II. Epidemiology
- Most common extracranial solid tumor in children
- Accounts for 8 to 10% of all Childhood Cancers
- Incidence: 550 new cases in U.S. per year
- Age at diagnosis
- Age <1 year in 40% of cases
- Median age: 17 months
- Age <10 years in 97% of cases
III. Pathophysiology
- Cancer derived from postganglionic sympathetic neuroblasts (from neural crest)
IV. Risk Factors
- Maternal Opiate use (esp. Codeine)
- Maternal Folate Deficiency
- Gestational Diabetes Mellitus
V. Associated Conditions
- Central Hypoventilation
- Hirschprung Disease
- Neurofibromatosis Type 1
- Turner Syndrome
VI. Presentations: Based on distribution or primary lesions
- Abdominal primary lesions (most common - 40% adrenal, 25% abdominal)
- Abdominal Pain
- Abdominal fullness
- Retroperitoneal mass, Adrenal Mass or hepatic mass (e.g. Ultrasound)
-
Chest primary lesions (15%)
- Incidental Chest XRay lesions
- Cervical or high chest region primary lesions (5%)
- Paraspinal primary lesions
- Spinal cord compression (radiculopathy, back pain, extremity weakness, Bladder dysfunction)
- Metastatic Neuroblastoma
- Fever
- Bone pain
- Limping Child
- Anemia
- Periorbital Ecchymosis or Raccoon Eyes (from retrobulbar tumor infiltration)
- Opsoclonus-myoclonus syndrome
- Blue Skin Nodules (infants with Stage 4S tumors)
VII. Evaluation
- Evaluation (advanced imaging, labs) is typically deferred to tertiary center work-up
- Urine Catecholamines (e.g. vanillylmandelic acid)
VIII. References
- Arndt in Burg (2006) Current Pediatric Therapy, Saunders Elsevier, Philadelphia, p. 1185-7
- Shohet and Nuchtern in Park (2016) UpToDate, accessed on IOS 2/19/2016
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Related Studies
| Definition (CHV) | a kind of nerve tissue cancer |
| Definition (CHV) | a kind of nerve tissue cancer |
| Definition (MEDLINEPLUS) |
Neuroblastoma is a cancer that forms in your nerve tissue. It usually begins in the adrenal glands, which sit atop your kidneys. It may also begin in your neck, chest or spinal cord. The cancer often begins in early childhood. Sometimes it begins before a child is born. By the time doctors find the cancer, it has usually spread to other parts of the body. The most common symptoms are
Treatments include surgery, radiation therapy, chemotherapy, biologic therapy, or a combination. Biologic therapy boosts your body's own ability to fight cancer. Sometimes before giving treatment, doctors wait to see whether symptoms get worse. This is called watchful waiting. NIH: National Cancer Institute |
| Definition (NCI_CDISC) | A malignant neoplasm composed of neuroblastic cells. |
| Definition (NCI_NCI-GLOSS) | Cancer that arises in immature nerve cells and affects mostly infants and children. |
| Definition (NCI) | A neuroblastic tumor characterized by the presence of neuroblastic cells, the absence of ganglion cells, and the absence of a prominent Schwannian stroma formation. |
| Definition (MSH) | A common neoplasm of early childhood arising from neural crest cells in the sympathetic nervous system, and characterized by diverse clinical behavior, ranging from spontaneous remission to rapid metastatic progression and death. This tumor is the most common intraabdominal malignancy of childhood, but it may also arise from thorax, neck, or rarely occur in the central nervous system. Histologic features include uniform round cells with hyperchromatic nuclei arranged in nests and separated by fibrovascular septa. Neuroblastomas may be associated with the opsoclonus-myoclonus syndrome. (From DeVita et al., Cancer: Principles and Practice of Oncology, 5th ed, pp2099-2101; Curr Opin Oncol 1998 Jan;10(1):43-51) |
| Definition (CSP) | common neoplasm of early childhood arising from neural crest cells in the sympathetic nervous system, and characterized by diverse clinical behavior, ranging from spontaneous remission to rapid metastatic progression and death; histologic features include uniform round cells with hyperchromatic nuclei arranged in nests and separated by fibrovascular septa; the most common intraabdominal malignancy of childhood, but it may also arise from thorax, neck, or rarely occur in the central nervous system. |
| Concepts | Neoplastic Process (T191) |
| MSH | D009447 |
| ICD10 | M9500/3 |
| SnomedCT | 432328008, 269507008, 189931003, 87364003 |
| English | Neuroblastoma, Neuroblastomas, NEUROBLASTOMA, [M]Neuroblastoma NOS, Neuroblastoma NOS, neuroblastoma (diagnosis), neuroblastoma, Neuroblastoma (disorder), Neuroblastoma [Disease/Finding], neuroblastomas, [M]Neuroblastoma NOS (morphologic abnormality), NEUROBLASTOMA (SCHWANNIAN STROMA-POOR), NEURAL CREST TUMOR, MALIGNANT, NEUROBLASTOMA, MALIGNANT, NB - Neuroblastoma, Neuroblastoma (morphologic abnormality), neuroblastoma; unspecified site, Neuroblastoma, NOS, Neuroblastoma (Schwannian Stroma-Poor) |
| French | NEUROBLASTOME, Neuroblastome SAI, Neuroblastome, Sympathoblastome |
| Portuguese | NEUROBLASTOMA, Neuroblastoma NE, Neuroblastoma |
| Spanish | NEUROBLASTOMA, Neuroblastoma NEOM, neuroblastoma (trastorno), [M]neuroblastoma, SAI, [M]neuroblastoma, SAI (anomalía morfológica), neuroblastoma (anomalía morfológica), neuroblastoma, SAI, neuroblastoma, Neuroblastoma |
| German | NEUROBLASTOM, Neuroblastom NNB, Neuroblastom |
| Dutch | neuroblastoom NAO, neuroblastoom; niet gespecificeerde lokalisatie, neuroblastoom, Blastoom, neuro-, Neuroblastoom |
| Italian | Neuroblastoma NAS, Neuroblastoma |
| Japanese | 神経芽腫, 神経芽腫ＮＯＳ, ｼﾝｹｲｶﾞｼｭ, ｼﾝｹｲｶﾞｼｭNOS |
| Swedish | Neuroblastom |
| Czech | neuroblastom, Neuroblastom, Neuroblastom NOS |
| Finnish | Neuroblastooma |
| Russian | NEIROBLASTOMA, НЕЙРОБЛАСТОМА |
| Croatian | NEUROBLASTOM |
| Polish | Zwojak zarodkowy współczulny, Neuroblastoma, Nerwiak współczulny złośliwy, Nerwiak zarodkowy współczulny, Nerwiak zarodkowy |
| Hungarian | Neuroblastoma k.m.n., Neuroblastoma |
| Norwegian | Nevroblastom |
