Retroperitoneal teratomas are rare neoplasms in neonates, presenting with nonspecific symptoms and variable clinical features, making diagnosis challenging. Radiological investigations, particularly fetal ultrasound and contrast-enhanced computed tomography, play a critical role in their detection. Differential diagnoses include neuroblastoma, adrenal hemorrhage, and congenital cystic lesions, which share overlapping clinical and imaging features.
View Article and Find Full Text PDFBackground: Social determinants of health (SDOH) have been identified as factors that influence patient health outcomes. These are well described in adult and pediatric general populations, however, there is a paucity of data for surgical patients. This study compares the prevalence of health-related social needs (HRSN) among pediatric surgery and general pediatric patients.
View Article and Find Full Text PDFPurpose: We aimed to examine how various types of cancer, classified histologically, affect semen quality. Methods: The study group included 313 patients who were diagnosed with cancer and reached for a sperm cryopreservation before a gonadotoxic treatment (PG-Tx group). Their semen parameters were compared to those of two control groups: (a) individuals who attended a fertility investigation and were found to be above the limit of the lower reference value of the WHO 2010 manual (ARL group), and (b) fertile men, whose semen parameters were obtained from the dataset of the WHO 2020 manual.
View Article and Find Full Text PDFVentral hernia repair (VHR) is one of the most common surgical procedures performed in the United States. Surgical site infections (SSI) carry significant morbidity for the patient and pose a very challenging problem for the surgeon, associated with up to 6.6% of cases.
View Article and Find Full Text PDFTo repurpose therapeutics for fibrolamellar carcinoma (FLC), we developed and validated patient-derived xenografts (PDX) from surgical resections. Most agents used clinically and inhibitors of oncogenes overexpressed in FLC showed little efficacy on PDX. A high-throughput functional drug screen found primary and metastatic FLC were vulnerable to clinically available inhibitors of TOPO1 and HDAC and to napabucasin.
View Article and Find Full Text PDFFibrolamellar hepatocellular carcinoma (FLC) is a rare primary liver cancer found in adolescents and young adults without underlying liver disease. A deletion of ~400 kD has been found in one copy of chromosome 19 in the tumor tissue of all patients tested. This produces a fusion of the genes DNAJB1 and PRKACA which, in turn, produces a chimeric transcript and protein.
View Article and Find Full Text PDFFibrolamellar hepatocellular carcinoma (FLHCC) is a rare liver malignancy in adolescents and young adults. Surgery is the mainstay of therapy for primary and metastatic disease. Most patients relapse, with development of both local and distant metastases.
View Article and Find Full Text PDFPurpose: We performed a retrospective analysis to evaluate the risk of thyroid cancer in incidental thyroid nodules (ITNs) discovered on CT in patients with a history of pediatric cancer.
Methods: With IRB approval we reviewed the records of pediatric oncology patients age ≤21y with newly detected thyroid nodules on surveillance CT of the neck, chest, chest/abdomen/pelvis, or PET/CT performed between April 2008 and March 2015. Patients with <6months of follow-up after incidental findings, a history of primary thyroid malignancy, or incomplete records were excluded.
Purpose: To evaluate factors associated with progression-free and disease-specific survival in patients with paratesticular rhabdomyosarcoma, we performed a cohort study. Also, since many patients present to our institution after initial therapy, we analyzed the effects of salvage therapy for scrotal violation.
Patients And Methods: We retrospectively reviewed the records of all consecutive patients with histologically confirmed paratesticular rhabdomyosarcoma treated at our institution between 1978 and 2015.
Purpose: Cooperative studies support complete metastasectomy in osteosarcoma (OS). Pre-operative CT is used to identify and quantify metastases and can facilitate minimally invasive techniques. Here we assess the accuracy of pre-operative CT compared to findings at thoracotomy and its change over time.
View Article and Find Full Text PDFIntroduction: Non-central nervous system (non-CNS) rhabdoid tumors tend to present at a young age and have an extremely aggressive course, with dismal overall survival rates. Inactivation of the tumor suppressor gene SMARCB1 has been shown in rhabdoid tumors regardless of anatomic location, suggesting a common genetic basis. We retrospectively analyzed our institutional experience with non-CNS rhabdoid tumors to determine overall survival and prognostic variables.
View Article and Find Full Text PDFBackground: Although nephrectomy rates are higher in children with neuroblastoma who have image-defined risk factors and/or high-risk disease who undergo resection prior to chemotherapy, no published data outline the key radiographic and clinical characteristics associated with nephrectomy.
