Neuroblastoma among children in Southern and Eastern European cancer registries: Variations in incidence and temporal trends compared to US.

Neuroblastoma comprises the most common neoplasm during infancy (first year of life). Our study describes incidence of neuroblastoma in Southern-Eastern Europe (SEE), including - for the first time - the Nationwide Registry for Childhood Hematological Malignancies and Solid Tumors (NARECHEM-ST)/Greece, compared to the US population, while controlling for human development index (HDI). Age-adjusted incidence rates (AIR) were calculated for 1,859 childhood (0-14 years) neuroblastoma cases, retrieved from 13 collaborating SEE registries (1990-2016), and were compared to those of SEER/US (N = 3,166; 1990-2012); temporal trends were assessed using Poisson regression and Joinpoint analyses.

Cardiac Involvement in Duchenne Muscular Dystrophy and Related Dystrophinopathies.

Dystrophinopathies include Duchenne muscular dystrophy (DMD), Becker muscular dystrophy (BMD), X-linked dilated cardiomyopathy (XLCM), and facioscapulohumeral muscular dystrophy (FSHD). DMD/BMD are X-linked recessive disorders, related to the synthesis of dystrophin. Most of DMD after the third decade of their age develop cardiomyopathy that remains silent, due to relative physical inactivity.

Hemiscrotal Agenesis: A Rare Congenital Anomaly.

Hemiscrotal Agenesis (HSA) is the rarest developmental malformation of the scrotum. It is characterized by the absence of either half of the scrotal rugae with an intact midline raphe. We report the case of a 16-month-old boy with HSA, with an island of scrotal tissue in the pubic tubercle region and ipsilateral cryptorchidism.

Malignant pleural effusion from papillary thyroid carcinoma diagnosed by pleural effusion cytology: A case report.

Papillary thyroid carcinoma (PTC) is by far the most common thyroid malignancy (over 85%) of all the thyroid cancers. It has excellent prognosis and 10-year survival rate in most of the cases (95%). Most of the tumors are indolent and do not recur or metastasize after removal.

Safety of Systemic Agents for the Treatment of Pediatric Psoriasis.

Importance: Use of systemic therapies for moderate to severe psoriasis in children is increasing, but comparative data on their use and toxicities are limited.

Objective: To assess patterns of use and relative risks of systemic agents for moderate to severe psoriasis in children.

Design, Setting, And Participants: A retrospective review was conducted at 20 centers in North America and Europe, and included all consecutive children with moderate to severe psoriasis who used systemic medications or phototherapy for at least 3 months from December 1, 1990, to September 16, 2014.

Treatment of Hepatic Hydatid Cyst in a 7-Year-Old Boy Using a New Type of Radiofrequency Ablation Electrode.

BACKGROUND Radiofrequency ablation has been established as a treatment method for malignancies and some particular cystic lesions, especially in adults. Experience with radiofrequency ablation in the treatment of hydatid cysts, especially in children, is limited. CASE REPORT Although echinococcosis is rare, especially in children, we describe a 7-year-old boy with lung and liver cystic echinococcosis.

Upper Limb Function, Kinematic Analysis, and Dystonia Assessment in Children With Spastic Diplegic Cerebral Palsy and Periventricular Leukomalacia.

Assessment of upper limb function, kinematic analysis, and dystonia in patients with spastic diplegia cerebral palsy and periventricular leukomalacia. Seven children with spastic diplegia cerebral palsy and 8 controls underwent upper limb kinematics. Movement duration, average and maximum linear velocity, index of curvature, index of dystonia, and target accuracy and stability were analyzed.

Thermal Ablation in the Management of Adrenal Metastasis Originating from Non-small Cell Lung Cancer: A 5-year Single-center Experience.

