​Pathology - Neuroblastoma

​Pathology - Neuroblastoma 
Neoplasm originating from neural precursor cells; linked to N-myc oncogene amplification and deletions in the short arm of chromosome 1 (1p-).
Most frequently observed in young children, although does occasionally happen in adults.

Pathology: Typically originates in the adrenal medulla but can also develop in the sympathetic chain, pelvis, neck, or brain. It varies in size, may have well-defined borders, and can exhibit cyst formation or necrosis.

Microscopic examination reveals sheets of tiny cells with dark nuclei and little cytoplasm, typically organized in Homer-Wright pseudorosettes. These cells contain neurosecretory granules that store catecholamines.

The typical scenario involves a child under 2 years old presenting with a significant abdominal mass, high blood pressure, and weight loss. Additional symptoms consist of ecchymosis and proptosis (eye protrusion).
Older children might show signs of cancer spreading to the bone, liver, or lungs, which can cause symptoms including bone pain, respiratory issues, or gastrointestinal problems.
Laboratory results show elevated 24-hour urinary VMA and metanephrine levels, as well as higher plasma and urinary catecholamine levels.

Therapy 
Combination of surgical removal and chemotherapy.
The prognosis varies depending on the patient's age and the stage of cancer at diagnosis. Younger patients and those with lower cancer stages tend to have a more favorable prognosis.