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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, = 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

Background The aim of this study was to compare the nephrotoxicity risk of cisplatin (CPL) and ifosfamide (IFO) combination treatment (CT) with that of CPL alone and to evaluate the prevalence of CPL-induced long-term nephrotoxicity in pediatric cancer survivors (CS). Methods A total of 33 patients with pediatric solid tumors who have been cured of their disease were included in the study. They were divided into two groups based on the type of chemotherapeutics, either CPL (n = 21) or CT (n = 12), given during cancer treatment and were evaluated for glomerular and tubular function using the Skinner grading system. Results Nephrotoxicity was found in 15 CS (45.4%): seven (21.3%) of those had moderate, six (18.2%) had mild, and two (6.1%) had severe nephrotoxicity. Neither the rates of overall nephrotoxicity, glomerular toxicity and tubular toxicity, nor the mean overall, glomerular and tubular toxicity scores differed significantly among the CPL and CT groups (P > 0.05 for all parameters). Cumulative IFO dose and age at treatment were found to be independent risk factors for both development and severity of CPL-induced nephrotoxicity (P = 0.025 and P = 0.036 for development of nephrotoxicity; P = 0.004 and P = 0.050 for severity of nephrotoxicity, respectively). Conclusions Although CPL-induced long-term nephrotoxicity was found in half of the pediatric CS of solid tumors, clinically significant nephrotoxicity was detected only in a minority of them. Both higher cumulative IFO dose and younger age at treatment were found to be independent risk factors for both development and severity of CPL-induced nephrotoxicity.

Pinarli, Faruk Guclu/0000-0002-3241-2478; Albayrak, Meryem/0000-0003-2711-5150 WOS: 000406231400018 PubMed: 28697165 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

Thymus gland involvement in Langerhans cell histiocytosis (LCH) is usually part of multi-system disease and may be more common than previously recognized. However, thymic involvement causing an anterior mediastinal mass is an extremely rare presentation of multisystem LCH. Here we report a 2-month-old-boy admitted to hospital with a giant anterior mediastinal mass with multisystem LCH involving the thymus, lungs, liver and skin. The differential diagnosis of mediastinal mass in children should also include LCH, especially multisystem disease. LCH should also be kept in mind in the differential diagnosis of skin lesions in infants, even if spontaneous regression occurs.

Ob­jec­ti­ve: To investigate the late side effects of childhood cancer therapy on the thyroid gland and to determine the risk factors for development of thyroid disorder among childhood cancer survivors. Methods: One hundred and twenty relapse-free survivors of childhood cancer (aged 6-30 years) were included in this study. The diagnoses of patients were lymphoma, leukemia, brain tumor, rhabdomyosarcoma and nasopharyngeal carcinoma (NPC). The patients were divided into two groups depending on the treatment: group 1-chemotherapy (ChT) only (n=52) and group 2-combination therapy of ChT + radiotherapy (RT) (head/neck/thorax) (n=68). Thyroid function tests, urinary iodine levels, and thyroid gland ultrasound examinations were evaluated in both groups. Results: Incidence of thyroid disease was 66% (n=79) in the survivors. The thyroid abnormalities were: hypothyroidism (HT) (n=32, 27%), thyroid nodules (n=27, 22%), thyroid parenchymal heterogeneity (n=40, 33%), autoimmune thyroiditis (n=36, 30%), and thyroid malignancy (n=3, 2%). While the incidence of HT and thyroid nodules in group 2 was significantly higher than in group 1, the incidence of thyroid parenchymal heterogeneity and autoimmune thyroiditis was similar in the two patient groups. HT and thyroid malignancy were seen only in group 2. In multivariate logistic regression analysis, a history of Hodgkin lymphoma (HL), brain tumor and NPC, as well as cervical irradiation and 5000-5999 cGy doses of radiation were found to constitute risk factors for HT. History of HL and 4000-5999 cGy doses of radiation were risk factors for thyroid nodules. Head/neck irradiation and treatment with platinum derivatives were risk factors for autoimmune thyroiditis. In univariate analysis, a history of NPC, cervical + nasopharyngeal irradiation, and treatment with platinum derivatives were risk factors for thyroid parenchymal heterogeneity. Conclusion: Our results indicate that there is especially an increased risk of HT and thyroid nodules in patients treated with combination therapy of ChT with head/neck/thorax RT. Although chemotherapeutic agents per se do not seem to cause HT, longer follow-up is needed to assess whether or not there is an increased risk for autoimmune thyroiditis and thyroid parenchymal heterogeneity after antineoplastic therapy.

