ABSTRACT
Under these circumstances, low-dose radiotherapy localized to part of the liver (avoiding the spine, kidneys and ovaries [in girls]) and/or chemotherapy adjusted for age, with agents such as vincristine, cyclophosphamide, etoposide, or carboplatin is indicated. A delayed response is not unusual and the insertion of a large Silastic patch60 has been effective in relieving compression. However, this leaves the already compromised patient more open to infection and spontaneous liver regression may take weeks to occur. This form of treatment is not always successful.59,61
It is unlikely that resection of the primary tumor during the ‘florid’ stage of the disease is of therapeutic value. It may however be advisable, although this is controversial, to remove the primary tumor when the remote tumor has regressed because it is known that it may subsequently become active again.57 In most instances this will require an adrenalectomy, although occasionally in 4S disease the primary is elsewhere and indeed, in some, no primary is identified. Bourhis et al. showed, in a small series of neuroblastoma stage 4S patients, that N-myc amplification and/or diploidy had a fatal outcome and these biological parameters may identify patients for whom a more aggressive therapy is required.62
