Neuroblastoma is the third most common neoplasm in children. 

Virchow described Neuroblastoma in 1863. 

Zuckerkandl and Kohn described sympathetic origin of Neuroblastoma. 

Neuroblastoma could mature into ganglioneuroma. 

Neuroblastoma can be seen prenatally and differential consideration include adrenal hemorrhage.

 Locations:

• Adrenal medulla- 35%
• Extra adrenal retroperitoneum- 30-35%
• Posterior mediastinum- 20%
• Neck -1-5%
• Pelvis -2 -3 %
• 1% of metastatic disease no identifiable primary.        

It is one of very few cancers to secrete hormones, producing HMA and VMA. 

 Neuroblastoma Associated Abnormalities  –  Neuroendocrine cristopathy:

• Von Recklinghausen disease
• Beckwith Wiedemann Syndrome
• Hirschsprung disease
• Ondine curse syndrome
• DiGeorge syndrome 

Symptoms include mass, fatigue, bone pain, fever, proptosis, dark circles under the eyes most commonly. Less often, patients present with elevated blood pressure, diarrhea or rapid heart beat.  

 Presentations:

Thoracic neuorblastoma-  Horner syndrome(ptosis, pupillary constriction, ipsilateral facial anhidriosis and flushing).

 Hutchinson syndrome- mets to bone with limp and irritability. 

Watery diarrhea and hypokalemia from VIP (vaso active intestinal peptide)   

 Pepper syndrome-Diffuse liver mets

 Blueberry Muffin syndrome- skin mets

 Opsiclonus and myoclonus-2% of patients with abnormal eye movements and jerky limbs. Skull base mets- Raccoon eyes  

Workup includes ultrasound, MR, CT and nuclear imaging with bone scans and MIBG imaging.  

Treatment includes surgery, radiation therapy, chemotherapy, and bone marrow transplantation. 

Survival is about 85% in infancy, 55% ages 1-5 and 40% over age 5 depending upon staging and histology.  

Clue in this case is the extension of tumor into the retroperitoneal lymph nodes with elevation of the aorta off the spine. (Lymphoma can do the same)

Other neoplasms of the kidney to be considered in this age child with retroperitoneal adenopathy are clear cell or anaplastic sarcoma and rhabdoid tumor. 

Features associated with better prognosis:

•  Age at diagnosis< 1 year
•  Histological grade (Shimada system)  
•  Decreased n-myc amplification
•  Anatomic stagingStage 4S-Nearly 100% survival
•  Thoracic primary 

 Take Home Message:

Neuroblastoma tends to engulf and surround vessels.