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1.
J Pediatr Hematol Oncol ; 45(7): 383-391, 2023 10 01.
Article in English | MEDLINE | ID: mdl-37027243

ABSTRACT

PURPOSE: The aim of this study was to evaluate the prognostic value of textural parameters of primary tumors, serum lactate dehydrogenase (LDH), D -dimer, and ferritin in high-risk neuroblastoma patients. PATIENTS AND METHODS: The imaging findings of 22 neuroblastoma patients (14 girls and 8 boys; age, 36.6 ± 34.2 [range: 5 to 138] months) who underwent 18-fluorodeoxyglucose positron emission tomography/computed tomography for primary staging before therapy between 2009 and 2020 were retrospectively evaluated. Positron emission tomography-derived metabolic data (maximum standard uptake value, mean standard uptake value, metabolic tumor volume, and total lesion glycolysis) and textural features of primary tumors were obtained. Serum LDH, D -dimer, and ferritin levels at the time of diagnosis were recorded. Univariate and multivariate Cox proportional hazards regression models were used to identify predictors for progression-free survival (PFS) and overall survival (OS). Survival curves were estimated by using the Kaplan-Meier method. RESULTS: The median follow-up duration after diagnosis was 63 months (range: 5 to 141 mo). The median PFS and OS in all patients were 19 and 72 months, respectively. In multivariate Cox regression analyses with backward stepwise selection, grey level size zone matrix_size zone emphasis (GLSZM_SZE) was found as an independent predictor for both PFS and OS. Serum ferritin level was also found as an independent predictor for PFS. The Kaplan-Meier survival analysis showed that higher serum LDH, D -dimer, GLSZM_SZE, and zone size nonuniformity were significantly associated with shorter OS. CONCLUSION: Serum LDH, D -dimer, ferritin levels, and GLSZM_SZE of primary tumors may be used as prognostic biomarkers to identify patients with worse prognoses in high-risk neuroblastoma. GLSZM textural features showing higher tumor heterogeneity are significantly associated with shorter PFS and OS.


Subject(s)
Neuroblastoma , Positron-Emission Tomography , Male , Female , Humans , Infant , Child, Preschool , Child , Prognosis , Retrospective Studies , Positron Emission Tomography Computed Tomography/methods , Biomarkers , Fluorodeoxyglucose F18/metabolism , Neuroblastoma/diagnostic imaging , Ferritins , Tumor Burden , Radiopharmaceuticals
2.
J Pediatr Hematol Oncol ; 41(7): 542-550, 2019 10.
Article in English | MEDLINE | ID: mdl-30933019

ABSTRACT

The aims of our study were to compare F-18 fluorodeoxyglucose (FDG) positron-emission tomography/magnetic resonance imaging (PET/MRI) and PET/computed tomography (CT) in pediatric oncology patients in terms of anatomic correlation of FDG-positive lesions, and also to compare diffusion-weighted imaging (DWI) with PET to assess the correlation between apparent diffusion coefficient (ADC) values and standardized uptake value (SUV). Sequential PET/CT and PET/MRI images and/or whole-body DWI and ADC mapping in 34 pediatric patients were retrospectively analyzed. FDG-positive lesions were visually scored for CT, T1-weighted, T2-weighted, and DWI images separately in terms of anatomic correlation of FDG-avid lesions. Correlation analysis was performed for SUV parameters and ADC values. Among 47 FDG-positive lesions identified concurrently on PET/CT and PET/MRI, 37 were positive on CT and 46 were positive on at least one MRI sequence (P=0.012). Among 32 FDG-positive lesions for which DWI were available, 31 could be clearly depicted on DWI, resulting in significant difference compared with CT alone in the detection of FDG-positive lesions. No correlation was found between ADC and SUV. FDG PET/MRI exhibits better performance than PET/CT in terms of anatomic correlation of FDG-avid lesions. Therefore, PET/MRI may be more advantageous than PET/CT, not only due to reduced ionizing radiation dose but also for a better depiction of FDG-avid lesions in pediatric PET imaging.


