Congenital Lung Malformations: A Pictorial Review of Imaging Findings and a Practical Guide for Diagnosis.

The term congenital lung malformation (CLM) is used to describe a wide range of pathological conditions with different imaging and clinical manifestations. These anomalies stem from abnormal embryological lung development, potentially occurring across various stages of prenatal life. Their natural history can be variable, presenting in a wide range of severity levels and encompassing asymptomatic individuals who remain so until adulthood, as well as those who experience respiratory distress in the neonatal period.

Diagnosis and Management of Congenital H-Type Tracheoesophageal Fistula: Results of a National Survey.

Background: Congenital h-type tracheoesophageal fistula (H-TEF) without esophageal atresia (EA) represents about 4% of congenital esophageal anomalies. The diagnosis is challenging, and surgery is considered curative. The aim was to report a national survey on the diagnosis, management, and outcome of patients with congenital H-TEF.

Congenital lung malformations: a nationwide survey on management aspects by the Italian Society of Pediatric Surgery.

Introduction: Over the years, congenital lung malformations (CLM) management remains a controversial topic in pediatric thoracic surgery. The Italian Society of Pediatric Surgery performed a national survey to study the current management variability among centers, trying to define national guidelines and a standardized approach of children with congenital lung malformations.

Methods: Following a National Society approval, an electronic survey including 35 items on post-natal management was designed, focusing on surgical, anesthesiology, radiology and pneumology aspects.

WT1 and Cyclin D1 Immunohistochemistry: A Useful Adjunct for Diagnosis of Pediatric Small Round Blue Cell Tumors on Small Biopsies.

Pediatric small round blue cell tumors (SRBCTs) are a heterogeneous group of neoplasms with overlapping morphological appearance. Accordingly, their diagnosis is one of the most difficult in the field of surgical pathology. The most common tumors include rhabdomyosarcoma, Ewing's sarcoma, neuroblastoma, lymphoblastic lymphoma and Wilms' tumor (the blastemal component).

Desmoplastic Small Round Cell Tumor with "Pure" Spindle Cell Morphology and Novel Fusion Transcript: Expanding the Morphological and Molecular Spectrum of This Rare Entity.

Background: Desmoplastic small round cell tumor (DSRCT) is a rare pediatric soft tissue neoplasm composed of small round tumor cells with prominent stromal desmoplasia, polyphenotypic differentiation and gene fusion. We, herein, present a unique case of DSRCT, exhibiting a pure spindle cell morphology, absence of desmoplastic stroma and showing a novel fusion transcript.

Methods: A 12-year-old boy presented multiple intra-abdominal, confluent and mass-forming nodules that affected the entire abdominal and pelvic cavities.

Guidelines of the Italian Society of Videosurgery (SIVI) in Infancy for the minimally invasive treatment of Hypertrophic Pyloric Stenosis in neonates and infants.

The most appropriate treatment for the infantile Hypertrophic Pyloric Stenosis (HPS) is still debated. The non-surgical conservative treatment with oral or intravenous administration of atropine does not enjoy a widespread appreciation for several factors (..

Guidelines of the Italian Society of Videosurgery in Infancy (SIVI) for the minimally invasive treatment of fetal and neonatal ovarian cysts.

In the last three decades, fetal ovarian cysts were diagnosed more frequently, due to technological improvement and the increasing use of prenatal screening ultrasound. Nonetheless, treatment uncertainties are still present, either prenatally or postnatally. Recently, significant innovations on diagnosis and treatment have been proposed and a more conservative, minimally invasive approach may be offered to the Pediatrician or the Surgeon who face with this condition during prenatal or neonatal age.

Guidelines of the Italian Society of Videosurgery in Infancy for the minimally invasive treatment of the ureteropelvic-junction obstruction.

The hydronephrosis, characterized by the dilation of the renal pelvicalyceal system with possible functional damage to the renal parenchyma, is the most common congenital abnormality of the urinary system detected in utero through the prenatal ultrasound screening. (..

Guidelines of the Italian Society of Videosurgery in Infancy for the minimally invasive treatment of pediatric nephrectomy and partial nephrectomy.

Throughout history, the pediatric laparoscopic nephrectomy was first described at the beginning of the Nineties by Erlich and colleagues in a child and by Koyle and colleagues in an unweaned patient. (..

Guidelines of the Italian Society of Videosurgery in Infancy for the minimally invasive treatment of the esophageal atresia.

Esophageal Atresia (EA) is defined as an interruption in esophageal continuity that results in a proximal tract that ends in a blind pouch in 98% of cases, and a distal tract that in 87% of cases arises via a Fistula from the Trachea (TEF). (..

Current Status of Pediatric Robot-Assisted Surgery in Italy: Epidemiologic National Survey and Future Directions.

Pediatric robot-assisted surgery (RAS) is gaining increasing acceptance. We aimed to assess the diffusion of pediatric RAS in Italy, the training period, indications, preliminary outcomes, and limitations. An online questionnaire-based survey was performed.

Microenvironment in neuroblastoma: isolation and characterization of tumor-derived mesenchymal stromal cells.

