NEUROBLASTOMA

Sympathetic baby tumor

Neuroblastomas are malignant tumors of neuroblasts, which are primitive sympathetic ganglion cells that descend from neural crest cells and have the capacity to secrete catecholamines.  It is the third most common childhood cancer, accounting for 8%. The disease process begins in the embryo, but the diagnosis usually isn't made until the child is a few years old. The clinical picture of neuroblastomas varies widely, although they frequently cause sweating and opsoclonus-myoclonus syndrome (“dancing eyes, dancing feet”) due to catecholamine excess. The symptoms depend on where the tumor appears. They usually develop on the adrenal gland or sympathetic trunk. If it arises from the adrenal gland, then it may cause abdominal pain/mass or problems with urination. Importantly, neuroblastoma tumor masses can cross the midline of the abdomen. The tumors tend to calcify, which makes them appear as bright white flecks on XR and CT scans. Pathology shows Homer-Wright rosettes that stain positive for NSE and N-myc. 

Can you give an example of a typical presentation?

A child with fever, malaise, abdominal pain, and maybe with puzzling sympathomimetic symptoms such as flushing, sweating, and tachycardia. Sort of like a pheochromocytoma, but in an infant (typically normotensive though).