Daniel C. West

Randomized Controlled Trial of Interval-Compressed Chemotherapy for the Treatment of Localized Ewing Sarcoma: A Report From the Children's Oncology Group

PURPOSE Chemotherapy with alternating vincristine-doxorubicin-cyclophosphamide and ifosfamide-etoposide cycles and primary tumor treatment with surgery and/or radiation therapy constitute the usual approach to localized Ewing sarcoma in North America. We tested whether chemotherapy intensification through interval compression could improve outcome. PATIENTS AND METHODS This was a prospective, randomized controlled trial for patients younger than 50 years old with newly diagnosed localized extradural Ewing sarcoma. Patients assigned to standard and intensified treatment were to begin chemotherapy cycles every 21 and 14 days, respectively, provided an absolute neutrophil count greater than 750×10(6)/L and a platelet count greater than 75×10(9)/L. Patients received vincristine (2 mg/m2), doxorubicin (75 mg/m2), and cyclophosphamide (1.2 g/m2) alternating with ifosfamide (9 g/m2) and etoposide (500 mg/m2) for 14 cycles, with filgrastim (5 mg/kg per day; maximum, 300 mg) between cycles. Primary tumor treatment (surgery, radiation, or both) was to begin at week 13 (after four cycles in the standard arm and six cycles in the intensified arm). The primary end point was event-free survival (EFS). The study is registered at ClinicalTrials.gov (identifier: NCT00006734). RESULTS Five hundred eighty-seven patients were enrolled and randomly assigned, and 568 patients were eligible, with 284 patients in each regimen. For all cycles, the median cycle interval for standard treatment was 21 days (mean, 22.45 days); for intensified treatment, the median interval was 15 days (mean, 17.29 days). EFS at a median of 5 years was 65% in the standard arm and 73% in the intensified arm (P=.048). The toxicity of the regimens was similar. CONCLUSION For localized Ewing sarcoma, chemotherapy administered every 2 weeks is more effective than chemotherapy administered every 3 weeks, with no increase in toxicity.

Detection of circulating tumor cells in patients with Ewing's sarcoma and peripheral primitive neuroectodermal tumor.

PURPOSE To determine the feasibility of detecting Ewings sarcoma (ES) or peripheral primitive neuroectodermal tumor (PNET) through a reverse-transcriptase polymerase chain reaction (RT-PCR) of the t(11;22)(q24;q12) fusion transcript in blood and bone marrow samples from patients with these neoplasms. PATIENTS AND METHODS Peripheral-blood (PB) and/or bone marrow aspirate (BM) samples were obtained from 28 patients with ES or PNET at initial presentation or at relapse. Patients were divided into two groups: newly diagnosed patients with nonmetastatic disease and those with metastatic/relapsed disease. RNA was extracted from fractionated BM and PB samples, and RT-PCR was performed for the EWS/HumFLI1 fusion mRNA was transcribed across the t(11;22) breakpoint. RESULTS Among the 16 patients with nonmetastatic disease, three of 16 were RT-PCR positive for EWS/HumFLI1 RNA in BM and three of 10 were positive in PB. The total number of nonmetastatic patients who were positive in either PB or BM was four of 16 (25%). Among patients with metastatic/relapsed disease, two of six were positive in BM and five of 10 were positive in PB. The total fraction of patients with metastatic/relapsed disease that was positive in either BM or PB was six of 12 (50%). CONCLUSION In this study, we show that it is possible to amplify the EWS/HumFLI1 RNA by RT-PCR from the BM and PB of a subset of patients with both nonmetastatic and metastatic ES or PNET, which implies that occult tumor cells are present at these sites. The true biologic and clinical meaning of this information is unknown. However, it does suggest a possible application of RT-PCR for the monitoring of residual disease in patients who are undergoing therapy for ES or PNET. This approach may permit early identification of patients who may benefit from alternative therapy or who may be spared possible overtreatment.

