Low Risk Group

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The low-risk group consists of:

1. All patients with INSS stage 1

3. All ages INSS stage 2A, 2B, nonamplified MYCN, and favorable or unfavorable Shimada histology

4. All ages INSS stage 2A, 2B, or amplified MYCN and favorable Shimada histology

5. Infants with INSS stage 4S disease, favorable Shimada histology, nonamplified MYCN (single copy), and hyperdiploidy.

Treatment

1. All patients INSS stage 1:

a. Surgical removal of the primary tumor b. Disease-free survival (DSF): 90% for all age groups c. Indication for chemotherapy: recurrence d. Chemotherapy: cyclophosphamide (CPM)/Adriamycin (ADR) as follows: Cyclophosphamide: 150 mg/m2 PO, days 1-7

Adriamycin: 35 mg/m2 IV, day 8

e. Repeat every 21 days for a total of five cycles

2. All patients who are <12 months with INSS stage 2A or 2B:

In these cases incomplete surgical resection does not compromise the relapse-free survival in the setting of localized disease and favorable histologic features. This is a unique feature of neuroblastoma. Treatment: Surgery and chemotherapy. Chemotherapy consists of five cycles of low-dose sequential CPM/ADR (see above schema). Two-year disease-free survival: 85%. If a complete remission is not attained cisplatin/VM-26 chemotherapy should be used as follows: Cisplatin: 90 mg/m2 IV over 6 hours, day 1. VM-26 (teniposide): 100 mg/m2 IV over 1 hour, day 3. Repeat every 4 weeks for two cycles. Repeat imaging studies. If response occurs, then four more cycles should be administered.

3. All patients who are >12 months with stage 2A or 2B:

If the initial tumor is unresectable or if gross residual tumor is present, chemotherapy should be used as follows (alternating courses of OPEC and OJEC every 21 days):

Vincristine 1.5 mg/m2 IV bolus (max 2 mg) on day 1 Cisplatin 80 mg/m2 IV continuous infusion over 24 hours, day 1 Etoposide 200 mg/m2 IV over 4 hours, day 2.

b. OJEC therapy courses 2 and 4:

Vincristine 1.5 mg/m2 (max 2 mg) IV bolus, day 1 Cyclophosphamide 600 mg/m2 over 20 minutes, day 1 Etoposide 200 mg/m2 IV over 4 hours, day 1 Carboplatin 500 mg/m2 IV over 1 hour, day 1.

Second-look surgery should be done after course 5. Patients who are in complete remission following second-look surgery should receive one additional course each of OJEC and OPEC. Other patients should be treated with radiation therapy.

4. Infants with INSS stage 4S disease: Majority of patients with INSS stage 4S disease fall into the low-risk category with overall survival of 85%.

a. A diagnostic biopsy is indicated for evaluation of the biologic features of the tumor to plan treatment according to its correlation with risk category.

b. Tumors with favorable nonamplified MYCN usually regress spontaneously, especially in infants under 6 months of age, and should be observed closely without any treatment.

c. However, it has been suggested that sometime during the course of the disease, the primary tumor be resected because of a small chance of a late recurrence at the site of the primary tumor.

Infants under 2 months of age with 4S disease are at higher risk for developing hepatomegaly and multiorgan compromise resulting in a fatal outcome. Neonates who die from 4S neuroblastoma are those who have extensive hepatic involvement, which causes respiratory compromise, disseminated intravascular coagulopathy, and renal, inferior vena cava or gastrointestinal compromise.

If a massively enlarged liver compromises respiratory function, a short course of low-dose oral cyclophosphamide (5 mg/kg/day for 5 days every 2-3 weeks as needed) can induce remission. In addition, low-dose radiation (150 cGy two or three times to the anterior two thirds of the liver through lateral oblique ports avoiding radiation to the kidneys, ovary, and spine) may be delivered to the tumor. This dose is generally sufficient to halt the progression of the tumor and induce regression. The disadvantage of delivering neonatal radiation to the liver is the possibility of subsequent hepatic fibrosis or cirrhosis.

The rare stage 4S patients with unfavorable biologic features (diploidy, unfavorable histology, MYCN amplification) should be considered for either intermediate-risk (diploidy or unfavorable Shimada histology) or high-risk (MYCN amplification) treatment protocols.

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