Subject(s)
Magnetic Resonance Imaging/methods , Multimodal Imaging/methods , Neoplasms/diagnostic imaging , Positron Emission Tomography Computed Tomography/methods , Adolescent , Child , Child, Preschool , Female , Fluorodeoxyglucose F18 , Humans , Image Interpretation, Computer-Assisted/methods , Infant , Male , Medical Oncology/methods , Pediatrics/methods , Positron-Emission Tomography/methods , Radiopharmaceuticals
3.
Turk J Med Sci ; 49(4): 1198-1205, 2019 08 08.
Article in English | MEDLINE | ID: mdl-31385488

ABSTRACT

Background/aim: Bacteremia remains an important cause of morbidity and mortality during febrile neutropenia (FN) episodes. We aimed to define the risk factors for bacteremia in febrile neutropenic children with hemato-oncological malignancies. Materials and methods: The records of 150 patients aged ≤18 years who developed FN in hematology and oncology clinics were retrospectively evaluated. Patients with bacteremia were compared to patients with negative blood cultures. Results: The mean age of the patients was 7.5 ± 4.8 years. Leukemia was more prevalent than solid tumors (61.3% vs. 38.7%). Bacteremia was present in 23.3% of the patients. Coagulase-negative staphylococci were the most frequently isolated microorganism. Leukopenia, severe neutropenia, positive peripheral blood and central line cultures during the previous 3 months, presence of a central line, previous FN episode(s), hypotension, tachycardia, and tachypnea were found to be risk factors for bacteremia. Positive central line cultures during the previous 3 months and presence of previous FN episode(s) were shown to increase bacteremia risk by 2.4-fold and 2.5-fold, respectively. Conclusion: Presence of a bacterial growth in central line cultures during the previous 3 months and presence of any previous FN episode(s) were shown to increase bacteremia risk by 2.4-fold and 2.5-fold, respectively. These factors can predict bacteremia in children with FN.


Subject(s)
Bacteremia , Chemotherapy-Induced Febrile Neutropenia , Adolescent , Bacteremia/complications , Bacteremia/epidemiology , Bacteremia/physiopathology , Chemotherapy-Induced Febrile Neutropenia/complications , Chemotherapy-Induced Febrile Neutropenia/epidemiology , Chemotherapy-Induced Febrile Neutropenia/physiopathology , Child , Child, Preschool , Female , Humans , Male , Neoplasms/complications , Neoplasms/epidemiology , Retrospective Studies , Risk Factors
4.
J Pediatr Hematol Oncol ; 40(3): 243-245, 2018 04.
Article in English | MEDLINE | ID: mdl-28692552

ABSTRACT

Niemann-Pick type C disease (NPC) is an autosomal recessive disorder resulting in accumulation of unesterified lysosomal cholesterol. An 8-year-old girl with NPC disease had a painless, rigid, and fixed mass measuring 3 cm in diameter located on the left angular region of mandibula. The mass biopsy showed lipid-laden phagocytic cells infiltrating the lymph node consistent with Niemann-Pick cells. In NPC, accumulation of cholesterol in tissues could be seen not only in reticuloendothelial and nervous systems, but also in all systems. Our case is important for it being the first case of NPC with submandibular lymphadenopathy characterized with NPC cell infiltration.


Subject(s)
Lymph Nodes/pathology , Niemann-Pick Disease, Type C/pathology , Child , Cholesterol , Female , Histiocytes/pathology , Humans , Inclusion Bodies/pathology , Mandible/pathology
5.
Pediatr Hematol Oncol ; 35(2): 131-144, 2018 Mar.
Article in English | MEDLINE | ID: mdl-30020823

