Clinical factors, management, and outcomes of children under 3 years old with central nervous system tumors: single-center experience.

PURPOSE: Children under 3 years old represent a notable portion, about 25 to 30%, of all central nervous system tumor (CNS) cases. Their clinical course, prognosis, and treatment significantly differ from older children. This single-center retrospective study aims to comprehensively analyze survival factors in children under three diagnosed with CNS tumors. METHODS: Between April 2012 and December 2023, cases under 3 years of age with CNS tumors diagnosed at our center were retrospectively evaluated. RESULTS: Among 279 CNS tumor cases, 42 (15%) were evaluated. The 5-year overall and event-free survival rates were 67.4% (95% CI 47.5-81.1) and 39.8% (95% CI 24.2-55.0), respectively. Gender, symptom onset to diagnosis time, pathological neurological findings at diagnosis, and tumor location did not significantly impact survival (p > 0.05). However, cases with neurological symptoms showed significantly higher event-free survival rates (p < 0.05). Patients with embryonal tumors, metastases, inability for total surgical excision, relapsed/progressive diseases, and who under 1 year old had significantly lower survival rates (p < 0.05). Radiotherapy timing did not affect survival (p > 0.05). Event-free survival rates remained unchanged after the third year. CONCLUSION: The current treatments have been observed to have a positive impact on survival rates. Nonetheless, there is a need for novel treatments for patients with embryonal tumors, metastases, aged under 1 year, and those where total surgical excision is not feasible or in cases with progressive/relapse disease. This study underscores the importance of the first 3 years regarding relapse, progression, or mortality risk.

Atypical Teratoid Rhabdoid Tumor in a Newborn: Can IVF Be a Risk Factor?

BackgroundIn recent years, with the widespread use of assisted reproductive technologies, questions have arisen regarding the possible relationship between these infertile parents with assisted conception procedures and childhood cancers. Case report: We present a 23-day-old newborn conceived by in vitro fertilization (IVF) with a 53 × 46 × 38 mm intracranial mass detected by magnetic resonance imaging on the 15th postnatal day. The mass, removed on 23rd postnatal day, was an Atypical Teratoid Rhabdoid Tumor (ATRT), WHO grade 4. Conclusions: As far as we know, this is the only neonatally detected ATRT. Further studies are needed to investigate whether there is a causal relationship between IVF and childhood cancers.

Malignancy and lymphoid proliferation in primary immune deficiencies; hard to define, hard to treat.

BACKGROUND: Regarding the difficulties in recognition and management of the malignancies in primary immune deficiencies (PIDs), we aimed to present the types, risk factors, treatment options, and prognosis of the cancers in this specific group. METHODS: Seventeen patients with PID who developed malignancies or malignant-like diseases were evaluated for demographics, clinical features, treatment, toxicity, and prognosis. RESULTS: The median age of malignancy was 12.2 years (range, 2.2-26). Lymphoma was the most frequent malignancy (n = 7), followed by adenocarcinoma (n = 3), squamous cell carcinoma (n = 2), cholangiocarcinoma (n = 1), Wilms tumor (n = 1), and acute myeloid leukemia (n = 1). Nonneoplastic lymphoproliferation mimicking lymphoma was observed in five patients. The total overall survival (OS) was 62.5% ± 12.1%. The OS for lymphoma was 62.2% ± 17.1% and found to be inferior to non-PID patients with lymphoma (P = 0.001). CONCLUSION: In patients with PIDs, malignancy may occur and negatively affect the OS. The diagnosis can be challenging in the presence of nonneoplastic lymphoproliferative disease or bone marrow abnormalities. Awareness of susceptibility to malignant transformation and early diagnosis with multidisciplinary approach can save the patients' lives.

Post-traumatic stress disorder in mothers of children who have undergone cancer surgery.