Background: Treatment of adrenal metastasis from lung carcinoma may prolong survival in the selected patients. However, not all patients can undergo surgery; thus, minimally invasive ablation procedures such as radiofrequency ablation (RFA) and microwave ablation (MWA) have gained acceptance as alternative treatment methods. This study summarized a 5-year single-center experience regarding the evaluation of safety and efficacy of computed tomography (CT)-guided thermal ablation in the management of adrenal metastasis originating from non-small cell lung cancer (NSCLC).

Rare case of pulmonary histoplasmosis complicated with bronchocentric granulomatosis in a non endemic area.

Background: Pulmonary histoplasmosis is a fungal infection caused by histoplasma capsulatum, rarely diagnosed in non endemic areas and/or immunocompromised patients. Complication of pulmonary histoplasmosis with bronchocentric granulomatosis is extremely rare.

Case Report: A 48-year-old man with prolonged fever and nausea was admitted to our hospital.

Solitary Plasmacytoma of the Mesentery: A Systematic Clinician's Diagnosis.

Introduction: Plasmacytoma is an uncommon plasma cell neoplasm and its localized form is solitary plasmacytoma of the bone and solitary extramedullary plasmacytoma. Solitary plasmacytoma of the mesentery is extremely rare, reported only in a handful of cases.

Case Presentation: A 47-year-old man with nonspecific abdominal complains was found to have an ill-defined mass on his mesenteric root.

ADCY5-related movement disorders: Frequency, disease course and phenotypic variability in a cohort of paediatric patients.

Introduction: ADCY5 mutations have been recently identified as an important cause of early-onset hyperkinetic movement disorders. The phenotypic spectrum associated with mutations in this gene is expanding. However, the ADCY5 mutational frequency in cohorts of paediatric patients with hyperkinetic movement disorders has not been evaluated.

Busulfan and melphalan versus carboplatin, etoposide, and melphalan as high-dose chemotherapy for high-risk neuroblastoma (HR-NBL1/SIOPEN): an international, randomised, multi-arm, open-label, phase 3 trial.

Background: High-dose chemotherapy with haemopoietic stem-cell rescue improves event-free survival in patients with high-risk neuroblastoma; however, which regimen has the greatest patient benefit has not been established. We aimed to assess event-free survival after high-dose chemotherapy with busulfan and melphalan compared with carboplatin, etoposide, and melphalan.

Methods: We did an international, randomised, multi-arm, open-label, phase 3 cooperative group clinical trial of patients with high-risk neuroblastoma at 128 institutions in 18 countries that included an open-label randomised arm in which high-dose chemotherapy regimens were compared.

Differential diagnosis of Spitzoid melanocytic neoplasms.

Atypical Spitzoid neoplasms represent a controversial and incompletely defined diagnostic category for lesions with intermediate architecture and cytomorphology between Spitz nevus and melanoma. The vast majority of these neoplasms have a good overall prognosis. Only a small proportion of patients will end up developing distant metastases and death.

A Non-Invasive Droplet Digital PCR (ddPCR) Assay to Detect Paternal CFTR Mutations in the Cell-Free Fetal DNA (cffDNA) of Three Pregnancies at Risk of Cystic Fibrosis via Compound Heterozygosity.

Introduction: Non-invasive prenatal diagnosis (NIPD) makes use of cell-free fetal DNA (cffDNA) in the mother's bloodstream as an alternative to invasive sampling methods such as amniocentesis or CVS, which carry a 0.5-1% risk of fetal loss. We describe a droplet digital PCR (ddPCR) assay designed to inform the testing options for couples whose offspring are at risk of suffering from cystic fibrosis via compound heterozygosity.

Mudd's disease (MAT I/III deficiency): a survey of data for MAT1A homozygotes and compound heterozygotes.

Background: This paper summarizes the results of a group effort to bring together the worldwide available data on patients who are either homozygotes or compound heterozygotes for mutations in MAT1A. MAT1A encodes the subunit that forms two methionine adenosyltransferase isoenzymes, tetrameric MAT I and dimeric MAT III, that catalyze the conversion of methionine and ATP to S-adenosylmethionine (AdoMet). Subnormal MAT I/III activity leads to hypermethioninemia.

