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Abbreviations: TBI, total body irradiation; ABMT, autologous bone marrow transplant. aAll studies used purged autologous bone marrow unless otherwise noted. bThree-year event-free survival rate from time of autologous marrow infusion.

The original studies were done with ABMT but currently patients receive peripheral stem cell transplant.

Abbreviations: TBI, total body irradiation; ABMT, autologous bone marrow transplant. aAll studies used purged autologous bone marrow unless otherwise noted. bThree-year event-free survival rate from time of autologous marrow infusion.

The original studies were done with ABMT but currently patients receive peripheral stem cell transplant.

Minimal Residual Disease Therapy

Despite the effective induction and myeloablative consolidation therapy, the majority of high-risk neuroblastoma patients experience relapse. The aim of this phase of therapy is to eradicate any residual tumor cells using radiation to tumor sites and noncytotoxic agents, which are theoretically active against chemoresistant tumor cells that survive intensive induction and consolidation regimens. It is preferable to give radiation post-transplantation if the tumor volume was large at the time of diagnosis or liver metastases were present.

Several novel agents such as cis-retinoic acid, anti-GD2 monoclonal antibody, or interleukin-2 are targeted to the biology of neuroblastoma and are found to be effective in eradicating minimal residual disease. Measurable responses have been observed in refractory neuroblastoma patients. Such protocols are not yet available for routine clinical practice. These protocols are available only in multicenter clinical trials on an experimental basis.

Risk Factors for Specific Relapse at a Specific Site

1. For local relapse in primary site:

a. Incomplete resection of the primary tumor. Aggressive complete surgical removal of the primary tumor is critically important in determining long-term prognosis.

2. For bone marrow relapse:

a. Bone marrow tumor content at the time of bone marrow harvest >0.1%.

b. Involvement of bone marrow at the time of initial diagnosis.

3. For bone relapse:

a. Involvement of bone at the time of initial diagnosis.

Follow-Up Studies

Physical examination should be performed monthly in the first year, every second month in the second year, and every third month in the third year following diagnosis.

Table 18-11 lists follow-up laboratory studies to determine disease status.

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