Neurology Book

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Neuroblastoma

Aka: Neuroblastoma, Schwannian Stroma-Poor, Malignant Neural Crest Tumor
  1. Epidemiology
    1. Most common extracranial solid tumor in children
    2. Accounts for 8 to 10% of all Childhood Cancers
    3. Incidence: 550 new cases in U.S. per year
    4. Age at diagnosis
      1. Age <1 year in 40% of cases
      2. Median age: 17 months
      3. Age <10 years in 97% of cases
  2. Pathophysiology
    1. Cancer derived from postganglionic sympathetic neuroblasts (from neural crest)
  3. Risk Factors
    1. Maternal Opiate use (esp. Codeine)
    2. Maternal Folate Deficiency
    3. Gestational Diabetes Mellitus
  4. Associated Conditions
    1. Central Hypoventilation
    2. Hirschprung Disease
    3. Neurofibromatosis Type 1
    4. Turner Syndrome
  5. Presentations: Based on distribution or primary lesions
    1. Abdominal primary lesions (most common - 40% adrenal, 25% abdominal)
      1. Abdominal Pain
      2. Abdominal fullness
      3. Retroperitoneal mass, Adrenal Mass or hepatic mass (e.g. Ultrasound)
    2. Chest primary lesions (15%)
      1. Incidental Chest XRay lesions
    3. Cervical or high chest region primary lesions (5%)
      1. Horner Syndrome (Miosis, Ptosis, anhydrosis)
    4. Paraspinal primary lesions
      1. Spinal cord compression (radiculopathy, back pain, extremity weakness, Bladder dysfunction)
    5. Metastatic Neuroblastoma
      1. Fever
      2. Bone pain
      3. Limping Child
      4. Anemia
      5. Periorbital Ecchymosis or Raccoon Eyes (from retrobulbar tumor infiltration)
      6. Opsoclonus-myoclonus syndrome
      7. Blue Skin Nodules (infants with Stage 4S tumors)
  6. Evaluation
    1. Evaluation (advanced imaging, labs) is typically deferred to tertiary center work-up
    2. Urine Catecholamines (e.g. vanillylmandelic acid)
  7. References
    1. Arndt in Burg (2006) Current Pediatric Therapy, Saunders Elsevier, Philadelphia, p. 1185-7
    2. Shohet and Nuchtern in Park (2016) UpToDate, accessed on IOS 2/19/2016

Neuroblastoma (C0027819)

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

  • A lump in the abdomen, neck or chest
  • Bulging eyes
  • Dark circles around the eyes
  • Bone pain
  • Swollen stomach and trouble breathing in babies
  • Painless, bluish lumps under the skin in babies
  • Inability to move a body part

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, シンケイガシュ, シンケイガシュ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
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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