Sertoli–Leydig cell tumors are rare sex cord-stromal neoplasms that account for

Background A number of clinical studies conducted in adults have demonstrated the prognostic significance of angiogenic factors in malignancies, however, only a limited number of studies have been conducted in children. The aim of this study was to determine serum vascular endothelial growth factor (VEGF), endostatin, and leptin levels in children with lymphoma and to investigate whether these factors provide prognostic information. Procedure Serum samples from 36 children with lymphoma (non-Hodgkin lymphoma (NHL) N = 21, Hodgkin lymphoma (HL) N = 15) were collected at diagnosis and during remission. Serum samples were also collected from 18 healthy children as the control group. Serum VEGF and endostatin levels were quantified by using enzyme-linked immunosorbent assay (ELISA) and serum leptin by immunoradiometric assay. Results The serum VEGF levels were found elevated in patients compared to controls (P = 0.033), while endostatin and leptin levels were lower in patients than in controls (endostatin, 43.9 ± 5.8 ng/ml vs. 123.6 ± 13.5 ng/ml, P

Aims Asymptomatic long-term cancer survivors treated with anthracycline were investigated for late anthracycline cardiotoxicity using dobutamine stress echocardiography (DSE) and tissue Doppler (TD) velocities. Methods and results The study comprised 20 asymptomatic patient and 18 healthy children as the control group. Twenty patients were divided into two groups according to the myocardial wall motion during DSE: Group 1 (normal myocardial wall motion; six girls and five boys) and Group 2 (abnormal myocardial wall motion: nine boys). Intravenous dobutamine infusion was started at a dose of 5 µg/kg/min (D5) and gradually increased to 10 (D10), 15 (D15) and 20 µg/kg/min (D20). Echocardiographic assessment was performed at rest and after each dose of dobutamine infusion. Abnormal myocardial wall motion was observed at rest in 3 patients and during DSE in six patients. There were no significant differences between the patients and control groups at rest except the end systolic wall stress and mitral deceleration time measured by conventional methods; however, both patients group showed significant differences of systolic and diastolic functions at D20. In patients groups, systolic and diastolic functions of interventricular septum (IVS) and systolic function of left ventricle (LV) and right ventricle (RV) TD velocities showed significant changes compared with control group at rest. Significant differences of diastolic functions of IVS and RV were noted during dobutamine infusion in abnormal myocardial wall motion compared with other groups. Conclusion LV, RV and IVS TD velocities systolic function at rest and during DSE can provide valuable information for early detection of subclinical cardiac toxicity. TD velocities of diastolic functions during DSE are a valuable parameter for assessment of subclinical cardiac toxicity in patient with abnormal wall motion.

OBJECTIVE: Long-term survivors of asymptomatic children treated with anthracycline may have cardiac toxicity without clinical findings. The subclinical cardiac toxicity could be evaluated by dobutamine stress echocardiography (DSE) with exploring effective and safe doses of dobutamine. METHODS: Twenty asymptomatic survivors (mean age: 19.2+/-4.0 years) treated with cumulative dose of 282.1+/-125.9 mg/m2 of anthracycline were compared with 18 age-matched healthy volunteers. Total time completed this treatment was 10.2+/-2.2 years. This was a cross-sectional case-controlled study and patient and control groups were evaluated at the time of routine appointments. Echocardiographic studies were performed before and after each dobutamine infusion of 5, 10, 15, 20 microg/kg/min. STATISTICAL ANALYSIS: Mann-Whitney U test was used to evaluate the difference between the groups. ANOVA for repeated measurements test was used to compare each measurement of control and patients groups and Bonferroni posthoc test was used for correction. RESULTS: Hemodynamic changes are observed at the dobutamine doses of 15 microg/kg/min in the patient group. Before dobutamine infusion in the patient group only isovolumic relaxation and contraction times values were prolonged comparing to the control group. After the infusion of dobutamine ejection fraction, shortening fraction, left ventricular posterior wall thickening (%LVPWt), end-systolic wall stress (ESS), interventricular septum systolic thickening, left ventricular end-systolic and end-diastolic diameters, mitral acceleration (AT) and deceleration times values were deteriorated in the patient group compared to the control group (p=0.05 for all). The highest differences between the groups were observed in the %LVPWt, ESS and AT values at the end of test. CONCLUSION: The DSE is an effective and safe method to demonstrate the late anthracycline cardiotoxicity. Echocardiographic evaluation should be made at rest and dobutamine dose of 20 microg/kg/min. In the early diagnosis of late cardiac toxicity; assessment of %LVPWt, AT and ESS values in addition to standard echocardiographic examination could be the guidance for early diagnosis of late cardiac toxicity.

melikoglu, mustafa/0000-0001-9646-3787; Aydin, Burca/0000-0002-6013-6271 WOS: 000278808800001 PubMed: 20367260 Aim: To standardize diagnosis and treatment of childhood Wilms tumor (WT) in Turkey. Methods and patients: Between 1998 and 2006, WT patients were registered from 19 centers. Patients 2 years had significantly more advanced disease. 1/11 cases with recurrent disease died; 2/165 had progressive disease, 2/165 had secondary cancers, and all 4 died. In all cases 4-year OS and EFS were 92.8 and 86.5%, respectively. Both OS and EFS were significantly worse in stage IV. Conclusions: Despite problems in patient management and follow-up, treatment results were encouraging in this first national experience with a multicentric study in pediatric oncology. Revisions and modifications are planned to further improve results and minimize short- and long-term side effects.