BACKGROUND: The aim of this study was to investigate the rate of post-traumatic stress disorder (PTSD) and associated risk factors among mothers of children who underwent cancer surgery. METHOD: This cross-sectional, multi-center study included a total of 60 mothers whose children underwent major thoraco-abdominal surgery and were under follow up in the outpatient setting between February 2016 and May 2016. Clinical Data Form, Hospital Anxiety and Depression Scale (HADS), and Clinician-Administered PTSD scale were used. RESULTS: Of all participants, 13 (21.7%) were diagnosed with PTSD. These mothers had shorter duration of marriage, longer duration of hospital stay after surgery, and higher HADS scores, compared with the others without PTSD. Thoughts of guilt such as "I am being punished or tested" were more frequent in mothers with PTSD. Insomnia, irritability, concentration problems, and psychological reactivity were the most common symptoms. CONCLUSION: Post-traumatic stress disorder is a severe disorder that may worsen the daily functioning of mothers and may also have an unfavorable effect on child. It is therefore of utmost importance for clinicians to recognize PTSD and the associated risk factors in order to guide these parents.

Low- versus high-flow oxygen delivery systems in children with lower respiratory infection.

BACKGROUND: Delivery of supplemental oxygen is the initial vital management of hypoxemic acute lower respiratory infection (HALRI). Oxygen delivery systems include low-flow and high-flow devices. In high-flow devices such as the Venturi mask, a constant mixture of oxygen is delivered. As a result, increased rate of breathing does not affect the concentration of oxygen delivered. In this study, we compared the efficacy of oxygen masks and Venturi masks in the management of hypoxemia in pediatric patients. METHODS: A total of 65 children, aged 3-36 months, diagnosed with HALRI, were enrolled. Patients were allocated into groups, via simple alternate randomization, to receive oxygen through an oxygen mask or through a Venturi mask. Respiratory rate, heart rate, retraction, blood gas parameters, oxygen saturation, length of hospitalization, and oxygenation were recorded before and after oxygen treatment. RESULTS: After 24 h of treatment, respiratory rate was significantly lower among patients in the Venturi mask group compared with the oxygen mask group. Duration of supplemental oxygen and length of hospitalization were significantly lower in the Venturi mask group compared with the oxygen mask group. CONCLUSION: In both groups, there was marked improvement in all measured parameters following introduction of supplemental oxygen. Oxygen was delivered more efficiently, however, by high-flow systems. The Venturi mask may decrease the total duration of oxygen usage time as well as the length of hospitalization among young children with HALRI through rapid symptom resolution.

The diagnostic value of soluble urokinase plasminogen activator receptor compared with C-reactive protein and procalcitonin in children with febrile neutropenia.

The aim of the present study was to determine the diagnostic value of soluble urokinase plasminogen activator receptor (suPAR) in pediatric patients with febrile neutropenia. A prospective case-control study was performed. Patients included 29 children with febrile neutropenia (FN) and 27 control subjects without any infection or immunosuppressive condition. Blood samples were obtained on the day of admission and on the 4th to 7th days of the hospital stay. The median (minimum-maximum) serum levels of suPAR obtained on the first day of the admission were 2.08 (0.93-9.42) and 2.22 (1.08-5.13) ng/mL for the FN group and the control group, respectively. The median serum levels of suPAR in the FN and control groups were not significantly different (P = .053). The mean serum suPAR level was significantly higher in nonsurvivors than in survivors in the FN group (P < .05). In the FN group, the area under the receiver operating characteristics curve (AUCROC) for suPAR was 0.546, but no optimum cutoff value, sensitivity, specificity, negative predictive value (NPV), or positive predictive value (PPV) was obtained. We conclude that suPAR is not useful as a diagnostic biomarker in children with febrile neutropenia; however, persistent high serum suPAR level may predict mortality in FN in children.

Translation and cultural adaptation of health utilities index with application to pediatric oncology patients during neutropenia and recovery in Turkey.

