Long-Term Outcome and Role of Biology within Risk-Adapted Treatment Strategies: The Austrian Neuroblastoma Trial A-NB94.

We evaluated long-term outcome and genomic profiles in the Austrian Neuroblastoma Trial A-NB94 which applied a risk-adapted strategy of treatment (RAST) using stage, age and amplification (MNA) status for stratification. RAST ranged from surgery only to intensity-adjusted chemotherapy, single or multiple courses of high-dose chemotherapy (HDT) followed by autologous stem cell rescue depending on response to induction chemotherapy, and irradiation to the primary tumor site. Segmental chromosomal alterations (SCAs) were investigated retrospectively using multi- and pan-genomic techniques.

Multiple relapses in high-grade osteosarcoma: when to stop aggressive therapy?

The prognosis after relapse of high-grade osteosarcoma is poor and complete resection of all tumors is essential for survival. A 6-year old was diagnosed with high-grade osteosarcoma and treated according to the COSS-96 protocol. Within 5 years from initial diagnosis, five osteosarcoma relapses occurred and every time it was possible to achieve complete surgical remission.

Spatially resolved optical and ultrastructural properties of colorectal and pancreatic field carcinogenesis observed by inverse spectroscopic optical coherence tomography.

Field carcinogenesis is the initial stage of cancer progression. Understanding field carcinogenesis is valuable for both cancer biology and clinical medicine. Here, we used inverse spectroscopic optical coherence tomography to study colorectal cancer (CRC) and pancreatic cancer (PC) field carcinogenesis.

Hypertrophic obstructive cardiomyopathy in an infant with an adrenocortical tumor.

Nonfamilial cardiomyopathies in childhood have been only sporadically ascribed to endocrine disorders. We report on a 4-month-old male infant presenting with Cushing's syndrome associated with excessive body weight (8.9 kg; >97th percentile) and features of virilization (Tanner stage 2 for pubic hair development).

Interleukin 8 correlates with intestinal involvement in surgically treated infants with necrotizing enterocolitis.

Purpose: The aim of this study was to test the predictive value of interleukin (IL) 8 in the assessment of intestinal involvement in necrotizing enterocolitis (NEC).

Methods: Forty infants with surgically treated NEC were classified into 3 groups based on intestinal involvement during laparotomy: focal (n = 11), multifocal (n = 16), and panintestinal (n = 13). Preoperatively obtained serum levels of IL-8, C-reactive protein, white blood cell count, and platelet count were correlated with intestinal involvement using logistic regression models.

Clinical and immunological correction of DOCK8 deficiency by allogeneic hematopoietic stem cell transplantation following a reduced toxicity conditioning regimen.

Dedicator of cytokinesis 8 protein (DOCK8) deficiency is a combined immunodeficiency disorder characterized by an expanding clinical picture with typical features of recurrent respiratory or gastrointestinal tract infections, atopic eczema, food allergies, chronic viral infections of the skin, and blood eosinophilia often accompanied by elevated serum IgE levels. The only definitive treatment option is allogeneic hematopoietic stem cell transplantation (HSCT). We report a patient with early severe manifestation of DOCK8 deficiency, who underwent unrelated allogeneic HSCT at the age of 3 years following a reduced toxicity conditioning regimen.

Decision making among different treatment options for neurologically impaired boys with undescended testis: a multinational pediatric survey.

Objective: To determine the attitude of referring pediatricians towards the decision of treatment modalities for undescended testis (UDT) in neurologic impaired boys (NIB).

Methods And Materials: An online questionnaire was offered to registered pediatricians in Austria and Germany for online completion.

Results: 221 male (61.

A combined transcriptome and bioinformatics approach to unilateral ureteral obstructive uropathy in the fetal sheep model.

Purpose: Fetal obstructive uropathy is a leading cause of loss of renal function. Characterizing the molecular fingerprint of cellular responses to obstruction in a fetal model of complete unilateral ureteral obstruction may help elucidate the activated mechanisms and suggest new therapeutic interventions.

Material And Methods: Unilateral ureteral obstruction was created in 3 sheep fetuses at day 60 of gestation.

Appendix vermiformis as a left pyelo-ureteral substitute in a 6-month-old girl with solitary kidney.

Extensive ureteral loss in early childhood is a rare but dramatic event. We present the case of a 6-month-old girl with a iatrogenic extensive pyelo-ureteral loss and solitary kidney. She successfully underwent left ureteral substitution using the appendix vermiformis.

Trends in hypospadias surgery: results of a worldwide survey.

Background: Hypospadias is a challenging field of urogenital reconstructive surgery, with different techniques currently being used.

Objective: Evaluate international trends in hypospadias surgery.

Design, Setting, And Participants: Paediatric urologists, paediatric surgeons, urologists, and plastic surgeons worldwide were invited to participate an anonymous online questionnaire (http://www.

Ovarian cysts on prenatal MRI.

Objective: Ovarian cysts are the most frequently encountered intra-abdominal masses in females in utero. They may, at times, require perinatal intervention. Using magnetic resonance imaging (MRI) as an adjunct to ultrasonography (US) in prenatal diagnosis, we sought to demonstrate the ability to visualize ovarian cysts on prenatal MRI.

Online hypospadias support group data analysis.

Background: Although hypospadias is the most common malformation of the penis, this condition is virtually unknown to the general population. This lack of information may lead to psychological distress to both parents and patients. In this study the content of an Internet-based hypospadias support group is analyzed.

