain dysfunction with MRI abnormalities in posterior cerebral white and grey matter. In most cases, symptoms resolve without neurological consequences. AIM: The aim of this paper is the analysis of predisposing factors, clinical outcomes and radiological features of PRES in eight children with hemato-oncological disease. MATERIAL AND METHODS: We analysed the medical records of eight hemato-oncological patients aged from 3.0 to 16.1 years. The mean of age at primary diagnosis was 8.5 years. RESULTS: All patients had both clinical and radiological PRES features. Seven out of eight underwent intensive chemotherapy regimens. Time elapsed from start of treatment to the occurrence of PRES ranged from 6 to 556 days. In one case, PRES occurred before chemotherapy and was the first symptom of cancer. Most (six of eight) patients had history of hypertension (> 95pc) and some (two of eight) occurred electrolyte imbalance-mainly hypomagnesaemia. Patients presented headache (seven of eight), disturbances of consciousness (six of eight), seizures (six of eight), visual changes (four of eight) and vomiting (three of eight). MRI demonstrated abnormalities in seven children: typical cerebral oedema in the white matter of the occipital to the parietal lobes. Most patient lesions in the MRI shrunk after 4 weeks, and clinical symptoms of PRES disappeared completely within a few hours to few days. CONCLUSION: PRES may complicate oncological treatment in children. Hypertension is the most important risk factor of PRES. PRES should be included in differential diagnosis in all patients with acute neurological symptoms.

[Difficulties of the therapy in a boy with coexisting type 1 diabetes mellitus and idiopathic thrombocytopenic purpura]. / Trudnosci terapeutyczne u pacjenta ze wspólistniejaca cukrzyca typu 1 oraz maloplytkowoscia immunologiczna.

Idiopathic thrombocytopenic purpura (ITP) is an acquired autoimmune disease, caused by antibodies against platelet glycoproteins, which provoke platelet destruction and inhibit platelet production in bone marrow. Type 1 Diabetes Mellitus (T1DM) is an acquired autoimmune disease in witch beta cells are destroyed by autoantibodies. Patient with T1DM since the age of 6, was treated by intensive functional insulin therapy by insulin pump. At the age of 14,5 he was also diagnosed with ITP. Due to the short effect of immunoglobulin therapy, glucocorticoids were introduced. After 3 months of glucocorticotherapy the platelet count was 46 G/l. Patient developed various adverse effects of glucocorticoids, among others stretch marks covering all surface of his abdomen, buttocks, arms and thighs and raise in the daily requirement of insulin by 200%. Adverse effects of glucocorticotherapy made impossible the therapy by insulin pump and imposed urgent revision of the ITP therapy. Side effects of the glucocorticotherapy can make impossible the treatment by insulin pump of T1DM in children.

Serum levels of vascular endothelial growth factor and basic fibroblast growth factor in children with brain tumors.

BACKGROUND: Angiogenesis is the process of new vessel formation originating from the existing vascular network. It plays an important role in the growth and spread of malignancies, including brain tumors. The process of angiogenesis is characterized by increased expression of vascular endothelial growth factor (VEGF) and basic fibroblast growth factor (bFGF), and by the release of their soluble forms into circulation. OBJECTIVES: The aim of the study was to evaluate serum levels of VEGF and bFGF in children with malignant and benign brain tumors. MATERIAL AND METHODS: The study group (group N) included 106 children diagnosed with brain tumors. The children in group N were classified according to tumor pathology into 3 subgroups: N1 (n = 63): patients with malignant tumors, excluding anaplastic astrocytoma (AA) and glioblastoma multiforme (GBM); N2 (n = 25): patients with benign tumors; and N3 (n = 18): patients with high grade gliomas (AA and GBM). VEGF and bFGF were determined by ELISA in blood samples before the initiation of chemotherapy. VEGF and bFGF levels were compared within the subgroups in relation to tumor grading and the extent of surgery. RESULTS: The median VEGF in patients with brain tumors was significantly higher than in the control group. The median levels of VEGF and bFGF in subgroup N1 were significantly higher than in the control group. The differences in VEGF and bFGF concentrations between the subgroups in relation to the extent of tumor resection were not significant. CONCLUSIONS: Significantly higher plasma VEGF levels in children with brain neoplasms may reflect enhanced angiogenesis in the tumors.

