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Neuroblastoma Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Treatment of Intermediate-Risk Neuroblastoma

The Children's Oncology Group (COG) intermediate-risk group assignment criteria are described in Table 7.

Table 7. Children's Oncology Group (COG) Neuroblastoma Intermediate-Risk Group Assignment Schema Used for the COG-A3961 Studya

INSS StageAgeMYCNStatusINPC ClassificationDNA Ploidyb
INPC = International Neuroblastoma Pathologic Classification; INSS = International Neuroblastoma Staging System.
a The COG-P9641 (low risk) and COG-A3961 (intermediate risk) trials established the current standard of care for non-high-risk neuroblastoma patients in terms of risk group assignment and treatment strategies.
b DNA Ploidy: DNA Index (DI) > 1 is favorable, DI = 1 is unfavorable; hypodiploid tumors (with DI < 1) will be treated as a tumor with a DI > 1 (DI < 1 [hypodiploid] to be considered favorable ploidy).
c INSS stage 3 or stage 4 patients with clinical symptoms as listed above receive immediate chemotherapy.
d INSS stage 4S infants with favorable biology and clinical symptoms are treated with immediate chemotherapy until asymptomatic (2-4 cycles). Clinical symptoms include the following: 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.
3c<365 dNonamplifiedAnyAny
≥365 d-21 yNonamplifiedFavorable-
4c<548 d[1,2,3]NonamplifiedAnyAny
4Sd<365 dNonamplifiedAny=1
<365 dNonamplifiedUnfavorableAny

(Refer to the Treatment of Stage 4S Neuroblastoma section of this summary for more information about the treatment of stage 4S neuroblastoma.)

Treatment Options for Intermediate-Risk Neuroblastoma

Treatment options for intermediate-risk neuroblastoma include the following:

  1. Chemotherapy with or without surgery.
  2. Surgery and observation (in infants).
  3. Radiation therapy (only for emergent therapy).

Chemotherapy with or without surgery

Patients categorized as intermediate risk have been successfully treated with surgery and four to eight cycles of chemotherapy (carboplatin, cyclophosphamide, doxorubicin, and etoposide; the cumulative dose of each agent is kept low to minimize permanent injury from the chemotherapy regimen) (COG-A3961). As a rule, patients whose tumors had unfavorable biology received eight cycles of chemotherapy, compared with four cycles for patients whose tumors had favorable biology. The COG-A3961 phase III trial demonstrated that therapy could be significantly reduced for patients with intermediate-risk neuroblastoma while maintaining outstanding survival.[4]

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