Evaluation of Disease Response

According to the INSS, disease response should evaluated CT/MRI of the primary and metastatic sites, MR of epidural or CNS involvement, bone scan, and MIBG scan; however, it must be remembered that occasionally ganglioneuromas are detected by MIBG scintigraphy. Prior to second-look surgery, a repeat CT scan of the primary site is important for surgical planning. In addition to the primary tumor, adjacent lymph nodes in the thorax, retroperitoneum, and pelvis should be evaluated. Small lymph nodes are better identified on CT than MR, and may be too small to characterize on MIBG scanning.

For patients with intermediate- and high-risk disease, CT/MRI examinations are typically performed in conjunction with MIBG scans at 3- to 6-month intervals during treatment and for 1-2 years following the completion of therapy. Routine scanning after this time should be continued only for those patients with persistent abnormalities. Routine follow-up bone scans do not yield much extra information, unless progression is suspected and or local radiation is planned. Equivocal findings can be further evaluated with FDG-PET scans (Fig. 10.2).

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