Renal function in children treated for central nervous system malignancies.

AIM: The aim of the study was to evaluate renal function and to assess the usefulness of the following nephrotoxicity markers: cystatin C (CYS C), beta-2 microglobulin (B2MG) and neutrophil gelatinase-associated lipocalin (NGAL) in 38 (18 girls, 20 boys) children previously treated for central nervous system malignancy. MATERIAL: Median age at evaluation was 13.7 years (range 2.1-22 years). The mean follow-up time after the completion of chemotherapy was 3.2 years (range 0.16-6.5 years). RESULTS: Subclinical chronic kidney disease (estimated glomerular filtration rate: eGFR 90-60 ml/min/1.73 m(2)) was found in 22 patients (58 %), while renal insufficiency (eGFR 30-60 ml/min/1.73 m(2)) was found in six children (16 %). It has been demonstrated statistically significant negative correlation between the eGFR and cystatin C concentration (p < 0.0001) and eGFR and beta-2 microglobulin concentration (p < 0.02). Conversely, there was no correlation between eGFR and NGAL. Thirteen children (34 %) developed drug-induced tubulopathy: decreased tubular reabsorption of phosphate (TRP) and renal tubular threshold for phosphate (Tmp/GFR). CONCLUSION: Children treated for CNS tumours often develop drug-induced chronic renal disease, involving the glomeruli and/or renal tubules. Cystatin C and beta-2 microglobulin seemed to be good markers for chronic kidney damage in these patients, which is probably not true for NGAL.

Infratentorial ependymomas--a study of the centre in Katowice.

The aim of the study was to assess the correlation of the results of the treatment of infratentorial ependymomas with the degree of resection and histopathological diagnosis. The study was conducted on a group of 19 patients, 13 boys and 6 girls aged 3 months to 16 years, with infratentorial ependymoma treated at the Department of Paediatric Neurosurgery of the Medical University of Silesia in Katowice from January 2000 until December 2008. The most significant factor having an impact on overall survival and progression-free survival was totality of tumour resection. There has been no statistically significant influence of the histopathological type of ependymoma on the result of treatment. The tendency to report better results of treatment of non-anaplastic ependymoma seems to derive from a statistically higher frequency of total removal of tumours of this type.

Leptin concentration and nutritional status in the course of treatment in children with brain tumours--preliminary report.

PURPOSE: To assess the nutritional status in children with central nervous system (CNS) tumours, including concentration of leptin, the neuropeptide responsible for regulation of energetic homeostasis in an organism. METHOD: The studied group comprised 44 children with brain tumours, aged (4.02-18.7). In all children during the whole therapy (from the start to the period of 1 year and more after the end of therapy), a number of standard deviations (SDs) for the body mass index (SDS BMI) was derived from anthropometric measurements. Concentrations of leptin were assayed simultaneously. RESULT: The lowest values of the anthropometric indices were found in children during the maintenance therapy. Concentrations of leptin in patients with malignant CNS tumours and significant undernutrition were slightly greater as compared to patients presenting normal nutritional status; however, without statistical significance. CONCLUSION: In children with tumours of the central nervous system, there are quantitative disorders of the nutritional status which correlate with the period of the treatment. The most significant disorders in the nutritional status are observed during maintenance chemotherapy. There was no statistically significant correlation between the concentration of leptin and nutritional status in children with malignant brain tumours during the course of treatment and after its completion.

Rhabdomyolysis: rare complications with a difficult prognosis in the course of anticancer treatment.

Rhabdomyolysis refers to a number of clinical and biochemical symptoms, which result from the destruction of skeletal muscles. The following triad of symptoms is considered typical: myalgia, muscle weakness, and dark urine. The most common reasons for rhabdomyolysis in children are infections. It has also been reported that rhabdomyolysis may be caused by chemotherapy drugs. The most difficult complication of rhabdomyolysis is renal failure. The authors present a 17-year-old boy diagnosed with Ewing sarcoma and a 16-year-old boy suffering from acute leukemia, both with rhabdomyolysis developed in the course of infection caused by Clostridium difficile, and drug-induced neutropenia.

Proangiogenic cytokines vascular-endothelial growth factor and basic fibroblst growth factor in childhood lymphadenopathy.

