Pediatric emergency medicine trisk 2698 2698

Neuroblastoma has a wide range of clinical presentations, depending on tumor
location as well as duration of symptoms. A high index of suspicion should be
maintained in diagnosing these malignancies.
CLINICAL PEARL AND PITFALLS
The periorbital ecchymoses of neuroblastoma and the subcutaneous
pigmented lesions of stage 4S neuroblastoma seen in infants can both be
mistaken for child abuse.

Current Evidence
Neuroblastoma is derived from neural crest cells that exist within the adrenal
medulla and along the sympathetic chain. It is the most common solid tumor of
childhood outside the CNS, accounting for 7% to 10% of pediatric tumors overall.
With an incidence of 1 per 7,000 live births, neuroblastoma preferentially affects
very young children; 50% of cases are diagnosed by age 2 and 90% by age 5. In
approximately two-thirds of cases, the primary tumor is in the abdomen, specifically
in the adrenal gland.

Clinical Considerations
Clinical Recognition
Neuroblastoma presentation can be clinically variable, depending on location. An
abdominal mass may be present, and depending on its size may be initially
asymptomatic. Larger masses may cause GI dysmotility, such as constipation or
bowel obstruction, as well as inability to tolerate oral intake and secondary cachexia.
Bone pain may develop if the tumor involves osseous sites. Marrow replacement can
cause signs or symptoms of anemia, thrombocytopenia, or neutropenia. Large
masses of the chest or abdomen may impair pulmonary function and cause
respiratory distress.
Potentially life-threatening or organ-threatening complications of neuroblastoma
include the development of SVC syndrome from a mass in the posterior
mediastinum that extends anteriorly or cord compression from tumor growing
through the neural foramina into the spinal canal. See sections on “Tumors of the

Thorax” and “Tumors in and Around the Spinal Cord” for the approach to these
complications. However, at times neuroblastoma presents in a healthy, wellappearing child with an abdominal mass incidentally detected. Other unique
presentations include ipsilateral Horner syndrome (ptosis, miosis, and anhidrosis)
from involvement of the cervical sympathetic ganglia, “raccoon eyes” from
periorbital bone and soft tissue involvement causing proptosis and ecchymosis, and