Background: It has been proposed that mesenchymal stromal cells (MSCs) promote tumor progression by interacting with tumor cells and other stroma cells in the complex network of the tumor microenvironment. We characterized MSCs isolated and expanded from tumor tissues of pediatric patients diagnosed with neuroblastomas (NB-MSCs) to define interactions with the tumor microenvironment.

Methods: Specimens were obtained from 7 pediatric patients diagnosed with neuroblastoma (NB).

Guidelines of the Italian Society of Videosurgery in Infancy for the minimally invasive treatment of pediatric nephrectomy and partial nephrectomy.

Not available.

Guidelines of the Italian Society of Videosurgery in Infancy for the minimally invasive treatment of the ureteropelvic-junction obstruction.

Not available.

Guidelines of the Italian Society of Videosurgery in Infancy for the minimally invasive treatment of the esophageal atresia.

Not available.

Severe disease in Cystic Fibrosis and fecal calprotectin levels.

Fecal calprotectin (FC) is used to asses the presence of intestinal inflammation also in patients with Cystic Fibrosis (CF) and recent studies showed a correlation between bowel and lung disease in these patients. The aim of this study was to analyze the levels of FC in CF and correlate them with different phenotypes of disease. We enrolled a cohort of 54 CF patients and 50 healthy controls.

Bilateral adrenal neuroblastoma in the infant: Is it an image-defined risk factor?

The treatment of neuroblastoma is based on the International Neuroblastoma Risk Group stratification considering life-threatening symptoms, image-defined risk factors (IDRFs), presence and site of metastases, biology, and histopathology. The authors present an infant with bilateral nonmetastatic adrenal neuroblastoma with favorable biology. Both tumors were resectable and without IDRFs, but bilateral resection was considered mutilating, so it was decided to operate one side only.

A difficult diagnosis of Hodgkin lymphoma due to immune thrombocytopenia.

We report a rare clinical presentation of childhood Hodgkin lymphoma with immune thrombocytopenia. Diagnostic biopsy of the abdominal mass was performed after administration of intravenous immunoglobulins, steroids, and platelet transfusion. Concomitant thrombocytopenia complicated the whole diagnosis work up and the initial management of neoplasia.

European Society of Pediatric Endoscopic Surgeons (ESPES) guidelines for training program in pediatric minimally invasive surgery.

Purpose: The aim of this paper was to propose structured guidelines for a European pediatric MIS training program created by ESPES.

Methods: A questionnaire, focused on how the pediatric training program in MIS has to be structured, was proposed to all participants at the ESPES Congress in Marseille in 2013.

Results: We received 178 questionnaires but only 139 questionnaires were fully completed and analyzed.

Evaluation of neoadjuvant chemotherapy effects on liver parenchyma in resected pediatric malignancies.

Neoadjuvant chemotherapy for colorectal liver metastases in adults is responsible for chemotherapy-associated liver injury (CALI), characterized by steatosis, steatohepatitis, and sinusoidal obstruction syndrome. These alterations cause delayed operation to reduce the risk of hemorrhage, portal hypertension, and hepatic failure. Children with hepatic malignancies usually receive neoadjuvant chemotherapy prior to surgery.

Unclassified pediatric renal stromal tumor overlapping with metanephric stromal tumor and solitary fibrous tumor with diffuse S-100 protein expression.

Metanephric stromal tumor (MST) is a rare pediatric neoplasm unique to the kidneys that is currently included in the spectrum of metanephric tumors, along with metanephric adenoma and adenofibroma. We herein report an unusual case of pediatric renal stromal tumor overlapping with MST and solitary fibrous tumor (SFT). Histologically, the tumor was composed of bland-looking spindle to stellate cells embedded in a fibro-sclerotic stroma that focally surrounded native entrapped renal tubules or blood vessels with abortive rings or collarettes.

One-trocar-assisted pyeloplasty.

Purpose: One-trocar-assisted pyeloplasty (OTAP) is a recent, not yet well-known surgical technique for the treatment of ureteropelvic junction obstruction, described for the first time by El Gohary in 2004. The first step of this procedure is to identify the pyeloureteral junction (PUJ) through a laparoscopic approach using one trocar, and the pelvic-ureteric anastomosis is carried out in "open" surgery. We present our experience with OTAP, an innovative technique in pediatric surgery.

Altered cytoskeletal structure of smooth muscle cells in ureteropelvic junction obstruction.

Purpose: Ureteropelvic junction obstruction is one of the most common causes of hydronephrosis in children. A malfunction of smooth muscle cells is believed to be the underlying mechanism causing obstruction. We investigated the expression of some integrins, talin and β-dystroglycan, considered the main compound of smooth muscle cell cytoskeleton, and active caspase 3 at the level of the ureteropelvic junction obstruction.

Activation of adenosine A2A receptors by polydeoxyribonucleotide increases vascular endothelial growth factor and protects against testicular damage induced by experimental varicocele in rats.

In rat experimental varicocele, polydeoxyribonucleotide (PDRN) induces vascular endothelial growth factor (VEGF) production, thereby enhancing testicular function. This may point to a new therapeutic approach in human varicocele.