Increased angiogenesis is observed both in the inflammatory and in the neoplasmatic tissue. The aim of the study was to assess the diagnostic significance of serum concentration of vascular-endothelial growth factor (sVEGF) and basic fibroblast growth factor (sbFGF) in the various forms of lymphadenopathy in children. Ninety-four children with lymphadenopathy were studied: group A, 52 patients with lymphadenitis; group B, 42 patients with lymphomas. Group B was divided into subgroups: B(P), children with lymphomas in peripheral lymph nodes and B(M), children with lymphomas in peripheral lymph nodes and mediastinal tumor. The healthy control group was 20 children. Using enzyme-linked immunosorbent assays the authors quantified VEGF and bFGF in serum of healthy children and of children with lymphadenopathy. The sVEGF in group A was significantly higher than controls (313.8 versus 44.6 pg/mL; P <.05) and in group B was 633.4 pg/mL and was significantly higher than controls (P <.0001). The sVEGF and bFGF in group A versus subgroup B(P) were significantly lower (P(VEGF) <.05, P(bFGF) <.05), and sVEGF in subgroup B(P) versus B(M) was significantly lower (P <.05). These results show that the evaluation of serum VEGF concentration might be useful as noninvasive diagnosis of some chronic peripheral lymphadenopathies in children.

[Mediastinal involvement in the course of proliferative diseases in children in the materials of one institution]. / Zmiany w sródpiersiu w przebiegu chorób rozrostowych u dzieci w materiale jednego osrodka.

UNLABELLED: The mediastinum region may be the primary or secondary localization of neoplasms. The aim of our study was the assessment of clinical symptoms, histopathology and outcome of mediastinal tumours in children. MATERIALS AND METHODS: A group of 27 children, chosen from the group of 128 patients with malignancies, which were treated in the years 2000-2004 in the Oncology and Haematology Unit of the Paediatric Department in Katowice, has been studied. In this group there were 14 boys and 13 girls at the age between 2 and 12 years. We analysed the clinical symptoms prior to the diagnosis and the duration of these symptoms, histologic type of tumours and results of treatment. RESULTS: The primary localization in the mediastinum was diagnosed in 23 patients (85,2%) among them 15 (55,5%) of these children have been diagnosed as having Hodgkin's disease, and in 8 (29,6%) non Hodgkin's lymphoproliferative disease. Four of the children (14,8%) had mediastinal secondary localization of solid tumours (2 - neuroblastoma, 1 - carcinoma suprarenalis, 1 - carcinoma epitheliale of unknown origin). The most frequent symptoms were: fever (70,3%), weakness (66,6%), cough (55,5%), madiastinalpain (33,3%). The duration of these symptoms prior to the diagnosis was between 5 days and 182 clays. CONCLUSIONS: 1. In the group of neoplasms localized in the mediastinum, lymphoproliferative diseases are the most frequently diagnosed disorders. 2. In case of fever of unclear etiology and persistent cough, it is necessary to exclude mediastinal malignancy. 3. There is a need to improve the system of oncological education for medical students and doctors.

[Orbital pseudotumour imitating a proliferative process]. / Pseudotumor orbitae--schorzenie imitujace proces rozrostowy.

INTRODUCTION: Orbital pseudotumour is a non-specific inflammatory process of the orbit of unknown origin. It is a rarely diagnosed disease particularly in children, which imitates a neoplastic process. Typical clinical picture is a tumour localized in the orbit, causing various degree of exophtalmus and a decrease of globe mobility and vision. The extent of intraorbital changes are revealed by imaging studies (USG, TK, MR). Diagnosis is based on histopathology of tumour sample. In treatment steroid therapy, radiotherapy or chemotherapy in resistant cases are used. Relapse and malignant transformation are observed. CASE REPORT: We present a case of a 5-years old girl with orbital pseudotumour. In the histopathological examination there a small lymphoid cells, immunohistochemically there is mixed lymphocytic T and B infiltration (CD 3 (+), CD 20 (+), bcl (+), CD 43 (-)). She was treated with steroid therapy, and achieved complete regression of the tumour. At present she is regularly oncologically examined because of the possibility of malignant transformation. CONCLUSIONS: 1. Orbital pseudotumour should be included in the differential diagnosis of children with an orbital tumour 2. Corticosteroids seem to be the treatment of choice in orbital pseudotumour 3. Children with orbital pseudotumour should be regularly oncologically examined because of the possibility of malignant transformation.