Sonographic appearances of the epididymis in boys with acute testicular torsion but preserved testicular blood flow on color Doppler.

Background: Little attention has been given to the sonographic appearances of the epididymis in testicular torsion.

Objective: To describe the position and morphology of the epididymis in childhood acute testicular torsion when testicular flow is present on color Doppler sonography.

Materials And Methods: We studied the sonographic findings in boys with clinically and surgically proven acute testicular torsion who were examined sonographically from May 2013 to May 2014 and who had preserved intratesticular flow on color Doppler sonography.

Immunohistological analysis of the jun family and the signal transducers and activators of transcription in thymus.

The Jun family and the signal transducers and activators of transcription (STAT) are involved in proliferation and apoptosis. Moreover, c-Jun and STAT3 cooperate to regulate apoptosis. Therefore, we used double immunostaining to investigate the immunotopographical distribution of phospho-c-Jun (p-c-Jun), JunB, JunD, p-STAT3, p-STAT5, and p-STAT6 in human thymus.

Hypothalamic-pituitary-adrenal axis in HIV infection and disease.

HIV infection induces hypothalamic-pituitary-adrenal (HPA) axis derangements. Partial glucocorticoid resistance has been observed in a subset of AIDS patients, possibly owing to HIV-induced altered cytokine secretion and action. Because glucocorticoids have immunomodulatory effects, the severity of the HPA axis disorder could play a central role in disease progression.

CT-guided radiofrequency tumor ablation in children.

Background: Image-guided radiofrequency ablation is a well-accepted technique of interventional oncology in adults.

Objective: To evaluate the efficacy and safety of CT-guided radiofrequency ablation as a minimally invasive treatment for metastatic neoplasms in children.

Materials And Methods: A total of 15 radiofrequency ablation sessions were performed in 12 children and young adults (median age 9.

Neuroblastoma mRNAs predict outcome in children with stage 4 neuroblastoma: a European HR-NBL1/SIOPEN study.

Purpose: To evaluate the hypothesis that detection of neuroblastoma mRNAs by reverse transcriptase quantitative polymerase chain reaction (RTqPCR) in peripheral blood (PB) and bone marrow aspirates (BM) from children with stage 4 neuroblastoma are clinically useful biomarkers of risk.

Methods: RTqPCR for paired-like homeobox 2b (PHOX2B), tyrosine hydroxylase (TH), and doublecortin (DCX) mRNA in PB and BM of children enrolled onto the High-Risk Neuroblastoma Trial-1 of the European Society of Pediatric Oncology Neuroblastoma Group (HR-NBL1/SIOPEN) was performed at diagnosis and after induction therapy.

Results: High levels of TH, PHOX2B, or DCX mRNA in PB or BM at diagnosis strongly predicted for worse event-free survival (EFS) and overall survival (OS) in a cohort of 290 children.

Are females predisposed to complications in head and neck cancer free flap reconstruction?

Purpose: To investigate gender and other possible factors affecting the frequency and severity of postoperative complications in head and neck cancer free flap reconstruction.

Materials And Methods: A prospective longitudinal cohort study was conducted at the Greek Anticancer Institute, St. Savvas Hospital in head and neck cancer free flap reconstruction concerning postoperative complications.

Omitting or reducing radiotherapy in childhood or adolescence Hodgkin Lymphoma.

Despite high cure rates, treatment of childhood Hodgkin Lymphoma (HL) is associated with late effects caused mainly by radiotherapy (RT). In the GPOH-HD95 trial of the German Society of Pediatric Oncology and Hematology that was recently published in the Journal of Clinical Oncology, RT was spared in patients achieving a stringently defined complete remission (CR) with chemotherapy and reduced in patients with a good partial remission (PR). Overall, RT-treated patients had superior PFS, but overall survival (OS) was almost identical within each risk-stratified treatment group irrespectively of the use of RT.