The purpose of this study was to define the role of tissue and flow myocardial performance index (MPI) measured during dobutamine stress and tissue Doppler echocardiography in the early diagnosis of late cardiotoxicity among patients with childhood cancer treated with anthracycline. The study included 20 patients (14 male and 6 female; mean age 18.4 +/- A 3.2 years) as the study group and 18 healthy volunteers (14 female and 4 male, mean age: 19.2 +/- 4.0) as the control group. The mean cumulative dose of anthracycline treatment was 282.1 +/- A 125.9 mg/m(2), and the mean time period after the last dose of anthracycline was 10.2 +/- A 4.0 years. Echocardiography was performed during rest and at infusions of 5, 10, 15, and 20 mu/kg/min dobutamine. Although only isovolumetric relaxation and contraction times of the patient group were prolonged at rest, dobutamine infusion showed significant differences in % left ventricle (LV) posterior wall thickening, LV end-systolic wall stress, LV diastolic and systolic diameter, mitral acceleration, and deceleration time in the patient group compared with the control group. Tissue and flow MPI of the LV, tissue MPI of the right ventricle (RV), and interventricular septum of the patient group were higher than the control group throughout the test. LV tissue MPI increased much more than LV flow MPI when stress was increased. In conclusion, LV tissue MPI value during stress is more valuable than LV flow MPI in the early diagnosis of late cardiotoxicity. RV function can be assessed by tissue Doppler MPI.

This immunohistochemical study was carried out to evaluate the role of vascular endothelial growth factor (VEGF), matrix metalloproteinase-2 and -9 (MMP-2 and MMP-9), their inhibitor (tissue inhibitor of metalloproteinase-1, TIMP-1), and microvessel density (MVD) in the clinicopathologic behavior of childhood Hodgkin's lymphoma (HL). Paraffin-embedded histologic sections from 15 children with HL were immunohistochemically stained with MMP-2, MMP-9, TIMP-1, VEGF, and CD31 antibodies to investigate the correlation between the expression of these markers and the clinicopathologic characteristics of HL. Expression of MMP-2 and VEGF in Hodgkin and Reed–Sternberg cells (HRS) was more frequent in nodular sclerosis than in other subtypes (p=0.07 and 0.08, respectively). None of the study parameters in HRS cell were associated with age, sex, disease stage, extranodal disease, and the occurrence of bulky tumor. There was a trend toward advanced stage in negative TIMP-1 staining in HRS cells (p=0.06). In reactive lymphocytes, MMP-2 expression was correlated with MVD (r=0.68, p=0.005), and MMP-9 expression was correlated with B symptoms (p=0.003). Also, low TIMP-1 expression in reactive lymphocytes was frequently found in patients with advanced stage (p=0.048). There was a positive correlation with the ratio of MMP-2 expression in reactive lymphocytes and MVD (r=0.68, p=0.005). Expression of MMP-9 in reactive lymphocytes was correlated with MVD without statistical significance (r=0.487, p=0.06). Our results suggest that, as in many solid tumors, angiogenesis and angiogenic factors may play an important role in childhood HL. Larger series of patients are needed to determine the prognostic value of angiogenesis in childhood HL.

The present study was carried out to clarify the role of matrix metalloproteinase-2 and -9 (MMP-2 and MMP-9), tissue inhibitor of metalloproteinase-1 (TIMP-1), vascular endothelial growth factor (VEGF), and microvessel density (MVD) in the clinicopathologic behavior of childhood B-cell non-Hodgkin lymphoma (NHL). Paraffin-embedded biopsy specimens from 25 children with NHL were studied by immunohistochemically and the correlate the expression of these markers to clinicopathologic characteristics. Positive MMP-9 staining was associated with an increased prevalence of B-symptoms (p = .046). High microvessel density (MVD) showed a tendency toward an adverse outcome and it was correlated with clinical stage (p = .023). The event-free survival of high MVD patients was less than for those with low MVD, but the difference was not statistically significant (64.1% vs. 85.71% respectively, p = 0.15). The overall survival of high MVD patients was less than for those low with MVD and the difference was statistically significant (55.53% vs. 100% respectively, p = .039). Neither gelatinases nor VEGF correlated with age, sex, disease stage, the occurrence of bulky disease, or extranodal disease. The results showed that angiogenesis and angiogenic factors might have a role in development and clinical behavior of childhood NHL. Larger series of patients are needed to determine the prognostic value of angiogenesis in childhood non-Hodgkin lymphoma.