Concept mapping assessment in medical education: a comparison of two scoring systems

Background  Concept mapping has the potential to measure important aspects of a students evolving knowledge framework in a way that conventional examinations cannot. This is important because development of an elaborate and well‐structured knowledge framework is a critical step toward becoming an expert in a particular field. Little is known about the best way to score concept maps in the setting of medical education. Therefore, as a preliminary step in addressing this question, we compared two different scoring systems for validity: a structural method based on the organization of a maps hierarchical structure and a relational method based, not on structure, but on the quality of each individual map component.

Clinical features and outcomes in patients with extraskeletal Ewing sarcoma.

Ewing sarcoma can arise in either bone or soft tissue. The purpose of this study was to investigate whether patient characteristics, treatment strategies, and outcomes differ between skeletal Ewing sarcoma and extraskeletal Ewing sarcoma (EES).

Development, implementation, and dissemination of the I-PASS handoff curriculum: A multisite educational intervention to improve patient handoffs

Patient handoffs are a key source of communication failures and adverse events in hospitals. Despite Accreditation Council for Graduate Medical Education requirements for residency training programs to provide formal handoff skills training and to monitor handoffs, well-established curricula and validated skills assessment tools are lacking. Developing a handoff curriculum is challenging because of the need for standardized processes and faculty development, cultural resistance to change, and diverse institution- and unit-level factors. In this article, the authors apply a logic model to describe the process they used from June 2010 to February 2014 to develop, implement, and disseminate an innovative, comprehensive handoff curriculum in pediatric residency training programs as a fundamental component of the multicenter Initiative for Innovation in Pediatric Education–Pediatric Research in Inpatient Settings Accelerating Safe Sign-outs (I-PASS) Study. They describe resources, activities, and outputs, and report preliminary learner outcomes using data from resident and faculty evaluations of the I-PASS Handoff Curriculum: 96% of residents and 97% of faculty agreed or strongly agreed that the curriculum promoted acquisition of relevant skills for patient care activities. They also share lessons learned that could be of value to others seeking to adopt a structured handoff curriculum or to develop large-scale curricular innovations that involve redesigning firmly established processes. These lessons include the importance of approaching curricular implementation as a transformational change effort, assembling a diverse team of junior and senior faculty to provide opportunities for mentoring and professional development, and linking the educational intervention with the direct measurement of patient outcomes.

Successful self-directed lifelong learning in medicine: A conceptual model derived from qualitative analysis of a national survey of pediatric residents

Purpose Self-directed lifelong learning is integral to medical professionalism, yet how best to encourage its development during clinically intensive training is unknown. The authors develop a model for successful self-directed learning by analyzing qualitative data from a national survey of residents. Method Pediatric and medicine/pediatric residents at 46 training programs completed a Web-based survey in 2008–2009. Self-reported barriers to and strategies for achieving self-directed learning goals were systematically analyzed through inductive iterative review. Results A total of 992 out of 1,739 (57%) residents responded. Barriers to achieving self-directed learning goals were categorized into difficulty with personal reflection, environmental strain, competing demands, difficulty with goal generation, and problems with plan development and implementation. Strategies for achieving learning goals included creating goals that were important (relevant to the learner and prioritized by the learner as important to achieve), specific (with broad goals broken down into incremental steps and a specific plan for each step), measurable, accountable (with reminder and tracking systems and building in internal and external accountability), realistic (achievable goals which utilize existing opportunities and constant self-adjustment), and included a timeline for completing the goal (and incorporating the goal into their daily routine). Conclusions On the basis of the data, the authors propose a conceptual model for self-directed lifelong learning involving creation of learning goals and plan development based on individual reflection and self-assessment, and continual revision of goals and/or plans based on degree of goal attainment. This model could be broadly applicable throughout medical education.

