Background: The Joint Commission defines a sentinel event as "surgery or other invasive procedure performed at the wrong site, on the wrong patient, or that is the wrong (unintended) procedure for a patient regardless of the type of procedure or the magnitude of the outcome." At our institution, we observed a low but consistent rate of incorrect surgical line placement for pediatric patients with cancer.
Study Design: Following quality improvement methodology and using the resources available on a large academic medical campus we designed and implemented a new multi-factorial process to schedule and place surgical central lines for pediatric patients with cancer.
J Pediatr Hematol Oncol
March 2024
Metastatic central nervous system (CNS) involvement is rare in pediatric primary extracranial Ewing sarcoma (ES). We describe the incidence and course of 6 patients with extracranial ES who developed metastatic CNS lesions treated at a single institution. The median time to CNS disease detection was 16.
View Article and Find Full Text PDFPurpose: Effective, empathic communication is crucial for pediatric oncology clinicians when discussing palliative and end-of-life (PC/EOL) care with parents of children with cancer. Unfortunately, many parents report inadequate communication at these distressing times. This study evaluates the communication skills training (CST) clinicians received to deliver a PC/EOL communication intervention as part of a multi-site randomized-controlled trial (RCT).
View Article and Find Full Text PDFPurpose: In the absence of a standardized tool to assess the quality of pediatric hematology/oncology training programs, the Education Program Assessment Tool (EPAT) was conceptualized as a user-friendly and adaptable tool to evaluate and identify areas of opportunity, pinpoint needed modifications, and monitor progress for training programs around the world.
Methods: The development of EPAT consisted of three main phases: operationalization, consensus, and piloting. After each phase, the tool was iteratively modified based on feedback to improve its relevance, usability, and clarity.
Background: Until recently, no uniform requirements for parental leave (PL) existed in graduate medical education. We implemented a national survey, with the objective of ascertaining fellows' perceptions of PL policies and their impact. This is the first study to focus exclusively on pediatric subspecialty fellows.
View Article and Find Full Text PDFAnn Otol Rhinol Laryngol
February 2022
Introduction: Cancer predisposition syndromes are germline pathogenic variants in genes that greatly raise the risk of developing neoplastic diseases. One of the most well-known is Li-Fraumeni syndrome (LFS), which is due to pathogenic variants in the gene. Children with LFS have higher risks for multiple malignancies before adulthood, often with rare and aggressive subtypes.
View Article and Find Full Text PDFThe detection of tumor-specific nucleic acids from blood increasingly is being used as a method of liquid biopsy and minimal residual disease detection. However, achieving high sensitivity and high specificity remains a challenge. Here, we perform a direct comparison of two droplet digital PCR (ddPCR)-based detection methods, circulating plasma tumor RNA and circulating plasma tumor DNA (ptDNA), in blood samples from newly diagnosed Ewing sarcoma patients.
View Article and Find Full Text PDFDICER1 syndrome is a rare hereditary cancer predisposition syndrome that has relevance to pediatric urology providers due to its association with many various pediatric genitourinary malignancies. We describe the case of a pediatric patient who was eventually diagnosed with a pathogenic DICER1 germline variant after undergoing resection of a cystic nephroma and pleuropulmonary blastoma.
View Article and Find Full Text PDFWith the increasing numbers of individuals surviving a diagnosis of cancer, an aging population, and more individuals experiencing multi-morbidity, primary care providers (PCPs) are seeing more patients with a history of cancer. Effective strategies are needed to adequately prepare the primary care workforce for the phase of cancer care now widely recognized as survivorship. A survivorship education program for rural primary care practices was developed using a community engagement process and delivered at the practice level by community health liaisons.
View Article and Find Full Text PDFLangerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplasia with a highly variable clinical presentation affecting people of all ages. Mutations in BRAF V600E are the most identifiable molecular alteration in LCH although its incidence in pediatric patients with isolated pituitary stalk involvement is not well described. Pediatric patients with LCH and isolated pituitary stalk involvement typically present with central diabetes insipidus.
View Article and Find Full Text PDFSertoli-Leydig cell tumors (SLCTs) are rare ovarian neoplasms in pediatric patients. More exceedingly rare are SLCTs that also contain heterologous rhabdomyosarcoma (RMS) elements. For these patients, there is no standardized treatment.
View Article and Find Full Text PDFBackground: Primary hemophagocytic lymphohistiocytosis is a rare syndrome characterized by immune dysregulation and hyperinflammation. It typically manifests in infancy and is associated with high mortality.
Methods: We investigated the efficacy and safety of emapalumab (a human anti-interferon-γ antibody), administered with dexamethasone, in an open-label, single-group, phase 2-3 study involving patients who had received conventional therapy before enrollment (previously treated patients) and previously untreated patients who were 18 years of age or younger and had primary hemophagocytic lymphohistiocytosis.
