Neuroblastoma Presentations in the ED

No one wants to inform a family that their child has a malignancy, but our jobs certainly place us in a position where it is occasionally necessary for us to do so. While this may not be an aspect that we look forward to, by actively considering potential malignancies on our differential diagnosis lists we can benefit the patient by offering early detection and treatment. Unfortunately, many malignancies initially have subtle and various presentations that can be misconstrued as more benign illnesses initially. Neuroblastoma is a great example of this.

Neuroblastoma Basics

1. 2nd most common malignant solid tumor in pediatric patients.
2. Malignancy arising from neural crest tissue.
   1. The pattern of these neuroblasts’ migration correlates with the sites of primary neuroblastoma (ex, sympathetic ganglia, adrenal glands).
   2. 75% of primary sites are in the abdomen.
   3. 2/3 of abdominal tumors originate from the adrenal glands.

Presentation

• Early symptoms can be very nonspecific.

1. 1. Fatigue
   2. Anorexia
   3. Fever
   4. Lymphadenopathy
   5. Bone pain
   6. Hepatosplenomegaly
   7. Weight loss
   8. Irritability
   9. Weakness.
   10. Diarrhea (4% of cases will have severe diarrhea from a tumor secreting vasoactive intestinal peptides).

• Later symptoms/findings often depend on tumor location.

1. Abdominal Mass
   1. May be asymptomatic (discovered during exam)
   2. Often noted to be hard with irregular border, in contrast to the smooth edges found with renal tumors.
   3. May lead to abdominal distension and/or constipation symptoms.
2. Focal neurologic deficit
   1. Spinal cord compression (from extension between the neuronal foramina)
   2. Weakness, limping, paralysis, or bladder/bowel dysfunction.
   3. Lower limb paresis from epidural extension of paraspinal tumor occurs in 4% of cases.
   4. Neurologic complaint plus Abdominal Mass/Discomfort should evoke consideration of neuroblastoma.
   5. Horner Syndrome (cervicothoracic ganglion can be primary site).
3. Proptosis and Periorbital Ecchymosis (Raccoon Eyes)
   1. Neuroblastoma has a predilection for periorbital metastasis.
   2. Can be mistaken for signs of physical abuse.
4. Chronic cough (posterior mediastinal mass).
5. Compression of local major blood vessels leading to venous stasis and edema.
6. Skin lesions – bluish, firm papules and nodules, similar in appearance to “blueberry muffin” skin findings in congenital rubella or CMV.
7. Opsoclonus-polymyoclonus (paraneoplasitc syndrome) – progressive ataxia, dementia, and “dancing eyes.”