Background Important in the cancer therapy is the increasingly use of complementary/alternative medicine (CAM). The current study aims to establish the extent of use, the types of therapies employed, factors influencing, the reasons for choosing and the cost of CAM used in pediatric cancer patients in our clinic in Ankara, Turkey. Procedure A detailed questionnaire regarding demographic data and information about the use of CAM was completed by 95 patients who have attended the clinic between 1999 and 2000. Results Forty-nine patients (51.6%) had used one or more than one type of CAM. The most frequent (71.4%) was herbal medicine and biologic intake (stinging nettle, plant essence, honey of Anzer). The second one was religious therapy (40.8%). No correlation could be found between the use of CAM and parents education status, the level of income, number of siblings, the prognosis of the disease, the rate of satisfaction with the level of information given by the doctor at the time of diagnosis or the belief in the information given by the doctor about the prognosis (P > 0.05). All patients used CAM in addition to conventional therapy. Conclusions More than half of the patients used CAM, with stinging nettle being the most common agent. Our patients place a high level of trust in their physicians and the conventional therapies offered by them; however, they also believe that the complementary therapies are harmless and, therefore, worth trying. Pediatr Blood Cancer 2007;48:540–543. © 2006 Wiley-Liss, Inc.

Nodular regenerative hyperplasia (NRH) and focal nodular hyperplasia (FNH) of the liver rarely occur in children after completion of tumor therapy. These lesions mimic hepatic metastasis and they must be distinguished from metastatic lesions. The authors present 2 children, one with NRH and one with FNH, after undergoing antineoplastic therapy for non-hepatic childhood solid tumors and discuss their patients in the context of the literature.

In recent years, there have been reports that glutamine support improves immune functions in adult patients with malignancy, but there is a lack of data in children. Oral glutamine support of 4 g/m(2)/day was given to 21 children with various solid tumors, aged 1-17 years (9.86 +/- 5.38) for all 5 days of a chemotherapy course. The same parameters in another course of the same protocol without glutamine supplementation were considered as controls. There were significant improvements of some nutritional and immunological parameters in the glutamine-supplemented course. Also glutamine seemed to reduce antibiotic necessity. Oral glutamine supplementation could be considered in children with solid tumors receiving chemotherapy.

Pinarli, Faruk Guclu/0000-0002-3241-2478 WOS: 000231623800008 PubMed: 16007600 Background. The goals of this study included: (1) Identification of factors prognostic for event-free survival (EFS) and overall survival (OS), and (2) Definition of risk groups for risk adapted therapy in children with Hodgkin disease (HD). Procedure. From 1991 to 2003, 69 children with newly diagnosed, untreated biopsy-proven stage I-IV HD were treated with chemotherapy (CT) and low-dose involved field radiotherapy (LD-IFRT). The relationship of pretreatment factors to EFS and OS was analyzed by univariate and multivariate analysis. Results. The 5-year EFS and OS for all patients were 90.77% and 96.22%, respectively with a median follow-up of 73 months (3-137 months). Male to female ratio was 3:1 and 21 children (32.3%) were less than 7 years of age. Mixed cellularity was the predominant histologic subtype (38.5%). Factors associated with inferior EFS by univariate analysis were extranodal disease, hemoglobin level < 11 g/dl, number of involved lymph node regions and stage. By multivariate analysis only stage IV disease was significant. Conclusion. Our study confirms that excellent results are achievable with combined modality therapy in childhood HID. In order to use risk-adapted therapy in children with HID, clinical prognostic factors should be validated with large, multicentered prospective clinical studies.

Anterior mediastinal and adenoid masses in children after cessation of chemotherapy for malignant disease open cause a diagnostic problem. Differential diagnosis of thymic enlargement and adenoid hyperplasia from recurrence frequently poses a challenge both for the radiologist and the physician. In this study the authors evaluated, 491 patients with different malignant tumors for thymic and adenoid hyperplasia. Thymic hyperplasia was seen in 18 patients (5 Hodgkin disease (HD), 5 non-Hodgkin lymphoma (NHL), 4 Wilms tumor, 2 germ cell tumor, 1 Ewing sarcoma, and 1 neuroblastoma), only adenotonsillar hyperplasia was seen in 6 patients, all with NHL, and both thymic and adenotonsillar hyperplasia were seen in 3 patients (1 HD, 2 NHL). In 5 patients, adenoid hyperplasia was proven by biopsy; 1 patient. underwent to adenoidectomy. Their histopathologic investigation showed polyclonal follicular hyperplasia. The authors recommend that patients with thymic and/or adenotonsillar enlargement after successful treatment of their primary malignancy should be evaluated cautiously before an invasive procedure is planned.

Background The therapeutic potential of anthracycline antibiotics is limited by their cardiotoxicity. Electrocardiography, exercise testing, and two-dimensional echocardiography are non-invasive techniques used in the follow-up of children for cardiotoxicity. Plasma B-type natriuretic peptide (BNP) levels are thought to be useful markers in the early detection of AC induced cardiomyopathy. Procedure We evaluated cardiac status of 34 patients with solid tumors treated with anthracycline antibiotics. All of the patients were asymptomatic and had no evidence of residual malignancy. They were evaluated by electrocardiography, exercise testing, echocardiography, and plasma BNP levels measured before and after the exercise testing. Results Electrocardiography revealed only minor abnormalities of little clinical significance. All of the patients completed the exercise testing without complication, and the duration of the exercise for each patient was between normal limits. Cardiac output (CO) and wall stress (WS) were significantly increased in patients, than in controls in echocardiographic evaluation of systolic functions (P 0.05). Conclusion Our findings demonstrated that echocardiographic and biochemical abnormalities could be found even at low cumulative doses of AC antibiotics. The use of serial echocardiographic studies and plasma BNP determinations to identify high-risk patients for cardiotoxicity needs to be verified by additional studies. © 2004 Wiley-Liss, Inc.

