Neuroblastoma (peds)

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
  • 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
  • paraneoplastic syndrome highly associated with neuroblastoma
• 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