Understanding Your Diagnosis
The treatment of your child’s Neuroblastoma will be chosen initially based on how he or she falls into a particular “risk group”.  In order to identify which risk group a patient is in, the diagnosis includes identifying what “stage” the disease is in, and what other factors exist, especially age and Histology, or biological and genetic makeup of the tumor tissue. There are currently three risk group categories that are used; Low risk, Intermediate risk and High risk.  Your child’s treatment selection will usually be based on which risk group he or she is identified with. Staging and Risk Grouping (Children's Oncology Group Neuroblastoma Risk Group Assignment schema) Age   Risk Group  1 0-21y Any Any Any Low 2A/2B* <365d Any Any Any Low   ≥365d-21y NonAmp Any - Low   ≥365d-21y Amp Fav - Low   ≥365d-21y Amp Unfav - High 3*** <365d NonAmp Any Any Intermediate   <365d Amp Any Any High   ≥365d-21y NonAmp Fav - Intermediate   ≥365d-21y NonAmp Unfav - High   ≥365d-21y Amp Any - High 4*** <548d [13-15] NonAmp Any Any Intermediate   <548d Amp Any Any High   ≥548d-21y Any Any - High 4S** <365d NonAmp Fav >1 Low   <365d NonAmp Any =1 Intermediate   <365d NonAmp Unfav Any Intermediate   <365d Amp Any Any High             Biology Defined By: MYCN Status: Amplified (Amp) versus NonAmplified (NonAmp) Shimada Classification: Favorable (Fav) versus Unfavorable (Unfav) DNA Ploidy: DNA Index (DI) >1 is favorable, = 1 is unfavorable; hypodiploid tumors (with DI <1) will be treated as a tumor with a DI >1 (DNA index <1 [hypodiploid] to be considered favorable ploidy).
* INSS 2A/2B symptomatic patients with spinal cord compression, neurologic deficits, or other symptoms are treated on the LOW RISK NB Study with immediate chemotherapy for 4 cycles (Course 1). ** INSS 4S infants with favorable biology and clinical symptoms are treated on the LOW RISK NB Study with immediate chemotherapy until asymptomatic (2 to 4 cycles). Clinical symptoms defined as: respiratory distress with or without hepatomegaly or cord compression and neurologic deficit or inferior vena cava compression and renal ischemia; or genitourinary obstruction; or gastrointestinal obstruction and vomiting; or coagulopathy with significant clinical hemorrhage unresponsive to replacement therapy. *** INSS 3 or 4 patients with clinical symptoms as listed above (or if in the investigator’s opinion it is in the best interest of the patient) will receive immediate chemotherapy.

Treatment can vary from child to child, and from treatment center to treatment center... you and your child’s health care team will determine which course to take. Every case is unique. Staging and risk grouping is not a “cookie cutter” approach to selecting treatment. Nevertheless it is a guideline within which treatment plans are developed

Low risk patients may have surgery alone or even watchful waiting for some infants.  Others may require some chemotherapy or radiation therapy.  3 year survival is generally greater that 90%.

Intermediate risk patients may have treatment that includes chemotherapy, surgery, and /or radiation therapy.  3 year survival is variable and generally 70-90%.

High risk Neuroblastoma treatment requires an aggressive approach. Treatment may include high dose chemotherapy, surgery, radiation therapy, and most often stem cell transplant. Treatment regimens usually include 13-cis retinoic acid.  Many children are then enrolled in a clinical trial where newer treatments are being investigated.  There  are many national and /or international clinical trials that are ongoing.  See NCI web site-http://www.cancer.gov/clinicaltrials. Three year survival is again very variable but generally about 30%.

Thankfully there is considerable research underway concerning the treatment of Neuroblastoma.  As some of this research advances the above chart may be adjusted accordingly, better defining risk group assignment in the future and producing new lines of treatment.

When you are ready, there is more detailed treatment information available. You can refer to the NCI PDQ link Please note that there are three versions of the Neuroblastoma PDQ available online.  One version is for patients, another is for healthcare professionals, and is more detailed, and the third is available in Spanish. The information here seems to be updated regularly, and the date that it was last modified is noted at the top of the page. There is also information about Neuroblastoma that may be progressive through treatment or recurrent, (comes back).

Each case is as unique as each child. Treatment plans often vary.  Treatment selection can be as much an art as a science.  Please discuss your particular case with your oncology team.  It can all be very confusing and totally overwhelming, especially at diagnosis.  Be informed. Don’t be afraid to ask questions, or seek other opinions.  There are not always clear answers and often parents are expected to make critical decisions. These can best be made by educated parents working in conjunction with a specialized team of physicians.  Most specializing hospital websites also now have some general information about Neuroblastoma and information specific to your hospital.
(Please see the section under Curesearch, where you can search for a COG (Childrens Oncology Group) Hospital by state.