Ewing sarcoma (ES) is a malignant neoplasm usually affecting the skeletal system. Extraskeletal ES is a rare tumor. To date, only 1 case of primary mesocolon ES has been previously reported in an adult. Herein, we present the first case of ES in the mesocolon in a child. (C) 2012 Elsevier Inc. All rights reserved.

Infantile haemangiomas, benign vascular tumours seen in 4-10% of infants are characterised by their spontaneous remission following a 3-9 month period of dynamic growth. Propranolol has been reported to be used as a successful treatment of severe symptomatic infantile haemangiomas. Hyperkalaemia has not been recognised as a serious effect of propranolol since recently. Here, we would like to portray a 2-year-old male patient with intestinal haemangiomatosis who presented with severe hyperkalaemia and was successfully managed with hydration, loop diuretics, potassium binding granules, inhaler β-2 agonists and insulin. To date, this is the first case of intestinal haemangiomatosis complicated with severe hyperkalaemia. Our case suggested the idea of close monitorisation of potassium levels as well as haemodynamic status at the initialisation of the propranolol treatment.

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.

Proconvulsive tendency of imipenem/cilastatin is one of its well-known side effects. Most studies report incidence rates ranging from 1.5 to 3% . There is no study on the incidence rate among children with systemic malignancies. Eighty-two patients with various malignancies who received imipenem/cilastatin 143 times for neutropenic fever between March 1994 and October 1999 in Department of Pediatric Oncology, Gazi University, were identified. Three of these patients had convulsions attributed to imipenem/cilastatin; 3.6% of the patients had seizure, or 2% of imipenem/ cilastatin administrations was followed by a seizure attack.

Methotrexate (MTX) is the chemotherapeutic fdr which the serum levels can be detected. If the MTX level is detected in time, high toxicity risk can be decreased. In this study, intermediate doses of MTX (1 g/m(2)) infusions are administered to B-cell non-Hodgkin lymphoma patients between 3 and 13 years old. The toxicity of MTX in accordance with serum levels and the toxicity of other combined drugs are investigated. Blood samples were collected consecutively, and MTX levels were detected by highperformance liquid chromatography. When hematological, gastrointestinal, and renal toxicity scores were compared with the 24-h serum levels of MTX, they showed a significant positive correlation. Hematological toxicity scores increased by Ifosfamide, Etoposide, and Cytarabine combined with MTX without altering the serum levels. Antibiotic combination with MTX has no effect on the toxicity scores. In conclusion, if MTX is combined with other myelosuppressive, hepatotoxic, and nephrotoxic drugs, the measurement of MTX serum levels alone is not a sufficient parameter to show the toxicity. (C) 1999 Elsevier Science Inc. All rights reserved.

Castleman disease is a rare lymphoproliferative disorder of unclear etiology. It usually presents as localized enlarged lymph nodes in children. Surgical excision is curative in localized form. Clinical findings of malabsorption are rarely reported in the literature. Herein, we describe a 14-year-old girl who presented with anemia, failure to thrive, osteoporosis, zinc, and vitamin deficiency. She was diagnosed as localized mesenteric mixed type of Castleman disease. Her clinical findings improved after surgical excision of the mass.

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.

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.

Ewing's sarcoma family of tumors (ESFTs) are indicated by malignant, small, round and blue cell tumors of the bone and soft tissue. Gene rearrangements between EWS gene on chromosome 22q12 and members of the ETS gene family are common in and specific to ESFTs. Another defining characteristic of ESFTs is their membranous expression of the CD99. In contrast, such translocations and immunoreactivity are not found in central primitive neuroectodermal tumors (cPNETs). The aim of this study was to investigate the detection of EWS/FLI1 translocations and CD99 immunoreactivity in order to evaluate their clinicopathological features and their roles in the differential diagnosis of these tumors. In this study, we investigated CD99 immunoreactivity using immunohistochemistry and Ewing's sarcoma / Friend leukaemia virus integration 1 (EWS/FLI1) translocation using the fluorescence in situ hybridization (FISH) method in 23 cases. CD99 expression was detected in 10/11 (90%) ESFT cases and 2/7 cPNET cases. In 18 cases EWS/FLI1 translocation was examined using the FISH method. The EWS/FLI1 translocations were detected in 7/8 (87.5%) ESFTs cases, whereas non of 8 cPNET cases were detected with this translocation. One case could not be classified as either central or peripheral, showed EWS/FLI1 translocation. There was a statistically significant difference in CD99 expression (p = 0.0013) and EWS/FLI1 translocation (p = 0,002) between cPNETs and ESFTs cases. In conclusion, CD99 expression and EWS/FLI1 translocation are specific and sensitive markers in the diagnosis of ESFTs. However, these were often not found in cases of cPNET. Therefore, in the diagnosis of ESFTs, clinical, radiological, histopathological and immunohistochemical parameters should always be evaluated together.