Tumor disease and associated congenital abnormalities on prenatal MRI.

Objective: Fetal tumors can have a devastating effect on the fetus, and may occur in association with congenital malformations. In view of the increasing role of fetal magnetic resonance imaging (MRI) as an adjunct to prenatal ultrasonography (US), we sought to demonstrate the visualization of fetal tumors, with regard to congenital abnormalities, on MRI.

Materials And Methods: This retrospective study included 18 fetuses with tumors depicted on fetal MRI after suspicious US findings.

Major complication after intrauterine vesico-amniotic shunting.

Bilateral foetal uropathy is the leading cause of chronic renal failure in childhood. Vesico-amniotic shunting (VAS) is a simple, feasible, and widely used procedure for decompressing the foetal urinary system. We report a case of a boy with bilateral foetal uropathy who underwent VAS at a gestational age of 29 weeks.

Delayed referral despite appropriate knowledge in cryptorchidism as a cause of delayed orchidopexies in Austria.

Aim: Guidelines recommend early orchidopexy in cryptorchidism to decrease the risk of infertility and tumor formation. We aimed to asses if a delayed initial presentation for cryptorchidism was due to unawareness of these guidelines or for any other reasons.

Methods: A short questionnaire exploring timing and referral habits when assessing children with cryptorchidism was sent by email to all registered pediatricians in Austria.

Neuroendocrine tumor of the common hepatic duct, mimicking a choledochal cyst in a 6-year-old child.

We report a rare case of a neuroendocrine tumor of the extrahepatic biliary tree in a child. A 6-year-old girl who presented with jaundice and pruritus was found to have elevated liver enzymes along with a cystic dilatation of the common hepatic duct. After further diagnostic testing, a working diagnosis of a type I choledochal cyst was established.

Clinical, radiological, and pathological findings in four children with gastrointestinal stromal tumors of the stomach.

The incidence of gastrointestinal stromal tumors (GISTs) in children is exceptionally low. However, during the last decade these tumors attracted increasing attention, because they were found to express the cell surface transmembrane receptor kit (CD117) that has tyrosine kinase activity. This tyrosine kinase can be semi-selectively inhibited by signal transduction inhibitors such as imatinib mesylate (Glivec), which is a competitive inhibitor of c-kit, c-abl, platelet-derived growth factor receptor-alpha (PDGFR-alpha) and PDGFR-beta, and abl-related gene (arg).

Surgical risk factors in primary surgery for localized neuroblastoma: the LNESG1 study of the European International Society of Pediatric Oncology Neuroblastoma Group.

Purpose: Although tumor resection is the mainstay of treatment for localized neuroblastoma, there are no established guidelines indicating which patients should be operated on immediately and which should undergo surgery after tumor reduction with chemotherapy. In an effort to develop such guidelines, the LNESG1 study defined surgical risk factors (SRFs) based on the imaging characteristics.

Patients And Methods: A total of 905 patients with suspected localized neuroblastoma were registered by 10 European countries between January 1995 and October 1999; 811 of 905 patients were eligible for this analysis.

Positive troponin T without cardiac involvement in inclusion body myositis.

Cardiac troponin T (cTnT) is considered as a specific marker for acute myocardial infarction. Here, we present a case with elevated cTnT, determined by a third-generation assay, without signs of a myocardial lesion. Routine investigation of a 66-year-old female patient with indolent B-cell lymphoma revealed increased serum levels of creatine kinase (CK), MB fraction of CK (CK-MB), and cTnT, although she did not complain of cardiac symptoms.

Treatment for soft tissue sarcoma in childhood and adolescence. Austrian results within the CWS 96 study.

Objective: The aim of the CWS 96 Study was to achieve an optimal treatment in children and adolescents with soft tissue sarcoma (STS) implementing a further refinement of risk-adapted allocation to chemotherapy, surgery and radiotherapy.

Methods: Treatment stratification was based on tumour histology, TNM status, postsurgical stage, localisation and age. Local tumour control was ensured by surgery and risk-adapted radiotherapy.

Prenatal magnetic resonance imaging as a useful adjunctive to ultrasound-enhanced diagnosis in case of a giant foetal tumour of the neck.

Large cervical masses in the prenatal period are rare and can cause life threatening situations after birth. All available diagnostic techniques should therefore be used to determine the best mode of delivery in the case of such malformation. A large cervical mass was detected by ultrasound in a 41-year-old women, gravida 4, para 3, at 29 + 5 weeks of gestation.

Longitudinal observation of antenatally detected congenital lung malformations (CLM): natural history, clinical outcome and long-term follow-up.

Objective: The objective of the study is to present longitudinal observations in antenatally detected congenital lung malformations (CLM), particularly pulmonary sequestration (PS) and cystic adenomatoid malformation (CAM).

Methods: Fetuses found to have a CLM on prenatal ultrasound (US) were included in this study and followed up until delivery. In all newborns radiographs and computerized tomography (CT) studies of the thorax were performed.

Fetal ovarian cysts: development and neonatal outcome.

Introduction: The aim of the study was to evaluate the outcome of fetal ovarian cysts in relation to their size and ultrasonic appearance.

Methods: We retrospectively analysed pre- and postnatal charts of 61 infants with a prenatal diagnosis of ovarian cysts between 1991 and 2000.

Results: In a total of 61 fetuses 65 ovarian cysts were detected by transabdominal ultrasound: 35 (57 %) cysts on the left side, 22 (36 %) on the right side and 4 fetuses (7 %) had bilateral cysts.