Overview
What is pediatric neuroblastoma?
What are the chromosomal and molecular markers of pediatric neuroblastoma?
What are the most common anatomic sites for pediatric neuroblastoma?
How are pediatric neuroblastoma detected?
What is the pathophysiology of pediatric neuroblastoma
What are the histologic characteristics of pediatric neuroblastoma?
What is the Shimada histopathologic classification system of pediatric neuroblastoma?
What is the US prevalence of pediatric neuroblastoma?
What is the global prevalence of pediatric neuroblastoma?
What are the mortality rates of pediatric neuroblastoma?
What is the morbidity associated with pediatric neuroblastoma?
What are the racial predilections of pediatric neuroblastoma?
What are the sexual predilections of pediatric neuroblastoma?
Which age groups have the highest prevalence of pediatric neuroblastoma?
Presentation
Which clinical history findings are characteristic of pediatric neuroblastoma?
Which physical findings are characteristic of pediatric neuroblastoma?
What causes pediatric neuroblastoma?
DDX
What are the differential diagnoses for Pediatric Neuroblastoma?
Workup
What is the role of lab tests in the workup of pediatric neuroblastoma?
What is the role of imaging studies in the workup of pediatric neuroblastoma?
What is the role of biopsy in the workup of pediatric neuroblastoma?
How is pediatric neuroblastoma diagnosed?
How is pediatric neuroblastoma staged?
Treatment
How is pediatric neuroblastoma treated?
How is low-risk pediatric neuroblastoma treated?
How is intermediate-risk pediatric neuroblastoma treated?
How is high-risk pediatric neuroblastoma treated?
Which medications are being investigated for the treatment of pediatric neuroblastoma?
What is the role of surgery in the treatment of pediatric neuroblastoma?
Which specialist consultations are beneficial to patients with pediatric neuroblastoma?
Which dietary modifications are used in the treatment of pediatric neuroblastoma?
Which activity modifications are used in the treatment of pediatric neuroblastoma?
Medications
What is the role of medications in the treatment of pediatric neuroblastoma?
Follow-up
What is included in the long-term monitoring of pediatric neuroblastoma?
When is inpatient care indicated for the treatment of pediatric neuroblastoma?
Which medications are used in the treatment of pediatric neuroblastoma?
What is the role of a primary care provider in the treatment of pediatric neuroblastoma?
How is pediatric neuroblastoma prevented?
What are the possible complications of pediatric neuroblastoma?
Which factors are used to determine response to treatment and prognosis of pediatric neuroblastoma?
What is the prognosis of pediatric neuroblastoma?
What are the criteria used to evaluate response to treatment in pediatric neuroblastoma?
What is included in patient education about pediatric neuroblastoma?
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Histologic subtypes of neuroblastoma. Top right panel, neuroblastoma: A monotonous population of hyperchromatic cells with scant cytoplasm. Bottom left panel, ganglioneuroblastoma: Increased schwannian stroma. Bottom right panel, ganglioneuroma: Mature ganglion cell with schwannian stroma.
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CT scan of abdomen in a patient with a retroperitoneal mass arising from the upper pole of the left kidney and elevated urine catecholamines.
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MRI of a left adrenal mass. The mass was revealed by fetal ultrasonography at 30 weeks' gestation. During infancy, the mass was found on the inferior pole of the left adrenal and was completely resected. Before surgery, the metastatic workup was negative. Surgical pathology service confirmed a diagnosis of neuroblastoma. After 3 years of follow-up care, no recurrence was observed.
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A one-week-old neonate had abdominal ultrasonography for evaluation of projectile vomiting. A right adrenal mass (100% cystic) was an incidental finding. Evaluation of the mass by CT was consistent with an adrenal bleed (3.6 x 3.1 x 2.4 cc). The infant was followed at 2 weeks (2-dimensional size diminished to 1.5 x. 2.4 cm2 on ultrasonography) and then at 6 weeks to document that the adrenal bleed continued to involute. Urine catecholamines were normal.
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Table. A Consensus Pretreatment Classification schema by the International Neuroblastoma Risk Group (INRG). This schema is based in the INRG stage, age, histologic category, tumor grade of differentiation, MYCN sastus, 11q-aberrations and DNA ploidy. A combination of these characteristics results in four risk groups noted in the last column: very low, low, intermediate and high risk, with the following 5 year EFS: >85%, >75%-85%, >50%-75%, and < 50%. These risk groups are distributed among the different stages and labeled alphabetically from A to R (without letters L and M to avoid confusion with the INRG stage notation). Notations in the table are as follow: L1, localized tumor confined to one body compartment; L2, locoregional tumor with presence of one or more risk factors defined radiologically; M, distant metastatic disease (except stage MS); MS, metastatic disease confined to skin, liver and/or bone marrow in children < 18 months of age. GN, ganglioneuroma; GNB, ganglioneuroblastoma; Amp, amplified; n/amp, not amplified. (Adapted from The International Neuroblastoma Risk Group (INRG) Classifications System: An INRG Task Force Report by Cohn, et al. Journal of Clinical Oncology 27(2):289-297, 2009).