Treatment

The focus of this chapter is on the subset of patients with neuroblastoma who can do well without cyto-toxic therapy and lack clinical characteristics that connote a poor prognosis, namely, highly locally invasive unresectable primary tumor, cortical bone metastases, and extensive bone marrow involvement. Osteomedullary involvement is an objective finding, but resectability is partly dependent on subjective, nonquantifiable factors. An aversion to perform major surgery might be strengthened by imaging studies showing extensive disease. Resectability, however, can only be definitively assessed during surgery. Low-risk tumors are frequently characterized by a firm consistency that makes resection a fea sible option, even if the tumor extends across the midline. By contrast, primary tumors in patients with high-risk disease are often impossible to mobilize and resect due to hemorrhagic friability and adhesiveness to neighboring tissues. These disparities in tumor consistency likely reflect biological differences. Although low-risk neuroblastomas might potentially be resectable in their entirety, complete resections sometimes entail the risk of significant morbidity such as brachial plexopathy with a cervical tumor; thus, a treatment plan that includes chemotherapy regardless of the extent of tumor resection may make it unjustifiable to undertake a difficult procedure needed to achieve a gross total excision. Alternatively, a partial resection followed by chemotherapy or observation alone may be reasonable, given the very small risk that residual biologically favorable tumor might evolve into lethal metastatic disease.

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