Neuroblastoma (peds)

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Background

Neuroblastoma: (a) Abdominal CT-scan: large heterogeneous right abdominal mass with a tumor thrombus of the inferior vena (Arrow). (b) Gross examination of the tumor: heterogeneous partially necrotizing tumor of the right adrenal gland. (c):Histology of the tumor: neuroblastoma poorly differentiated.
  • Arises from primitive ganglion cells of sympathetic nervous system
  • Synthesize and secrete catecholamines
  • Most common neoplasm in 1st yr of life
  • May arise anywhere along sympathetic nervous system
    • Adrenal gland (40%)
    • Abdomen (25%)
    • Chest (15%)
  • Mets are most common in lymph nodes, bone marrow, cortical bone, dura, orbits, liver, and skin

Clinical Features

  • Depends on location
    • Abdominal mass
      • 2/3 of primary neuroblastoma arise in the abdomen[1]
    • Abdominal pain or constipation
    • Proptosis or periorbital ecchymoses
    • Horner syndrome: miosis, ptosis, anhidrosis
    • Paraspinal mass
    • Spinal cord compression, which can cause localized back pain or weakness
    • Systemic symptoms, bone pain, anemia
  • Opsoclonus-myoclonus: rapid, dancing eye movements, rhythmic jerking of trunk or limbs, and/or ataxia
  • Neutropenia/pancytopenia suggests bone marrow involvement

Differential Diagnosis

  • Wilms' tumor
  • Hepatoblastoma
  • Lymphoma
  • Other adrenal gland tumor

Evaluation

Abdominal CT demonstrating (A) a heterogeneously enhancing mass in the pancreatic body and tail with (B) encasement of the celiac axis.
Neuroblastoma accompanied by hyperaldosteronism.
  • Catecholamines; VMA and HVA of serum and urine
  • CT or MRI
    • Although US is often the initial radiologic study of choice for abdominal mass, primary tumor site and possible nodes on are better evaluated on CT or MRI
  • CXR
  • Biopsy for definitive diagnosis

Management

  • No acute treatment

Disposition

  • Admission for tumor biopsy and further workup

See Also

External Links

References