ABSTRACT

The aim of the study was to investigate the expression and methylation status of seven distinctive genes with tumor suppressing properties in childhood and adolescent lymphomas. A total of 96 patients with Hodgkin Lymphoma (HL, n = 41), Non-Hodgkin Lymphoma (NHL, n = 15), and reactive lymphoid hyperplasia (RLH, n = 40, as controls) are included in the research. The expression status of CDKN2A, SPI1, PRDX2, DLEC1, FOXO1, KLF4 and DAPK1 genes were measured with QPCR method after the RNA isolation from paraffin blocks of tumor tissue and cDNA conversion. DNA isolation was performed from samples with low gene expression followed by methylation PCR study specific to promoter regions of these genes. We found that SPI1, PRDX2, DLEC1, KLF4, and DAPK1 genes are significantly less expressed in patient than the control group (p = 0.0001). However, expression of CDKNA2 and FOXO1 genes in the patient and control groups were not statistically different. The methylation ratios of all genes excluding the CDKN2A and FOXO1 were significantly higher in the HL and NHL groups than the controls (p = 0.0001). We showed that SPI1, PRDX2, DLEC1, KLF4 and DAPK1 genes are epigenetically silenced via hypermethylation in the tumor tissues of children with HL and NHL. As CDKN2A gene was not expressed in both patient and control groups, we conclude that it is not specific to malignancy. As FOXO1 gene was similarly expressed in both groups, its relationship with malignancy could not be established. The epigenetically silenced genes may be candidates for biomarkers or therapeutic targets in childhood and adolescent lymphomas.


Subject(s)
Death-Associated Protein Kinases/biosynthesis , Gene Expression Regulation, Neoplastic , Gene Silencing , Kruppel-Like Transcription Factors/biosynthesis , Lymphoma/metabolism , Peroxiredoxins/biosynthesis , Proto-Oncogene Proteins/biosynthesis , Trans-Activators/biosynthesis , Tumor Suppressor Proteins/biosynthesis , Adolescent , Child , Female , Humans , Kruppel-Like Factor 4 , Lymphoma/pathology , Male
6.
J Pediatr Hematol Oncol ; 39(6): 458-462, 2017 08.
Article in English | MEDLINE | ID: mdl-28697165

ABSTRACT

Vincristine is a widely used chemotherapeutic agent in the treatment of childhood malignancies. Neuropathy is the most common adverse effect. CYP3A4 and CYP3A5 enzymes of cytochrome p450 enzyme system are responsible in vincristine metabolism. Genetic polymorphism may alter the vincristine metabolism and the neurotoxicity rate. In this study, distribution of CYP3A5 alleles among Turkish children with malignancies, relation between CYP3A5 genotype and neurotoxicity rates, as well as severity and duration of neuropathy and total vincristine doses were investigated. Patient group consisted of 115 patients (age, 1 to 17 y) with acute lymphoblastic leukemia and solid tumors, who were treated with vincristine consisting chemotherapy protocols. Control group consisted of 50 children without any neurological symptom or disorders. All patient files were reviewed for presence and severeness of neurotoxicity symptoms. Blood samples were obtained and CYP3A5 genotypes were analyzed. Neurotoxicity occurred in 20.8% of patients. Although it was found to occur more frequently after 4 doses of vincristine, and rates were higher in the low-dose vincristine group suggesting other contributing factors. Although neurotoxicity rate in the CYP3A5*1/*3 genotype was 17.6%, it was 21.6% in the CYP3A5*3/*3 genotype and the difference was not statistically significant (P<0.05). This study suggested that vincristine-related neurotoxicity is dose-independent and genotype is not the only causative factor in the occurrence of neurotoxicity in these patients.


Subject(s)
Antineoplastic Agents, Phytogenic/adverse effects , Cytochrome P-450 CYP3A/genetics , Neoplasms/drug therapy , Neurotoxicity Syndromes/etiology , Precursor Cell Lymphoblastic Leukemia-Lymphoma/drug therapy , Vincristine/adverse effects , Adolescent , Antineoplastic Agents, Phytogenic/therapeutic use , Case-Control Studies , Child , Child, Preschool , Dose-Response Relationship, Drug , Genotype , Humans , Infant , Neoplasms/complications , Neoplasms/genetics , Precursor Cell Lymphoblastic Leukemia-Lymphoma/complications , Precursor Cell Lymphoblastic Leukemia-Lymphoma/genetics , Turkey , Vincristine/therapeutic use
9.
Pediatr Hematol Oncol ; 33(3): 178-85, 2016 Apr.
Article in English | MEDLINE | ID: mdl-26984313

