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Pediatric emergency medicine trisk 2698 2698

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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 per 7,000 live births, neuroblastoma preferentially affects very young children; 50% of cases are diagnosed by age and 90% by age 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

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