Background: Polycystic kidney disease (PKD) is an inherited condition characterized by progressive development of end-stage renal disease, hypertension, hepatic fibrosis, and cysts in the kidney, liver, pancreas, spleen, thyroid, and epididymis. While malignancies have been reported in association with PKD in adults, the incidence of malignancies in children with PKD is not currently known.
Methods: We report on five patients with a known history of PKD who developed a malignancy as children at the University of California, Los Angeles and the University of Colorado Anschutz Medical Campus.
The incidence of central nervous system (CNS) involvement in patients with rhabdomyosarcoma (RMS) is low, and the outcome is dismal. We present a single institution analysis of CNS involvement of pediatric RMS. In 59 patients, the prevalence of CNS involvement was 11.
View Article and Find Full Text PDFPurpose: To determine, using the National Cancer Database (NCDB), the impact of the surgery to radiation therapy interval (SRI) on survival in contemporary patients with Wilms tumor (WT).
Methods And Materials: The NCDB was queried for patients aged ≤25 years diagnosed from 2004 to 2013 with unilateral WT who underwent definitive surgery and radiation therapy. The SRI was calculated for each patient.
Purpose: The best method for diagnosing pediatric nonnephroblastoma solid intraabdominal tumors is unknown. We hypothesized that core needle biopsy (CNB) is noninferior to open wedge biopsy (OWB) for pathologic diagnosis.
Methods: We prospectively enrolled children aged 1day to 17years with radiographic evidence of nonnephroblastoma solid intraabdominal tumors scheduled for OWB from 5/2013 to 12/2015 at a single institution.
Introduction: Testicular germ cell tumors (GCTs) are the most common solid tumor among adolescent and young adult (AYA) males. AYA patients with GCTs most typically have non-seminoma compared with seminoma, and accordingly there are fewer data reported on the AYA experience with testicular seminoma.
Objective: To evaluate national trends in postoperative treatment and overall survival (OS) outcomes in testicular seminoma by age group, specifically comparing AYAs with older adults.
Background: The ability of intraoperative frozen section (IFS) to reliably diagnose renal tumors in children and adolescents is largely unknown. The objective of our study is to evaluate the ability of IFS to establish a histologic diagnosis for renal tumors in this population.
Methods: We reviewed our experience with patients who underwent IFS at the time of surgery for a renal tumor suspicious for malignancy from 2005 to 2015.
Background: In adolescents, approximately 90% of testicular germ cell tumors (T-GCTs) are non-seminomas (NS T-GCTs). Few studies have evaluated the impact of age, specifically in adolescence, on outcomes of NS T-GCTs.
Objective: The purpose of this study was to review all patients diagnosed with NS T-GCTs in the Surveillance, Epidemiology, and End Results (SEER) database to evaluate the association between age (adolescents vs.
Purpose: The purpose of this study was to evaluate the risk of cardiac death in pediatric Hodgkin's lymphoma (HL) survivors and identify high-risk groups that may need additional surveillance.
Methods: The Surveillance, Epidemiology and End Results program database was queried to analyze the rates of radiation therapy (RT) use and cardiac-specific mortality (CSM) in HL patients, aged 0-21 years, treated from 1973 to 2007. Primary endpoint was cardiac mortality.
Purpose: Describe the development and evolution of a primary-care-based, multidisciplinary clinic to support the ongoing care of adult survivors of childhood cancer.
Methods: A consultative clinic for adult survivors of childhood cancer has been developed that is located in an adult, academic internal medicine setting and is based on a long-term follow-up clinic model available at Children's Hospital Colorado.
Results: The clinic opened in July 2008.
Purpose: Non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) are a heterogeneous group of sarcomas that encompass over 35 histologies. With an incidence of ∼500 cases per year in the United States in those <20 years of age, NRSTS are rare and therefore difficult to study in pediatric populations. We used the large Surveillance, Epidemiology, and End Results (SEER) database to validate the prognostic ability of the Children's Oncology Group (COG) risk classification system and to define patient, tumor, and treatment characteristics.
View Article and Find Full Text PDFPurpose: Anterior mediastinal masses (AMM) pose a diagnostic challenge to surgeons, oncologists, anesthesiologists, intensivists, and interventional radiologists as induction of general anesthesia can cause airway obstruction and cardiovascular collapse. We hypothesized that in the majority of patients, diagnosis can be obtained through biopsy of extrathoracic tissue.
Methods: We performed a retrospective review of all patients in the solid tumor oncology clinic with a diagnosis of AMM between 2002 and 2012 including preoperative evaluation and management prior to obtaining a tissue diagnosis, clinical course and complications.
Background: Minimally invasive surgical (MIS) techniques have become an established part of the care of the adult oncology patient. As surgeons have become more experienced with these advances in technique, MIS has recently seen an expanding role in the diagnosis and treatment of pediatric malignancies. We hypothesize that MIS techniques can be used to provide reliable diagnosis and safe therapeutic resection of many pediatric malignancies.
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