ABSTRACT

The aim of this study was to determine subclinical atherosclerosis and endothelial functional disturbance with measurement of carotid intima-media thickness (IMT), brachial artery reactivity (BAR), and levels of serum adhesion molecules in children with solid tumors who were treated with anthracyclines and are actually in complete remission. Fifty patients who were in remission and 30 healthy children were included in the study. Mean ages of patient and control groups were 13.5 ± 4.7 years (range: 3-23 years) and 12.00 ± 4.3 years (range: 4-21 years), respectively. The patients were divided into 3 groups according to cumulative doxorubicin dose: Group 1, ≤100 mg/m(2); Group 2, 101-299 mg/m(2); Group 3, ≥300 mg/m(2). The BAR and carotid IMT were measured in order to determine the endothelial function. The serum adhesion molecule levels in our patients and controls were also measured. The BAR of the patients with cumulative anthracycline dose ≥300 mg/m(2) was significantly lower than the patients with cumulative anthracycline dose ≤100 mg/m(2) and healthy controls (P =.005 and P =.003, respectively). Also, there was a negative correlation between brachial artery reactivity and increasing cumulative anthracycline dose (r = -.287, P =.044). We also found significant difference between the mean carotid IMT of the patients and the healthy children (P =.041). No statistically significant difference was detected between the serum levels of sICAM-1 (soluble intercellular adhesion molecule-1), sVCAM-1 (soluble vascular cell adhesion molecule-1), sE-selectin of the patients and controls. The use of anthracyclines in pediatric patients with cancer could result in increase of the carotid IMT and endothelial dysfunction.


Subject(s)
Anthracyclines/therapeutic use , Brachial Artery/physiopathology , Carotid Intima-Media Thickness , Cell Adhesion Molecules/blood , Neoplasms/drug therapy , Adolescent , Adult , Child , Child, Preschool , E-Selectin/blood , Female , Humans , Intercellular Adhesion Molecule-1/blood , Male , Neoplasms/pathology , Neoplasms/physiopathology , Vascular Cell Adhesion Molecule-1/blood
10.
Pediatr Hematol Oncol ; 32(6): 374-81, 2015.
Article in English | MEDLINE | ID: mdl-26269940

ABSTRACT

Breastfeeding is well-known to have a protective effect against infection in infants. It has been suggested that breast milk may play a role in the prevention of certain childhood cancer. We investigated this issue in a case-control study comprising 300 patients with childhood cancer. There was 73 patients (24.3%) with leukemia, 82 patients (27.3%) with lymphoma, and 146 patients (48.4%) with solid tumors (brain tumors, neuroblastoma, soft tissue sarcomas, germ cell tumors, renal tumor, bone tumor, retinoblastoma, hepatoblastoma, and others) and 316 controls matched for age and sex. Breastfeeding duration of the control group was found to be significantly longer than the patient group (X(2) = 57.774; P < .001). In conclusion, breastfeeding was found to be inversely associated with pediatric cancer in our study.


Subject(s)
Breast Feeding , Neoplasms/epidemiology , Adolescent , Case-Control Studies , Child , Child, Preschool , Female , Humans , Infant , Male , Neoplasms/prevention & control
11.
J Pediatr Hematol Oncol ; 36(5): e328-32, 2014 Jul.
Article in English | MEDLINE | ID: mdl-24608073

ABSTRACT

Fibrolamellar variant of hepatocellular carcinoma (FLHCC) does not have a favorable prognosis than conventional HCC, and there is no difference regarding the response to chemotherapy and the degree of surgical resectability. FLHCC commonly recurs after complete surgical resection, and there is a high rate of lymph node metastases. Herein, we report a 12-year-old girl with metastatic FLHCC with multiple recurrences aggressively treated with surgery, chemotherapy, and antiangiogenic agents. She is in complete remission after 4 years and 2 months after the diagnosis of metastatic FLHCC. The standard treatment of FLHCC is excision of the primary tumor and its metastases. Chemotherapy for FLHCC is controversial, and it has been suggested that cytoreductive chemotherapy was ineffective and adjuvant chemotherapy did not improve survival. Our patient with multiple recurrences was successfully treated with surgery, first-line chemotherapy with cisplatin and doxorubicin, second-line chemotherapy with 5-fluorouracil/interferon-α combination, and adjuvant antiangiogenic agents like cyclophosphamide and thalidomide. As FLHCC patients have no underlying liver disease, they can tolerate higher doses of chemotherapy compared with conventional HCC patients. We support the use of repeated aggressive surgery with adjuvant chemotherapy and antiangiogenic therapy, which provided complete remission in our patient with metastatic and recurrent FLHCC.


