The N6 approach reliably achieves significant cytoreduction against stage 4 neuroblastoma. This may eventuate in an improved cure rate, since consolidative treatments using myeloablative therapy, immunotherapy, or biologic response modifiers such as cis-retinoic acid are most likely to be effective against minimal residual disease.

Major toxicities were grade 4 myelosuppression and mucositis during chemotherapy, and self-limited pain and urticaria during antibody treatment. Late effects include hearing deficits and hypothyroidism.

Our results suggest that non-stage 4 patients without N-myc amplification can be spared cytotoxic therapy because (1) residual postsurgical or recurrent biologically favorable neuroblastoma rarely evolves into lethal stage 4 disease; and (2) neuroblastoma in lymph nodes has no prognostic significance. These findings are remarkable because no other cancer includes subtypes that are curable without therapy to ablate residual disease.

To determine the relative accuracy of the various radiologic signs of Hirschsprung disease (HD), we retrospectively reviewed both radiographs obtained after a barium enema and the medical records of 62 children who had surgery to prove or exclude the diagnosis of HD. The visualization of a rectosigmoid transition zone was highly predictive of HD, but nonvisualization did not rule out HD. A false positive transition zone at the splenic flexure was seen in four neonates who had small left colon syndrome rather than HD. Retention of barium seen on radiographs obtained 24 hours after a barium enema was not a specific sign, but it was the only sign of HD in seven neonates, including two who had total colonic aganglionosis. Anal manipulation prior to the barium enema examination did not affect the diagnostic value of that procedure. We conclude that the single most reliable radiographic sign of HD is the presence of a rectosigmoid transition zone. Statistically, the use of three radiographic features combined (rectosigmoid transition zone, retention of barium, and stool mixed with barium) correlated better with the presence or absence of HD than did any of these features alone. A comparison of 24 and 48 hour postevacuation radiographs may help to differentiate HD from meconium plug syndrome.

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