BACKGROUND: This is a report on the translation and cultural adaptation from English to Turkish languages of a standard HEALTH UTILITIES INDEX® (HUI®) questionnaire for interviewer-administration and proxy assessment of patients' health status, and its use for assessing 50 consecutive pediatric oncology patients during periods of and subsequent recovery from neutropenia. PROCEDURE: Preparation of the Turkish-language questionnaire was a collaborative effort by physician-researchers in Turkey, senior Health Utilities, Inc. staff with extensive experience in the development and translation of HUI questionnaires, and Turkish-born residents of Canada. Standard HUI questionnaires cover both HUI Mark 2 (HUI2) and HUI Mark 3 (HUI3). RESULTS: The mean improvement in overall health-related quality of life (HRQL) scores between neutropenic and non-neutropenic phases was clinically important according to both HUI2 and HUI3 instruments. Single-attribute utility scores showed clinically important size improvements between phases for HUI2 mobility, emotion, and HUI2 self-care. Significant reductions in disability rates between phases were also detected for pain (HUI2 and HUI3 rate decreases of 30%, P < 0.001) and HUI3 ambulation (rate decline of 14%, P = 0.020). CONCLUSIONS: The study results provide evidence that the Turkish-language questionnaire is acceptable, valid and useful for collecting parental assessments of health-status among young cancer patients in Turkey. Neutropenia was associated with important deficits in overall HRQL and disabilities in ambulation/mobility, emotion, self-care, and pain. Treatment plans should include strategies for addressing emotion and pain problems to improve the HRQL of neutropenic patients.

Treatment of Wilms tumor: a report from the Turkish Pediatric Oncology Group (TPOG).

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 <16 years with unilateral WT whose treatment started in first postoperative 3 weeks were included. Treatments were stage I favorable (FH) and unfavorable histology (UH) patients, VCR + Act-D; stage IIA FH, VCR + Act-D; stage IIB FH, VCR + Act-D + radiotherapy (RT); stage III-IV FH, VCR + Act-D + adriamycin (ADR) + RT; stages II-IV UH tumors, VCR + Act-D + ADR + etoposide + RT. RESULTS: 165/254 registered cases were eligible (bilateral, 5.9%) [median age 3.0 years; M/F: 0.99; 50/165 cases < or =2 years]. 9.7% cases had UH tumors. Disease stages were stage I 23.6%; IIA 36.4%; IIB 5.5%; III 22.4%; IV 12.1%. Cases >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.

Langerhans cell histiocytosis in Turkish children.

Langerhans cell histiocytosis (LCH) is a rare disorder with diverse forms of clinical presentation ranging from a benign course to diffuse progressive disease, and descriptions of LCH generally have been limited to single case reports and small case series. Since the condition is uncommon, we aimed in this study to describe our own experience and discuss the findings, treatment and outcome in our patients with LCH in light of the current literature. The eight patients (5 boys, 3 girls) ranged in age from 2 months to 3 years (mean 18 +/- 10.8 months). Multiple organ involvement was noted in 3, isolated bone involvement in 3, orbital involvement in 1, and pituitary gland involvement with rash in 1 patient. Treatment modalities used varied from simple observation to chemotherapy. Outcome results of our study demonstrated resolution of lesions in 5 patients and death in 2 patients. One patient with pituitary gland involvement developed diabetes insipidus and is receiving intranasal desmopressin acetate. Based on our results and a review of the literature, we recommend that any child with suspected solitary LCH undergo a full diagnostic investigation to rule out multiple lesions. A biopsy is recommended for a diagnosis at the time of presentation and should be attempted in any suspicious lesion. Chemotherapy is reserved for multiple systemic lesions and central nervous system (CNS) risk lesions. Follow-up investigations should be individualized but should consist of radiography and magnetic resonance imaging (MRI). Studies suggest that follow-up of at least four years is required.

25-hydroxy Vitamin D Levels in Pediatric Asthma Patients and its Link with Asthma Severity.