Primary renal primitive neuroectodermal tumor (PNET) is an extraordinarily rare neoplasm in childhood. It generally occurs in young adults and only a few pediatric cases have been reported. PNET in the kidney acts aggressively and the record shows poor therapeutic response. The authors present the case of a 16-year-old girl who was diagnosed with renal PNET and treated with high-dosage chemotherapy and peripheral blood stem cell transplantation (PBSCT).

The authors report a patient with abdominally relapsed Wilms tumor with rhabdomyomatous differentiation leading to renal failure and death 9 years after the initial diagnosis. The patient was treated with intensive chemotherapy because of inoperable tumor but no response was obtained. The prognosis of children with Wilms tumor relapsed in abdomen and in previously irradiated fields is poor and intensive chemotherapy protocols for differentiated tumors after chemotherapy will increase the risk of complications without Obvious benefit. Pediatr Blood Cancer 2009;52:675-677. (C) 2008 Wiley-Liss, Inc.

Vincristine commonly induces peripheral neuropathy but rarely causes voiding dysfunction. In this report, we describe a case of neurogenic bladder and peripheral neuropathy caused by the neurotoxic effect of vincristine and documented by urodynamic testing and electromyography. Neurologic evaluation aided in monitoring and understanding this problem.

Purpose To assess the immune response to influenza vaccine in children with solid tumors receiving chemotherapy or under the influence of chemotherapy. Methods Forty-five children (aged 1–18) with solid tumors on chemotherapy or within 6 months of completion of chemotherapy were included in the study. The children received two doses of intramuscular trivalent split influenza vaccine with 1 month apart in November–December 2003 (children 4 age 0.5 ml). Antibody titer was detected in the pre-vaccination and 4-week post-vaccination sera by hemagglutination inhibition (HI) method. Immune responses were measured as protective, geometric mean titers (GMT), and fourfold rises in HI titers. Results We revealed that the post-vaccination GMT for each of the three antigens in patients with solid tumors has increased significantly (P

It is well known that in haemodialysis patients suffering from oligoanuria, extracellular hypervolaemia develops and this hypervolaemia is the main reason for hypertension occurring in some of the patients. The absence of vasorelaxation during hypervolaemia may be secondary to an increased activity of vasoconstrictor systems and/or a decreased formation of vasodilator agents like prostaglandin E-2(PGE(2)) and prostaglandin I-2(PGI(2)). In the present study, arterial PGE, and leukotriene C-4(LTC4)-like activities and the effect of fluid removal on these arachidonic acid metabolites during haemodialysis were measured in normotensive and hypertensive patients. Plasma PGE(2) and LTC4-like activities were significantly different between hypertensive and normotensive patients. PGE(2)/LTC4 ratio did not change in normotensive patients while it was increased in hypertensive patients after haemodialysis. These results indicate that haemodialysis alters the synthesis of arachidonic acid metabolites especially in hypertensive patients.

The aim of this study was to evaluate children with lymphadenopathy and clinical approach to the suspicion of malignancy. The authors evaluated 457 patients with peripheral lymphadenopathy, less than 19 years of age, and referred to the Pediatric Oncology Department of Gazi University Medical School during the periods March 1996-April 2004. A total of 346 patients had benign disorders and 111 had malignant pathologies. Excisional biopsies were performed to 134 patients. A specific etiology could be found 39% in the benign group. Of the 457 patients, 218 were presented as acute, the rest as chronic lymphadenopathy. In the acute lymphadenopathy group, 98.2% of the patients had benign etiologies. The malignant disorders were mostly represented as chronic lymphadenopathy. Concerning the extension, 193 patients had localized lymphadenopathy and 264 had generalized lymphadenopaties. Cervical region was the most frequent site in both localized and generalized lymphadenopathy groups. Malignancies occurred as generalized lymphadenopathy. Supraclavicular area were involved only in the malignant group. Axillary involvement was predominant in BCG vaccine associated lymphadenitis and mycobacterium tuberculosis. All the lymph nodes less than 1 cm were due to benign causes. The malignant lesions were usually more than 3 cm in diameters. The following findings should alert the pediatrician for the probability of a malignant disorder: lymphadenopathy of more than 3 cm in size, of more than 4 weeks in duration, with supraclavicular involvement, and with abnormal laboratory and radiological findings.

We report a 2-year-old boy who had a family history of neurofibromatosis, multiple cafe-au-lait spots oil the trunk, tibial pseudarthrosis, and was diagnosed with a malignant triton tumor of the pelvis. To our knowledge our case is one of the youngest patients reported with a malignant triton tumor and the second pediatric case with a pelvic malignant triton tumor.

