Cases reported "Neuroblastoma"

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1/6. Two cases of neuroblastoma presenting to the emergency department.

    neuroblastoma is the second most common malignant solid tumor in the pediatric population. Recent advances in treatment options and identification of prognostic subsets have made early detection important. Early classification into a favorable stage and subset may carry a 10-year survival of about 90% (1). With newer treatment regimens, the probability of long-term survival in patients with metastatic disease has also increased about fourfold since 1979 (2). Emergency physicians can contribute to improved morbidity and mortality with appropriate evaluation and disposition of the patient presenting with neuroblastoma. Two patients, a 6-month-old and a 2-week-old, presented to the Emergency Department with weakness. Both had a presumptive diagnosis of neuroblastoma made by the emergency physician. A brief discussion of the incidence, presentation, and diagnosis of neuroblastoma follows.
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2/6. Spontaneous regression of neuroblastoma.

    Case of spontaneous regression of neuroblastoma continue to occur in the present multimodal therapy era at institutions where physicians are prepared to withhold treatment on certain patients with residual primary or metastatic disease. From a survey of the 22 member institutions of Children's Cancer Study Group, seven hospitals submitted data on 24 neuroblastoma patients whose disease underwent regression after minimal, unusual, or no treatment. An analysis of these patients and of 33 patients form two large series in the literature shows that the majority of patients are infants with Stage II or Stage IVS disease. The spontaneous regression usually consists of complete disappearance of the disease, but in some neuroblastomas, maturation to ganglioneuroma takes place. The various factors that may influence regression are discussed.
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3/6. extracorporeal membrane oxygenation after stem cell transplant: clinical decision-making in the absence of evidence.

    OBJECTIVE: To discuss the ethical dilemmas that arise in considering innovative therapies for critically ill children when there is little data to support their use. DESIGN: Case report of a 13-yr-old patient after autologous peripheral blood stem cell transplant for stage III neuroblastoma with sepsis and hemodynamic instability who survived to discharge after a 6-day course of extracorporeal membrane oxygenation (ECMO) support. The case serves as a source of discussion of the following: the use of available data in deciding to proceed with an unproved therapy, the approach to conversations to obtain informed consent, and the need for institutional oversight and hypothesis-driven data collection to advance pediatric critical care. SETTING: Pediatric intensive care unit at a university hospital. Patient: One adolescent with stage III neuroblastoma. RESULTS: Despite a lack of data to support the use of ECMO in a neutropenic oncology patient after autologous peripheral blood stem cell transplant, our patient had clinical features that suggested he was a reasonable ECMO candidate. His family gave informed consent to use ECMO and he survived. It is ethical to consider and use innovative therapies when patient characteristics are suggestive that the therapy may be successful even in the absence of evidence. This requires physicians' attention to the best interest of the patient and should occur in the setting of informed consent and rigorous data collection. CONCLUSIONS: The boundaries among standard therapy, innovative therapy, and research can be quite fluid. This case illustrates the ethical imperative to consider therapies that may be appropriate for a critically ill child even without evidence predictive of success, to have entry criteria and treatment protocols for such therapies, and to collect data from such experiences to advance the standard of care.
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4/6. Randomization, informed consent and physicians' communication skills in pediatric oncology: a delicate balance.

    parents asked to consent to a child's randomization in a pediatric cancer clinical trial are often unprepared to grasp the implications of this scientifically crucial but seemingly unfair process. physicians must adopt specific communication skills to engage families in open dialogue from the outset in order to elicit truly shared informed consent. Starting from the case of a family with an only child affected by disseminated neuroblastoma, we wish to comment on the problems surfacing in the informed consent process for treatment and research in pediatric oncology that implicate an understanding of bioethical issues and psychological principles. Although the outcome of childhood cancer has improved dramatically over the last 30 years, with overall survival rates now exceeding 70%, there are regretfully still types and stages of cancer carrying a very high risk of death that urgently require new clinical strategies. The response to this need has been the design of experimental protocols that often entail randomized controlled trials (RCT). A large number of these trials concern stage IV neuroblastoma, acute leukemia, rhabdomyosarcoma, and other types of childhood cancers presenting great heterogeneity both in terms of localization and responsiveness to therapy. Most trials for disease relapses also include one or more randomizations. The scientific motivation justifying an RCT is the need to compare and evaluate an innovative protocol (or part thereof) with reference treatment modalities. Nevertheless, the process brings to bear the ethical dilemma of having to weigh the needs of the single afflicted child against the benefit which may ensue for a much larger patient community.
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5/6. Curability of cancer in children.

    The case records of 173 patients treated for cancer during childhood, free of disease at least 5 years later and free of disease in 1967 were reviewed. There were 84 boys and 89 girls. Tumor types were lymphomas, including one acute leukemia, sarcomas of the bone and soft parts, Wilms' tumor, neuroblastoma and carcinomas. Among the long survivors there were clusters in the less than 1-year age group and more than twelve years. Although many of the children were well adjusted in later life, others developed emotional problems. Cancer detection in a well child population for the tumors characteristically seen in the different age groups is the responsibility of the parents and physicians rendering health supervision to the children.
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6/6. spinal cord compression--do we miss it?

    Four children with spinal cord compression due to malignant tumours are presented. The severity of the condition was not initially recognized by parents, or the nature of the likely cause by the initial physicians. Lower limb asymmetrical weakness, clear-cut sensory levels, and marked pain indicate need for urgent imaging and exclusion of a space occupying lesion. In 1997 diagnosis of guillain-barre syndrome should not be made without careful prior spinal imaging.
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