Results of therapy in Stage IV-S neuroblastoma with massive hepatomegaly

Abstract

To document the incidence and severity of symptomatic hepatomegaly in patients with Stage IV-S neuroblastoma, we reviewed the charts of children with neuroblastoma seen at Children's Hospital of Pittsburgh between 1951 and 1985. Sixteen met the criteria for IV-S disease, and 11 of these (69%) had massive hepatomegaly. Five children had symptoms referable to their liver size including respiratory distress, gastroesophageal reflux, or decreased urine output. Liver function appeared to be normal or only mildly abnormal in the 9 patients where data were available. Because therapy was so variable, it was not possible to correlate treatment regimen with outcome. However, 3 symptomatic patients who received ≤600 rad without chemotherapy had prompt subjective responses. Follow-up was available on 10 children 6 months-18 years (median 18 years) from diagnosis. Eight were alive with resolved or resolving hepatomegaly. High dose (≥3,300 rad) radiation-related side effects included multiple rib chondromas, chest- and pelvic-wall hypoplasia in one patient, and radiation nephritis with hepatic fibrosis resulting in death of a second patient. Our results support prior recommendations that for symptomatic hepatomegaly, low doses of radiation be considered.