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Results of therapy in stage IV-S neuroblastoma with massive hepatomegaly
Insights
Symptomatic hepatomegaly is common in Stage IV-S neuroblastoma. Low-dose radiation therapy, without chemotherapy, showed prompt responses in symptomatic patients, supporting its consideration for managing this condition.
Area of Science:
- Pediatric Oncology
- Hepatology
- Radiation Oncology
Background:
- Stage IV-S neuroblastoma frequently presents with massive hepatomegaly.
- Hepatomegaly can cause significant symptoms in affected children.
Purpose of the Study:
- To determine the incidence and severity of symptomatic hepatomegaly in Stage IV-S neuroblastoma.
- To evaluate the outcomes of low-dose radiation therapy for symptomatic hepatomegaly.
Main Methods:
- Retrospective chart review of pediatric neuroblastoma patients (1951-1985).
- Inclusion criteria: Stage IV-S neuroblastoma with symptomatic hepatomegaly.
- Analysis of treatment regimens and patient outcomes.
Main Results:
- 69% of Stage IV-S patients had massive hepatomegaly; 5 experienced symptoms.
- Three symptomatic patients receiving low-dose radiation (≤600 rad) without chemotherapy showed prompt subjective improvement.
- Long-term follow-up showed 80% survival with resolved/resolving hepatomegaly.
- High-dose radiation was associated with severe adverse effects, including chondromas and organ hypoplasia.
Conclusions:
- Low-dose radiation therapy is a viable option for symptomatic hepatomegaly in Stage IV-S neuroblastoma.
- Careful consideration of radiation dose is crucial to minimize long-term side effects.
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 less than or equal to 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 (greater than or equal to 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.