Aim Although the relationship between 25-hydroxy (25-OH) vitamin D and asthma is known, it is unknown if 25-OH vitamin D levels are correlated with asthma severity in pediatric patients. The aim of this study was to compare the blood 25-OH vitamin D levels of asthma patients and healthy control groups and to evaluate any correlation between asthma severity and blood 25-OH vitamin D levels in pediatric asthma patients. Methods This is a cross-sectional study which shows the 25-OH vitamin D levels of asthma patients and compared to healthy controls followed by a tertiary pediatric clinic. We investigated the effect of 25-OH vitamin D levels on the severity of asthma. The severity of asthma was determined mainly by the duration of asthma diagnosis, a number of attacks in the previous year, anti-inflammatory medication usage in a previous year, atopy presence in the family, skin prick test positivity, and immunoglobulin E (IgE) levels of asthma patients. Results Compared with control groups, asthma patients had significantly lower 25-OH vitamin D, calcium (Ca) levels, and higher number of patients who had a 25-OH vitamin D deficiency, (p<0.0001, p<0.0001, p<0.0001, respectively). We found a correlation between blood 25-OH vitamin D levels and force expiratory capacity in one second (FEV1) and forced vital capacity (FVC) respectively (p< 0.001, r=0.512), (p< 0.001, r=0.513). There was an association between FEV1, FVC and blood 25-OH vitamin D levels in terms of deficient levels (<20 ng/mL) or insufficient levels (≥20 and <30 ng/mL) (p<0.001, r=0.459), (p< 0.001, r=0.450), respectively. Conclusions The 25-OH vitamin D levels were lower in pediatric asthma patients with worse spirometry results. Effects of Vitamin D supplementation need to be evaluated by well-designed studies.

Intracerebral hematoma as a complication of intrathecal methotrexate administration.

Neurotoxicity of methotrexate is a well-documented issue, but development of an intracerebral hematoma following administration of intrathecal methotrexate is an extremely rare entity. A 6-year-old male with the diagnosis of non-Hodgkin lymphoma was put on a treatment regimen, including intrathecal methotrexate. Six days following the last intrathecal methotrexate administration, the patient developed a deteriorating state of consciousness. There was no history of trauma. Coagulation studies and platelet count were normal. Magnetic resonance imaging of the brain demonstrated a large left frontoparietal hematoma. Intracerebral hematoma may be a very rare, but serious, complication of intrathecal methotrexate administration.

Assessment of quality of life in asthmatic Turkish children.

Our aim was to assess impairment in quality of life (QOL) in asthmatic children and to determine the influencing factors. The study group consisted of 305 outpatients with asthma, aged 7-17 years, who were undergoing regular checkups in our outpatient clinic. QOL was assessed using the Pediatric Asthma QOL Questionnaire (PAQLQ). Asthma severity, presence of allergic rhinitis, and steroid usage were strongly and negatively associated with the overall PAQLQ score (p = 0.038) in multivariate regression models. Sex, IgE levels, positive skin tests and smoking status demonstrated no statistically significant effects on the overall or three individual domain PAQLQ scores (p = 0.307, 0.137, 0.470, 0.353, respectively). Lung function measures were strongly correlated with each other (Spearman correlation of 0.77), but were not associated with QOL (p = 0.441). Fifty-one percent of the patients reported that asthma affected their lives significantly. This study captured the baseline QOL information about the pediatric asthmatic population and factors influencing QOL and will facilitate longitudinal monitoring.

Impact of obesity on ventilatory function.

OBJECTIVE: Although obesity was found to be associated with severe impairment of ventilation, most of the study population has been morbidly obese adults. We aimed to explore the effects of mild obesity on ventilatory function in the pediatric age group. METHODS: In a cross-sectional controlled study, 80 patients (M/F: 35/45), who were evaluated in our outpatient clinic with the complaint of excess body weight, with no history of asthma or other atopic diseases were studied and compared to a control group of 50 normal weight children controlled for age and sex. The mean age of patients was 9.7+/-2.5 years (7 to 15 years). Anthropometric measurements and spirometry were performed in all subjects. Forced vital capacity (FVC) and forced expiratory volume in 1 second (FEV1) were used as measures of ventilatory function. RESULTS: There were no significant differences in FEV1%, FVC% and FEV1%/FVC% by study group (p > 0.05). Only three patients had obstructive abnormalities documented on their pulmonary function tests (two had moderately severe and one had mild obstructive abnormalities). No correlation was observed between pulmonary function parameters and anthropometric measurements. CONCLUSION: These data demonstrate that pulmonary function test parameters of the mildly obese children were similar to those of the normal weight children. Anthropometric measurements had no significant effect on spirometric measurements in children as they did on adults.