Subject(s)
Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Carcinoma, Hepatocellular/therapy , Liver Neoplasms/therapy , Neoplasm Recurrence, Local/therapy , Carcinoma, Hepatocellular/secondary , Child , Cisplatin/administration & dosage , Combined Modality Therapy , Cyclophosphamide/administration & dosage , Doxorubicin/administration & dosage , Female , Fluorouracil/administration & dosage , Hepatectomy , Humans , Interferon-alpha/administration & dosage , Liver Neoplasms/pathology , Lymphatic Metastasis , Neoplasm Recurrence, Local/pathology , Prognosis , Remission Induction , Thalidomide/administration & dosage
12.
Pediatr Hematol Oncol ; 30(3): 226-38, 2013 Apr.
Article in English | MEDLINE | ID: mdl-23363243

ABSTRACT

Anthracyclines can cause severe cardiac toxicity leading to heart failure. The aim of this study was to determine the effects of cardioprotective polyphenolic compound resveratrol (RES) and adipose-derived mesenchymal stem cells (ADMSCs) on cardiac tissue of rats treated with doxorubicin (DOX). Forty-two female and three male Wistar-Albino rats were included in the study. The study groups and the control groups were as follows: Group I: DOX; Group II: DOX + RES; Group III: DOX + ADMSCs; Group IV: DOX + RES + ADMSCs; Group V: Sham operation; and Group VI: normal saline. ADMSCs obtained from male rats were defined with stem cell markers [CD11b/c(-), CD45(-), CD90(+), CD44(+), and CD49(+)]. DOX 12 mg/kg intraperitoneally (i.p.) was injected as a single dose in female rats. Resveratrol 100 mg/kg was injected three times i.p. in Groups II and IV. ADMSCs 2 × 10(6) cells/kg/dose were labeled with bromodeoxyuridine (BrdU) and injected i.p. for a total of three times in Groups III and IV. When the study was terminated after 4 weeks, the beating hearts were connected to a Langendorff setup and records were obtained for 30 minutes. Histopathological, immunhistochemical, and immunofluorescent examination with H&E, Troponin I, and BrdU stains were also performed. Also, ADMSCs were demonstrated in the myocardium of transplanted rats. Left ventricle functions and myocardial histology demonstrated significant impairment in DOX only group compared to groups with ADMSCs (P < .05). We suggest that RES and ADMSCs were successful in the prevention and treatment of the doxorubicin cardiomyopathy in rats. The hypothetical mechanisms of regeneration are multiple, including cell differentiation and autocrine/paracrine effects of ADMSCs.


Subject(s)
Adipose Tissue , Antibiotics, Antineoplastic/toxicity , Doxorubicin/toxicity , Heart Diseases/prevention & control , Mesenchymal Stem Cell Transplantation , Mesenchymal Stem Cells , Stilbenes/therapeutic use , Animals , Cell Proliferation , Female , Heart Diseases/chemically induced , Male , Rats , Rats, Wistar , Resveratrol
13.
Turk J Pediatr ; 65(5): 801-808, 2023.
Article in English | MEDLINE | ID: mdl-37853971