Neutropenia is one of the undesirable effects of cancer chemotherapy, sometimes causing life-threatening events. The present study was conducted to identify the risk factors of neutropenia caused by chemotherapy. Between January 2001 and March 2004, a total of 77 children with 165 neutropenic attacks were enrolled in this study. Complete blood count was performed before chemotherapy and on days 1, 3, 5, 7, and 9 consecutively. The complete blood count results of the febrile neutropenia (FN) group were compared to those of the non-FN group. The incidence of FN was significantly higher in patients with ANC counts < or =0.5 x 10(9)/L on days 3, 5, and 7, lymphocyte counts < or =0.5 x 10(9)/L on days 7 and 9, < or =0.7 x 10(9)/L on days 5, 7, and 9, and with monocyte counts < or =0.15 x 10(9)/L on days 7 and 9, respectively. Further statistical analysis showed that lymphocyte count < or =0.7 x 10(9)/L on day 5, and monocyte count < or =0.15 x 10(9)/L on day 7 were independent risk factors for FN. The authors conclude that lymphocyte counts on the 5th day can be more a predictive factor than monocyte count.

In this report we describe an unusual case of neuroblastoma that presented with an initial manifestation of protein losing enteropathy of secondary to intestinal lymphangiectasia.

Pinarli, Faruk Guclu/0000-0002-3241-2478 WOS: 000239922600015 PubMed: 16923558 Growth impairment is one of the most important late sequelae in childhood malignancies. In the last few years, the contribution of cytotoxic agents to growth retardation has been a subject of investigation. The aim of this study was to evaluate the growth impairment in children treated for non-Hodgkin's lymphoma (NHL). The study group comprised 41 children ( eight girls, 33 boys) treated for NHL with three different chemotherapy protocols. All patients were in remission at the last visit. The control group consisted of 41 healthy age- and sex-matched children. All patients' standing heights and body weights were measured regularly from the time of diagnosis. Growth parameters were measured both at the time of diagnosis and at the end of treatment ( median treatment time: 6 months). Height and weight velocities were calculated at 6 month intervals after the diagnosis until the last visit. The mean height SDS of the patients was -0.66 +/- 1.42 at the diagnosis and 0.29 +/- 1.21 at the last visit. Height SDS of the patients showed a significant improvement at the end of the 2nd year after the diagnosis ( p = 0.005) and at the last visit ( p = 0.022) ( median follow-up time: 48 months after diagnosis). The height velocity SDS increase at the end of the 2nd year was particularly remarkable in short-term protocols such as BFM-90 B-NHL. The sitting height, the sitting height/height ratio and serum insulin-like growth factor-I (IGF-I) levels were found to be lower in the patients than those of control group at the last visit. One can conclude that chemotherapy might cause a reduction in growth velocity during treatment. The cumulative dosages of anti-neoplastic agents and serum IGF-I levels could have been implied in the pathogenesis of growth retardation.

The authors describe a 6-year-old boy diagnosed with mediastinal Burkitt lymphoma with tumor invasion into bone marrow and both kidneys. After receiving chemotherapy according to NHL BFM-95 protocol for the high-risk disseminated lymphoma, the patient reached complete remission. He relapsed in the mediastinum at 5 months from the diagnosis. He underwent thoracotomy and tumor mass was removed by inferior lobectomy of right lung. Residual tumor progressed rapidly. Autologous stem cell transplantation could not be performed because of unresponsiveness to cytoreductive chemotherapy. Twenty-three days after the last chemotherapy course, he received rituximab at a dose of 375 mg/m(2) by intravenous infusion weekly, for a total of 8 dose. However, multiple intra-abdominal metastatic lesions were detected at the end of the therapy. Palliative radiotherapy was applied to these sites. He died because of disease progression, 11 months after the diagnosis.

Summary Here we describe a case of a neuroblastoma patient with recurrent vancomycin-resistant enterococcal infection successfully treated with ciprofloxacin. This is the first case of recurrent vancomycin-resistant enterococal infection in our hospital and Turkey.

Pleuropulmonary blastoma is a rare and aggressive primary intrathoracic neoplasm of children. T'he prognosis is extremely poor with frequent metastasis to the brain and bone. A 4-year-old boy successfully treated with multimodal therapy despite unfavorable prognostic factors is presented. The authors support the use of aggressive treatment of pleuropulmonary blastoma with surgery, chemotherapy, and radiotherapy.

Retinoblastoma is the primary ocular malignancy affecting children under 6 years of age. The development of second malignant tumors in survivors of hereditary retinoblastoma is a well-known clinical entity and a major cause of morbidity and mortality. Rhabdomyosarcomas as second primary tumors have been only rarely described. The authors report a patient with bilateral retinoblastoma who developed a myogenic sarcoma of the orbit after 5.5 years of diagnosis. The short latency period may be explained by tumor histology with the contribution of radiotherapy and chemotherapy. The prognosis of second tumors is poor despite aggressive treatment.