Investigation of Vitamin D Receptor Gene Polymorphism in Pediatric Patients with Brain Cancer.

AIM: In recent years, it is believed that Vitamin D may play a protective role in some cancer types. Certain regions of the Vitamin D receptor (VDR) gene may show a genetic difference in structure. The most frequent polymorphisms in this gene are in Taq-1, Fok-1, and Bsm-1 regions. Some adult cancer types are associated with VDR gene polymorphism such as; colorectal carcinoma, breast carcinoma, and prostate carcinoma. Reviewing the medical literature, no such study had been done on children so far. MATERIALS AND METHODS: We investigated the association of the three most common gene polymorphisms (Taq-1, Fok-1, and Bsm-1 regions) in VDR gene in 32 children with brain tumors and forty control healthy volunteers. RESULTS: We could not find any relationship between childhood brain tumors and VDR gene polymorphism in these three regions. CONCLUSION: The present results suggest that the Taq-1, Fok-1, and Bsm-1 polymorphism in the VDR gene and pediatric brain cancers have no association.

Cleidocranial dysplasia in a mother and her daughter within the scope of neurosurgery. Report of two cases.

Cleidocranial dysplasia is an autosomal-dominant disorder characterized by late closure or nonclosure of the anterior fontanelle, late ossification of cranial sutures, defective clavicle, and delayed eruption of permanent teeth. In this article, two cases of cleidocranial dysplasia involving a mother and her daughter are reported, and a case management policy is suggested. The 1-year-old daughter was macrocephalic and brachycephalic, and had midface hypoplasia and hypertelorism. Plain radiographs revealed aplasia of the clavicles. Three-dimensional computerized tomography scanning demonstrated a large anterior fontanelle, a patent posterior fontanelle, and bone defects at the pterion and asterion, together with nonfused metopic and sagittal sutures. The mother was 22 years of age. She had an open anterior fontanelle, aplastic clavicles, and unerupted permanent teeth. Although it is a rare disorder, cleidocranial dysplasia should be recognized by neurosurgeons. A protective helmet can be provided in early childhood, and craniofacial remodeling can be undertaken at a later age, when the final size and shape of the skull become apparent.

Ewing Sarcoma of the External Ear Canal.

Background. Ewing sarcoma (ES) is a high-grade malignant tumor that has skeletal and extraskeletal forms and consists of small round cells. In the head and neck region, reported localization of extraskeletal ES includes the larynx, thyroid gland, submandibular gland, nasal fossa, pharynx, skin, and parotid gland, but not the external ear canal. Methods. We present the unique case of a 2-year-old boy with extraskeletal ES arising from the external ear canal, mimicking auricular hematoma. Results. Surgery was performed and a VAC/IE (vincristine, adriamycin, cyclophosphamide alternating with ifosfamide, and etoposide) regimen was used for adjuvant chemotherapy for 12 months. Conclusion. The clinician should consider extraskeletal ES when diagnosing tumors localized in the head and neck region because it may be manifested by a nonspecific clinical picture mimicking common otorhinolaryngologic disorders.

Methemoglobinemia due to application of prilocaine during circumcision and the effect of ascorbic acid.

Local anesthesia with prilocaine has become a routine part of ambulatory circumcision procedures. Methemoglobinemia is a rare but potentially lethal complication of local anesthetics. We report the case of a 42-day-old boy who presented with cyanosis after receiving local anesthesia with prilocaine. Methemoglobin level revealed severe methemoglobinemia (methemoglobin=44.5%). His cyanosis promptly resolved after intravenous administration of ascorbic acid. Cases of local anesthetic-induced methemoglobinemia in urology are under recognized. Although the association between prilocaine use and methemoglobinemia has generally restricted prilocaine use in infants, it is still widely used in ambulatory procedures, especially during circumcision in the neonatal period. Prilocaine should not be used in infants less than 3 months of age because of the risk of methemoglobinemia and alternative local analgesics should be considered among this age group. We also discuss the use of ascorbic acid during treatment in light of the literature.