5 year old boy who presents with painless skull mass and diplopia
â€¢ Bony inflitrative masses with bilateral calvarial and orbital involvement.
â€¢ Skull mass has both epidural and scalp extension.
â€¢ Orbital metastases invade superior and lateral extraconal spaces.
â€¢ Metastatic Neuroblastoma
â€¢ Langerhan histocytosis
This boy had an abdominal/retroperitoneal neuroblastoma that was diagnosed last year.
The differential diagnosis for adrenal gland calcification in children includes calcified adrenal hemorrhage, tuberculosis and histoplasmosis, and tumors, most commonly neuroblastoma and ganglioneuroma. Wolmanâ€™s disease is a reare genetic disorder of lipid metabolism associated with enlarged calcified adrenal glands and hepatosplenomegaly. Neuroblastoma is the most common abdominal malignancy in the newborn, but a rare disease with an incidence of 1:30,000. Most patients are less than two years of age; those diagnosed at less than one year of age have a better overall prognosis.
Neuroblastomas arise from cells of neural crest origin and can originate anywhere from cervical region to pelvis. Approximately two-thirds arise in the abdomen and two-thirds of these arise in the adrenal glands. 65% of patients present with metastatic disease, most often to bones, bone marrow, liver, lymph nodes and skin. 85-95% of neuroblastomas contain calcification , often visible on plain radiographs. Neuroblastomas readily extend across midline, with a characteristic tendency for vessel encasement.
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