The aim of this study was to investigate the late effects of treatment for childhood non-Hodgkin's lymphomas (NHL) on oral health and dental development. Thirty-six long-term survivors that had been treated with chemotherapy of childhood NHL were included in this study and 36 volunteers with similar age and sex distribution served as controls. Both groups underwent a complete orodental examination for decayed, missing and filled teeth and surfaces, gingival and periodontal health according to the Loe-Silness Gingival Index and Sillnes-Loe Plaque Index, enamel defects and discolorations, root malformations, eruption status, agenesis, premature apexifications and microdontia. The severity of these disturbances related to age at the time of NHL diagnosis were also evaluated by creating two groups as < 5 yr and > 5 yr. Although none of the parameters altered with age, patients had significantly higher plaque index, more enamel discolorations and root malformations than did the controls. The results show that long-term survivors of NHL patients exhibit some orodental disturbances that may be attributed to the chemotherapy regimens.

Primary renal non-Hodgkin's lymphoma is very rare in childhood. A six-year-old boy presented with bilateral non-obstructive multinodular nephromegaly and renal failure. Percutaneous needle biopsy showed large-cell lymphoma. The patient was started on chemotherapy. A right nephrectomy was done when systemic hypertension developed in the presence of a non-functional right kidney. Histopathologic examination revealed focal lymphomatous infiltration and xanthogranulomatous pyelonephritis which is an atypical form of chronic renal infection. The case is discussed in relation to previons reports.

(2001). PSEUDOHYPERKALEMIA DUE TO REACTIVE THROMBOCYTOSIS IN AN INFANT WITH YOLK SAC TUMOR. Pediatric Hematology and Oncology: Vol. 18, No. 4, pp. 303-304.

We present a 4-year-old girl with neurofibromatosis-1 who developed moyamoya syndrome characterized by bilateral stenosis or occlusion of the distal internal carotid arteries and their branches, leading to the development of an abnormal vascular network. In light of a literature review, the postradiation vasculopathy of the moyamoya type and its relationship with neurofibromatosis-1 are discussed. ( J Child Neurol 2000;15:765-767) .

This prospective study evaluated 382 pediatric patients with peripheral lymphadenopathy (LA) presenting at the Pediatric Oncology and Hematology Departments of Social Security Children's Hospital and Gazi University Medical Faculty Hospital. The ages of the patients ranged between 2 months and 16 years (median 7 years); 72% of the patients were male. Of the 382 patients, 138 had localized LA (a single anatomic area involved), 171 had limited LA (two or three areas involved), and 73 had generalized LA (four or more anatomic areas involved). The specific etiology (either benign or malign) was defined in 79% of patients with generalized LA. However, in patients with localized LA and limited LA, specific etiology could be identified only in 43 and 53% of patients, respectively. Based on this study, BCG-LA and pyogenic infections are more frequently manifested by localized LA; LA of unknown origin, Hodgkin's disease, tuberculosis, nasopharyngeal carcinoma, and toxoplasmosis are frequently manifested by localized or limited LA; and cytomegalovirus infection (CMV), infectious mononucleous, rubella, acute leukemia, non-Hodgkin's lymphoma are frequently manifested by limited or generalized LA. Out of 382 patients, 196 patients had a maximum lymph node diameter of less than 2 cm. A benign etiology was shown in 159/196 of these patients. In 37/196 of these patients LA was due to a malignancy, and these cases almost invariably had some apparent additional diagnostic clinical and laboratory findings. Based on this observation a maximum lymph node size of 2 cm was considered an appropriate limit to distinguish malignant disease from benign causes except when there is other evidence of an underlying malignant disease. However, lymphadenopathies located at supraclavicular region (27 patients) either localized or as part of generalized LA had a specific benign or malignant disease in etiology (malignancy in 20, tuberculosis in 3, CMV in 2, sarcoidosis in 1, and lipoma in 1) even though they were less than 2 cm in diameter.

Malnutrition in children with cancer is reported to be relatively uncommon at the time of diagnosis. However, in most studies nutritional status measurement has relied almost exclusively on weight-related indices. This can be misleading, because in children with malignancy, tumor masses can reach more than 10 % of total body weight. A controlled study was performed in 62 patients using arm anthropometry to provide a more accurate evaluation of the nutritional status of children, with cancer at presentation. Height, weight, midupper arm circumference (MUAC), and triceps skinfold thickness (TSFT) were measured in all patients (40 boys, 22 girls) and controls (18 boys, 13 girls). Weight for height (WFH) of each patient was compared with the national standards. MUAC and TSFT were also interpreted according to the standards developed by A. Roberto Frisancho. The mean ages were 6.5 +/- 3. 7 years (range 0.08-13) and 57 +/- 4.7 gears (range 0.25-15) in patients and control group, respectively. Results showed that although the WFH values for patients were normal, MUAC and TSFT values were significantly less than control values (P < 0.001). Moreover, 27% of patients showed malnutrition (ther had MUAC and TSFT below 5th percentile). Patients with intraabdominal solid tumors had significantly lower MUAC and TSFT values than those with extraabdominal solid tumors (P < 0.05). The data strongly indicate that malnutrition is common at the time of diagnosis in children with cancer, and arm anthropometry should replace the use of weight-related indices to identify malnutrition in children.