ABSTRACT

BACKGROUND: The spinal cord compression causes irreversible long-term permanent neurological sequelae. This study aims to increase awareness of childhood cancers that cause cord compression by comparing histopathological diagnosis, treatments, and survival rates to the literature. METHODS: Seventy-three patients (38 male, 35 female) with spinal cord compression, among 1085 patients diagnosed with solid tumors at Gazi University Department of Pediatric Oncology between 1991 and 2021 were retrospectively evaluated. RESULTS: The mean time between the onset of complaints and diagnosis was 27.5± 24.9 (2-150) days. The first three most common tumors that caused cord compression; were central nervous system tumors in 22 (30%), neuroblastoma in 17 (23%), and malignant germ cell tumors in 8 (10%) cases. Of the patients, 46 (63%) had compression due to extradural masses, and 27 (37%) patients had an intradural compression. The most common symptoms were pain in 60 (82%), weakness in 57 (78%), and pins and needles in 28 (38%) patients, respectively. The clinical physical neurological examination findings were motor deficit in 62 (84%), and deep tendon reflex changes in 54 patients (73.9%). Compression findings were detected in 58 (79.5%) patients at diagnosis, and in 15 (20.5%) of them during follow-up. The most common level of compression was seen in the thoracolumbar region in 19 (26%) cases. In 65 (89%) patients with cord compression, corticosteroids were given as anti-edema treatment. Surgical excision was performed in 39 (53%) patients. Spinal radiotherapy was given to 35 patients (48%) with radiosensitive tumors. Chemotherapy protocols were started in 52 (71.2%) cases according to their diagnoses. Complete neurological recovery was achieved in 33 (45%) patients. The 5-year overall survival rates for solid tumors with extradural compression and intradural compression were 62% and 22%, respectively (p=0.002). CONCLUSIONS: Neurological sequela-free recovery is possible with early diagnosis and urgent treatment. Spinal compression must be detected by detailed systemic and neurological examination and imaging methods. Patients should be rapidly transferred to pediatric oncology units after starting anti-edema treatment.


Subject(s)
Neuroblastoma , Spinal Cord Compression , Humans , Male , Child , Female , Spinal Cord Compression/etiology , Spinal Cord Compression/therapy , Spinal Cord Compression/diagnosis , Retrospective Studies , Neuroblastoma/complications , Neuroblastoma/therapy , Pain
14.
Int J Ophthalmol ; 16(8): 1274-1279, 2023.
Article in English | MEDLINE | ID: mdl-37602348

ABSTRACT

AIM: To assess the clinical and genetic characteristics of children diagnosed with retinoblastoma (RB) at Gazi University Faculty of Medicine's Department of Pediatric Oncology. METHODS: All cases diagnosed with RB and received treatment and follow-up in the Ophthalmology and Pediatric Oncology Department, October 2016 to May 2021 were evaluated retrospectively. The RB1 gene was analyzed by next-generation sequencing (NGS) technique in DNAs obtained from peripheral blood samples of the patients. RESULTS: This study included 53 cases with 67 RB-affected eyes during the study period. The mean age was 24.6 (median: 18.5, range: 3-151)mo. There were 15 (22.3%) Group D eyes and 39 (58.2%) Group E eyes. The RB1 gene was sequenced by the NGS method in 19 patients. Heterozygous RB1:NM_000321.3: c.54_76del (p.Glu19AlafsTer4) variant was detected in a 15-month-old female with bilateral RB. Heterozygous RB1:NM_000321.3: c.1814+3A>T variant was detected in a 5.5-month-old male with bilateral RB. The intronic RB1:NM_000321.3: c.1332+4A>G variant was detected in patient 14, a 13-month-old male with unilateral RB. The RB1:NM_000321.3: c.575_576del (p.Lys192SerfsTer10) variant was found in an 18-month-old female with an allele frequency of 37%. These variants have not been reported in the literature and mutation databases. CONCLUSION: Four novel variants are described and one of them is found in two different patients. This data is crucial for assessing prognosis. It serves as a guide for estimating the long-term risk of secondary malignancy as well as the short-term risk of developing additional malignancies in the same eye and the other eye.

15.
Transfus Apher Sci ; 47(1): 101-6, 2012 Aug.
Article in English | MEDLINE | ID: mdl-22592083

ABSTRACT

Major bleeding is a life threatening complication of severe thrombocytopenia. The aim of this study was to find out the indications and the threshold for platelet transfusions in the pediatric patients of our hospital throughout 1 year. Records of the hospital's blood bank and the files of the patients were retrospectively reviewed. One hundred and four patients, between ages 0-18 years received 378 platelet units. Pretransfusion platelet counts were found to be significantly lower in hematology-oncology groups compared to other clinics (p<0.05). Single donor apheresis was found to be the major source of platelets in hematology (80.8%, n=147) and oncology (86.5%, n=45) clinics. There is a tendency for using apheresis products without proven superiority compared to platelet concentrates in terms of efficacy. This practice can be abandoned by continuous education.


