Clinical findings and mutational spectrum of neurofibromatosis type 1 patients in a single center of south part of Turkey.

Aim: The aim of this study is to determine the mutation spectrums and clinical characteristics of NF1 patients followed up in our center and to investigate whether there is a genotype-phenotype relationship.

Material And Methods: Sixty-three children and 34 relatives diagnosed with NF1 were included in the study. Age, gender, family history, clinical features, tumors detected in the patient at the time of diagnosis or during follow-up, orbital and cerebral magnetic resonance imaging (MRI) findings were recorded.

Bone densitometry measurements in children with neurofibromatosis Type 1 using quantitative computed tomography.

Introduction: Neurofibromatosis type 1 (NF1) is an autosomal dominant neurocutaneous disease characterised by multisystemic involvement, including bone tissue. Deformities and reduced bone mass are the main bone manifestations in NF1. Quantitative computed tomography (QCT) provides true volumetric bone mineral density (BMD) measurement.

Temporal bone hemangioendothelioma as a rare vascular tumor in childhood: case report and review of the literature.

Background: Hemangiondothelioma is a rare vascular tumor that can occur in the bone. Temporal bone involvement has been reported extremely rare in the literature.

Case: Radiological examination of a one-year-old girl who was admitted due to facial paralysis revealed vascular tumor of the temporal bone and Galen vein aneurysm.

Vandetanib in a Child Affected by Neurofibromatosis Type 1 and Medullary Thyroid Carcinoma with Both and Homozygous Proto-oncogen Germ-line Mutations.

Cases of neurofibromatosis type 1 ()-associated medullary thyroid carcinoma (MTC) or C-cell hyperplasia are rarely associated with other endocrine tumors or cases with a multiple endocrine neoplasia type 2. In these patients, mutations were detected in the gene but no mutations were detected in the gene. Although vandetanib has been shown to improve progression-free survival in adults with advanced MTC, data in pediatric patients are limited.

Bone marrow involvement in pediatric malignancies: a comparison study of Positron emission tomographycomputed tomography and bone marrow biopsy.

Background And Objectives: The comparison of Positron emission tomography- computed tomography (PETCT) and bone marrow biopsy (BMB) modalities in detecting bone marrow disease is an up to date research topic. In this study, we aimed to compare the results of PET-CT and BMB procedures in detecting bone marrow involvement in pediatric malignancies.

Method: At the time of diagnosis, PET-CT imaging and BMB performed patients` data were evaluated, retrospectively.

Effective High-dose Interferon-α Therapy in a 13-Year-Old Girl With Erdheim-Chester Disease.

Erdheim-Chester disease (ECD) is a proliferative disorder of non-Langerhans histiocytes with a higher incidence in the fifth to seventh decades and rarer occurrence in the pediatric population. Although ECD typically involves bone, it can also affect the central nervous system, cardiovascular system, retro-orbital space, retroperitoneal space, and kidneys, lungs, and skin. A 13-year-old Syrian girl who presented with multisystemic involvement was diagnosed with ECD.

Mucoepidermoid Carcinoma in Warthin Tumor of the Parotis in Childhood: A Case Report and Review of the Literature.

Mucoepidermoid carcinoma arising in Warthin tumor of the parotid gland is an extremely rare entity. This is so far described only in the adult age group, and only one patient has been reported in the pediatric age group until today. Herein, we describe our patient and review the literature.

Evaluation of renal tumors in children.

Objective: Renal tumors are not uncommon in children. In this study, we aimed to evaluate the clinical and pathological features of renal tumors in children.

Material And Methods: Between January 2008 and December 2017, the records of children with renal tumors in our institution were retrospectively analyzed.

A Rare Cause of Abdominal Pain in Childhood: Cardiac Angiosarcoma.

Cardiac angiosarcomas are extremely rare in childhood, they are rapidly progressive tumours that often present themselves as diagnostic dilemmas, resulting in delayed diagnosis. Also, extracardiac manifestations, including abdominal pain, are extremely rare in patients with intracardiac tumors. We herein present the case of a 15-year-old girl who presented with abdominal pain.

Prevalence and Related Factors of Euthyroid Sick Syndrome in Children with Untreated Cancer According to Two Different Criteria.

Objective: In this study, we evaluated the frequency of euthyroid sick syndrome (ESS) among patients with childhood cancer and its association with the stage of disease, nutritional parameters and cytokines levels.

Methods: Eighty newly diagnosed children were included in the study. ESS was assessed in two different ways.

[Diagnostic accuracy of the LightCycler® SeptiFast assay in the childhood febrile neutropenia].