Ultrasound and MR imaging of fibromatosis colli (sternomastoid tumor of infancy)

Abstract The sonographic and CT findings of fibromatosis colli (sternomastoid tumor of infancy) have been described, but the MRI appearance has been reported in only one case in which the mass resolved over time. This case describes the detailed MRI findings in a biopsy-proven case of fibromatosis colli; the signal intensity of the mass on T2-weighted images was slightly less than on gradient-recalled T1-weighted images, consistent with the presence of some fibrous tissue within the muscle mass. The involved portion of the muscle was better defined on MRI than sonography. MRI was helpful in demonstrating the signal characteristics of the mass; localizing the mass to within the sternocleidomastoid muscle; and demonstrating clear surrounding fascial planes with lack of associated lymphadenopathy, airway compression, vascular encasement, bone involvement or intracranial/intraspinal extension associated with other neck masses.

Factors affecting resident performance: development of a theoretical model and a focused literature review.

Purpose The clinical performances of physicians have come under scrutiny as greater public attention is paid to the quality of health care. However, determinants of physician performance have not been well elucidated. The authors sought to develop a theoretical model of physician performance, and explored the literature about factors affecting resident performance. Method Using expert consensus panel, in 2002–03 the authors developed a hypothesis-generating model of resident performance. The developed model had three input factors (individual resident factors, health care infrastructure, and medical education infrastructure), intermediate process measures (knowledge, skills, attitudes, habits), and final health outcomes (affecting patient, community and population). The authors used factors from the model to focus a PubMed search (1967–2002) for all original articles related to the factors of individual resident performance. Results The authors found 52 original studies that examined factors of an individual residents performance. They describe each studys measurement instrument, study design, major findings, and limitations. Studies were categorized into five domains: learning styles/personality, social/financial factors, practice preferences, personal health, and response to job environment. Few studies examined intermediate or final performance outcomes. Most were single-institution, cross-sectional, and survey-based studies. Conclusions Attempting to understand resident performance without understanding factors that influence performance is analogous to examining patient adherence to medication regimens without understanding the individual patient and his or her environment. Based on a systematic review of the literature, the authors found few discrete associations between the factors of individual resident and the residents actual job performance. Additionally, they identify and discuss major gaps in the educational literature.

Factors associated with successful self-directed learning using individualized learning plans during pediatric residency.

OBJECTIVE The aim of this study was to determine whether resident or program characteristics are associated with effective self-directed learning of residents. METHODS A cross-sectional survey of pediatric and medicine/pediatric residents and program directors from a nationally representative sample of residency programs was conducted. Self-directed learning efficacy was measured by resident-reported progress on learning goals from their most recent individualized learning plan (ILP). Multilevel linear regression models were used to analyze the relationship between learner and program characteristics and self-directed learning efficacy of residents. RESULTS All program directors of participating programs (N = 46) completed the survey; the response rate from residents was 57% (992/1739). At the time of the survey, 78% of residents had previously written an ILP. Most residents achieved moderate self-directed learning efficacy. The most important factors associated with greater self-directed learning efficacy included using a system to track ones own progress in achieving learning goals, higher score on a propensity toward lifelong learning scale, and reporting greater confidence in self-directed learning abilities. Program characteristics, including program-level support for ILPs, had little or mixed association with resident self-directed learning efficacy. CONCLUSIONS The most important factors associated with effective self-directed learning were resident characteristics. Our findings imply that residency programs should invest their limited resources in curricula that help residents develop measurable goals and systems for tracking progress toward goal attainment. Since propensity toward lifelong learning was an important factor, medical schools and residency training programs should enhance their efforts to develop this characteristic in learners.

Renal cell carcinoma in long-term survivors of advanced stage neuroblastoma in early childhood

BackgroundRenal cell carcinoma (RCC) is rare in children and comprises only 1–3% of all pediatric primary renal tumors. Recently, several case reports have described RCC developing in patients previously treated for advanced stage neuroblastoma (NB).Methods and resultsOur experience with four patients treated for advanced stage NB during early childhood who developed RCC later in life are added to 14 others in the literature.ConclusionThese patients and our review of the literature suggest an association between RCC and NB that warrants further study.