Subject(s)
Hemorrhage/prevention & control , Platelet Transfusion , Thrombocytopenia/therapy , Adolescent , Child , Child, Preschool , Female , Hemorrhage/blood , Hemorrhage/etiology , Humans , Infant , Infant, Newborn , Male , Platelet Count , Retrospective Studies , Thrombocytopenia/blood , Thrombocytopenia/complications
16.
Pediatr Hematol Oncol ; 29(8): 686-90, 2012 Nov.
Article in English | MEDLINE | ID: mdl-23013405

ABSTRACT

The hypereosinophilic syndromes (HES) are characterized by prolonged nonreactive peripheral blood hypereosinophilia with tissue damage. The lymphocytic HES variant can precede malignant clonal T-cell disease in adults but it is extremely rare to be the presenting feature of lymphomas in children. Here we present a 2.5-year-old boy with HES and mediastinal T-cell anaplastic lymphoma kinase (ALK) negative systemic anaplastic large-cell lymphoma. Mature and immature eosinophils without blasts were shown on bone marrow aspiration while biopsy revealed malignant infiltration. The patient responded well to initial corticosteroid therapy, but high-risk features make a challenge of finding the cure in this extremely rare case.


Subject(s)
Eosinophils/pathology , Hypereosinophilic Syndrome/pathology , Lymphoma, Large-Cell, Anaplastic/pathology , Mediastinal Neoplasms/pathology , Receptor Protein-Tyrosine Kinases , Adrenal Cortex Hormones/administration & dosage , Anaplastic Lymphoma Kinase , Biopsy, Fine-Needle , Child, Preschool , Humans , Hypereosinophilic Syndrome/drug therapy , Lymphoma, Large-Cell, Anaplastic/drug therapy , Male , Mediastinal Neoplasms/drug therapy
17.
Contemp Oncol (Pozn) ; 16(1): 26-33, 2012.
Article in English | MEDLINE | ID: mdl-23788851

ABSTRACT

AIM OF THE STUDY: Although the survival for children with certain central nervous system (CNS) tumour types has improved through current surgical and adjuvant treatment modalities, the prognosis of many high-grade tumours remains poor despite aggressive treatment. The aim of this study is to analyse patients with high-grade brain tumours in our institution to determine the histopathology, clinical characteristics, treatment modalities, and survival. MATERIAL AND METHODS: A total of 74 patients with a diagnosis of high-grade brain tumour were analysed. There were a total of 31 patients with embryonal tumours, 27 patients with high-grade glial tumours, 12 patients with brain stem gliomas and 4 patients with other high-grade brain tumours. RESULTS: There were 48 (65%) boys and 26 (35%) girls (ratio: 1.85) with a median age of 99.7 months (range = 2-204 months). The median follow-up period was 19 months (range = 1-204 months). Tumour recurrence was observed in 38 patients (51.4%). The overall survival rate and event-free survival rate of our patients were 27% and 19.5%, respectively. CONCLUSIONS: Pediatric high-grade CNS tumours have a very aggressive behaviour and a significant number of children eventually succumb to disease despite multimodal treatment. There is a need of more effective therapeutic approaches for these tumours with poor prognosis. The future improvement in childhood high-grade brain tumour management depends on a better understanding of the molecular genetics and biology of brain tumours.