Infection is the main problem among the patients receiving cancer therapy. The mortality rate can be reduced by the appropriate treatment in the right time. Although blood culture is the gold standard for the diagnoses of sepsis, many factors influence the results of blood culture in children.

Metachronous Synovial Sarcoma After Treatment of Mixed Germ Cell Tumor in a Child with Complete Gonadal Dysgenesis.

Patients with complete XY gonadal dysgenesis (GD) show a high predisposition to germ cell tumors (GCT). Patients with coexistence of GCT and GD have been reported previously. Here we present a 15-year-old girl with mixed GCT and GD who also developed an intra-abdominal synovial sarcoma one year after the treatment.

Rare Endobronchial Inflammatory Myofibroblastic Tumor in Pediatric Patient Detected on PET/CT Imaging.

Inflammatory myofibroblastic tumor (IMT) can be seen in all age groups, although it is more common in children and adolescents. We report the FDG PET/CT findings in an 8-year-old boy with endobronchial IMT. Endobronchial IMT is more commonly seen in young adults.

Primary pulmonary Langerhans cell histiocytosis associated with smoking in an adolescent boy.

Pulmonary Langerhans cell histiocytosis (PLCH) is a well known entity in adults but is exceedingly rare in children. It is better described in adults than in children. Smoking is a major etiological factor in adulthood.

The diagnostic and prognostic value of angiopoietins compared with C-reactive protein and procalcitonin in children with febrile neutropenia.

Mimaroğlu E, Çıtak EÇ, Kuyucu N, Eskendari G. The diagnostic and prognostic value of angiopoietins compared with C-reactive protein and procalcitonin in children with febrile neutropenia. Turk J Pediatr 2017; 59: 418-425.

Malignant epitheloid angiomyolipoma of the kidney in a child treated with sunitinib, everolimus and axitinib.

The malignant variant of epithelioid angiomyolipoma (EAML) of the kidney is uncommon, extremely aggressive and behaves like a renal cell carcinoma. We present a case of a 12-year-old male with malignant EAML who was treated according to adult treatment protocols. To our knowledge, axitinib has not been used before in children.

Risk factors for cisplatin-induced long-term nephrotoxicity in pediatric cancer survivors.

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.

Primary lymph node gastrinoma: a rare cause of abdominal pain in childhood.

Gastrinoma is a hormone-secreting tumor associated with the Zollinger-Ellison syndrome. It is quite rare among children. The discovery of gastrinomas in unusual locations such as lymph nodes, bones, ovaries, and the liver poses a diagnostic dilemma as to whether the tumor is primary or metastatic.

Bacterial spectrum and antimicrobial susceptibility pattern of bloodstream infections in children with febrile neutropenia: experience of single center in southeast of Turkey.

Empirical antimicrobial therapy is usually started in febrile neutropenic patients without having culture results. The aim of this study was to help determine the policies of empirical antibiotic usage in febrile neutropenic children by detecting the antimicrobial susceptibility profile in this group of patients. In this study 811 blood cultures taken from neutropenic children hospitalized at the Department of Oncology of Gaziantep Children Hospital November 2007 and February 2010 were retrospectively evaluated.

Minor anomalies in childhood lymphomas and solid tumors.

Background: Despite the presence of reports on correlation between major congenital defects and cancer, very few studies have investigated the frequency of minor anomalies in childhood malignancy. The aim of this study was to determine the prevalence of minor anomalies in children with lymphoma and solid tumors.

Procedure: A total of 281 well-defined minor anomalies were determined in 116 patients.

Primary hepatic Burkitt lymphoma in a child and review of literature.

Primary hepatic non-Hodgkin lymphoma is extremely rare in childhood and can be overlooked in differential diagnosis of liver masses. In this study, we describe an unusual case of primary hepatic Burkitt lymphoma in childhood.

The evaluation of carotid intima-media thickness in children with beta-thalassaemia major.

Background: Cardiovascular complications and vascular changes are common in patients with beta-thalassaemia major. The aim of this study is to investigate the common carotid artery intima-media thickness in children.

Methods: The study population was consisted of 33 thalassaemic children (22 boys and 11 girls, with a median age of 8 years) and 30 healthy children for control (12 girls and 18 boys, with a median age of 8 years) who were matched for age and gender.

A retrospective chart review of evaluation of the cervical lymphadenopathies in children.

Objective: Cervical lymphadenopathy is a common finding of physical examination in childhood. The aim of this study was to evaluate the etiology and treatment of cervical lymphadenopathy in children.

Methods: Two hundred eighty two patients' chart reviewed retrospectively who were referred to the Pediatric Oncology Department with cervical lymphadenopathy during the period of November 2007-2009.