In the past this stage was routinely treated with intensive chemotherapy, but it is now clear that the non n-myc amplified tumours behave in a similar fashion to Stage 2 disease and a more conservative approach may be applied. If possible, surgical clearance should be attempted but if this is not feasible then non-intensive chemotherapy, such as OPEC/OJEC, followed by delayed surgery is appropriate. In the face of incomplete resection, local radiotherapy is not indicated. Recently, a 'wait and watch' policy has been advocated as for stage 2 disease.
There remains controversy about the importance of attempting to achieve complete clearance at the primary site, as this is often difficult and necessitates several hours of complex surgery. The main problem is infiltration of the tumour around IVC and aorta, or infiltration of the adjacent liver.
MIBG positivity, post-operatively, indicates likely residual primary tumour, and local radiotherapy is indicated. An alternative is the use of I131 MIBG-targeted radiotherapy. High-dose melphalan with peripheral blood stem rescue is standard in some protocols but this approach remains to be clearly proven. One randomized study indicates a significant prolongation of progression-free survival. Alternative high-dose intensity strategies, such as administering treatment every 10 days or escalating the dose of cyclophosphamide, are currently under evaluation.
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