18.
Nucl Med Commun ; 43(7): 778-786, 2022 Jul 01.
Article in English | MEDLINE | ID: mdl-35506271

ABSTRACT

PURPOSE: The aim of this study was to evaluate the prognostic value of PET-derived metabolic features and textural parameters of primary tumors in pediatric sarcoma patients. METHODS: The imaging findings of 43 patients (14 girls and 29 boys; age 11.4 ± 4.4 years) who underwent 18-fluorodeoxyglucose positron emission tomography (PET)/computed tomography for primary staging prior to therapy between 2005 and 2020 were retrospectively evaluated. The diagnoses were osteosarcoma in 10, rhabdomyosarcoma in 10, and Ewing sarcoma in 23 patients. PET metabolic data and textural features of primary tumors were obtained. Cox proportional hazards regression models were used to identify predictors for progression-free survival and overall survival. Survival curves were estimated by using the Kaplan-Meier method. RESULTS: Distant metastases were detected in primary staging in 13 patients (30.2%). The median follow-up duration after diagnosis was 28 months (range: 10-171 months). In multivariate Cox regression analysis, the presence of distant metastasis and neighborhood grey-level difference matrix_Contrast (ngldm_Contrast) were found as independent predictors for both progression-free survival and overall survival. Grey-level zone length matrix_Zone-length nonuniformity (glzlm_ZLNU) was also found as an independent predictor for overall survival. The Kaplan-Meier survival analysis showed that higher ngldm_Contrast and glzlm_ZLNU values of primary tumors were significantly associated with shorter progression-free survival and overall survival. CONCLUSION: In addition to the presence of distant metastasis at initial diagnosis, textural features of primary tumors may be used as prognostic biomarkers to identify patients with worse prognosis in pediatric sarcoma. Higher tumor heterogeneity is significantly associated with shorter progression-free survival and OS.


Subject(s)
Positron-Emission Tomography , Sarcoma , Adolescent , Child , Female , Fluorodeoxyglucose F18 , Humans , Male , Positron Emission Tomography Computed Tomography/methods , Prognosis , Radiopharmaceuticals , Retrospective Studies , Sarcoma/diagnostic imaging
19.
Turk J Pediatr ; 63(5): 758-766, 2021.
Article in English | MEDLINE | ID: mdl-34738358

ABSTRACT

BACKGROUND: Programmed death 1 (PD-1) is a co-receptor which is located at the surface of cells like natural killer, monocytes, T and B cells. It has two ligands including programmed death ligand-1 (PD-L1) and ligand-2 (PD-L2). T cell functions are inhibited by activation of PD-1/PD-L1 pathway and this pathway is used by viruses and some tumor cells in order to escape from immune eradication. In our study we evaluated PD-L1 expression in the tissue specimens of patients with Wilms tumor, neuroblastoma and other renal tumors. METHODS: Totally 60 patients who were followed up at Gazi University Hospital with the diagnosis of neuroblastoma, Wilms tumor and other renal tumors were included. PD-L1 expression was examined in tumor samples of the patients. RESULTS: Positive staining with PD-L1 was detected only in two male patients. Both of them had neuroblastoma and advanced stage disease. None of the patients with Wilms tumor and other renal tumors had positive PD-L1 staining. CONCLUSIONS: Unlike adult tumors, PD-L1 expression is not common in childhood tumors due to differences in immune system between children and adults. Further studies are needed to establish the importance and effects of PD-1/PD-L1 pathway in pediatric tumors.


Subject(s)
Kidney Neoplasms , Neuroblastoma , B7-H1 Antigen , Humans , Male , Programmed Cell Death 1 Receptor
20.
Turk J Pediatr ; 63(3): 434-442, 2021.
Article in English | MEDLINE | ID: mdl-34254488

ABSTRACT

BACKGROUND: Fever is one of the leading causes of hospital admissions in children. Although there are many ways to measure body temperature, the optimal method and the anatomic site are still controversial. In this study, we aimed to evaluate the performance of new methods of measuring body temperature and to compare the accuracy, sensitivity and specificity of these methods. METHODS: The body temperatures of the patients who were hospitalized as inpatients or who presented to the emergency room as outpatients between November 2014- March 2015 were measured and recorded. Mercury and digital axillary measurements, tympanic, temporal artery and non-contact skin temperatures were measured. Measurements were compared with each other. RESULTS: According to our results temperature tends to increase over time for up to 8 minutes after placement when using axillary thermometers. Non-contact skin thermometers should be used only for follow-up of patients with fever, because of their low sensitivity and low negative predictivity. At the first examination, tympanic thermometers and axillary thermometers may be preferable for the diagnosis of fever. CONCLUSIONS: According to our results, using non-contact thermometers seems feasible and logical during the follow-up ofpatients with fever, but not in cases whose exact body temperature should be known. For the first examination of the patient to diagnose fever, tympanic thermometers and axillary thermometers may be preferable. Future studies are warranted to expose the optimum way of measuring body temperature in children.


Subject(s)
Body Temperature , Thermometers , Axilla , Child , Fever/diagnosis , Humans , Sensitivity and Specificity , Tympanic Membrane
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