Methods: With IRB approval, imaging studies of children undergoing primary resection of intraabdominal neuroblastoma between 2000 and 2014 were retrospectively reviewed. Fisher's exact and Wilcoxon rank-sum tests were used to compare categorical and continuous variables, respectively, with p-values adjusted for multiple testing using the false discovery rate approach.
Background: Penetrating neck trauma is uncommon in children; consequently, data describing epidemiology, injury pattern, and management are sparse. The aim of this study was to use the National Trauma Data Bank (NTDB) to describe pediatric penetrating neck trauma (PPNT).
Methods: The NTDB was queried for children (defined as <15 years old) with PPNT between years 2008 and 2012.
Purpose: Treatment of progression in high-risk neuroblastoma remains challenging despite improved survival. We retrospectively evaluated outcomes in children with a first progression that included soft-tissue masses.
Methods: We reviewed records of 903 consecutive children with high-risk neuroblastoma diagnosed between 2004 and 2014, and identified 42 whose first progression included soft-tissue masses.
J Pediatr Surg Case Rep
October 2015
Gastric volvulus is a rare post-pneumonectomy complication. Although it has been described previously, published cases are limited to an older patient population. We report the youngest case of postpneumonectomy gastric volvulus to date, occurring in an 18-year-old male with a history of inflammatory myofibroblastic pseudotumor who underwent left intrapericardial pneumonectomy, and presented 13 years later with chronic intermittent mesenteroaxial gastric volvulus.
View Article and Find Full Text PDFProc Natl Acad Sci U S A
November 2015
Fibrolamellar hepatocellular carcinoma (FLHCC) tumors all carry a deletion of ∼ 400 kb in chromosome 19, resulting in a fusion of the genes for the heat shock protein, DNAJ (Hsp40) homolog, subfamily B, member 1, DNAJB1, and the catalytic subunit of protein kinase A, PRKACA. The resulting chimeric transcript produces a fusion protein that retains kinase activity. No other recurrent genomic alterations have been identified.
View Article and Find Full Text PDFUse of electronic health records (EHRs) is an important innovation for patients in jails and prisons. Efforts to incentivize health information technology, including the Medicaid EHR Incentive Program, are generally aimed at community providers; however, recent regulation changes allow participation of jail health providers. In the New York City jail system, the Department of Health and Mental Hygiene oversees care delivery and was able to participate in and earn incentives through the Medicaid EHR Incentive Program.
View Article and Find Full Text PDFBackground: Mild traumatic brain injury (mTBI) constitutes 75% of more than 1.5 million traumatic brain injuries annually. There exists no consensus on point-of-care screening for mTBI.
View Article and Find Full Text PDFFibrolamellar hepatocellular carcinoma is a rare primary liver tumor that often arises in the absence of cirrhosis or viral hepatitis. Compared with hepatocellular carcinoma, patients are typically younger with less comorbidities. Diagnosis is often multimodal and requires a high level of suspicion, as traditional liver pathology markers, such as serum α fetoprotein and transaminases, are often normal.
View Article and Find Full Text PDFThe aim of this study was to examine if isolated fetal ventricular septal defect (VSD) is associated with trisomy 21. One hundred twenty six cases with prenatal VSD diagnosed by a pediatric cardiologist were reviewed. Cases with known risk factors for congenital heart disease, the presence of other major anomalies, soft signs for trisomy 21 or a positive screen test for trisomy 21 were excluded.
View Article and Find Full Text PDFBackground: The incidence of congenital heart defects, reported to be 5-8/1000 in term infants, is not well established in very low birth weight infants.
Objectives: To establish the incidence of congenital heart defects in VLBW infants in the neonatal intensive care unit of our institution.
Methods: A retrospective analysis of the population in the NICU at our institution was performed.
Background: The recently released Amplatzer Ductal Occluder 2 (ADO2) was designed specifically for use in small children with moderate-sized shunts and larger children with small patent ductus arterioses (PDA). We report our initial experience with this device.
Methods: Patients referred with PDA underwent occlusion using the ADO2.
Background: Cardiovascular disease causes major morbidity and is an important determinant of premature death in the paediatric chronic kidney disease (CKD) population. It is composed of three separate, although interrelated, disease processes: atherosclerosis, arteriosclerosis (i.e.
View Article and Find Full Text PDFLeft ventricular hypertrophy (LVH) is a risk factor for cardiovascular disease, and it is prevalent in children with end-stage renal disease (ESRD) and after renal transplantation (RTx) on cross-sectional studies. Our aim was to compare prospectively left ventricular mass index (LVMI) in children with ESRD, before and after RTx. Thirteen patients aged 1.
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