Treatment of Stage 4S Neuroblastoma Particularly in Patients With Prenatally Diagnosed Tumor

Abstract

Purpose: To discuss particularly the treatment of prenatally diagnosed Stage 4S neuroblastoma (4SNB), we retrospectively reviewed the treatments and outcomes of patients with 4SNB.

Methods: We have treated 9 patients with 4SNB over the last 33 years. Their clinical characteristics, treatment and outcome, overall survival and prognostic factors were reviewed.

Result: There were 2 male and 7 female patients, 0 day to 9 months (median, 1 month) of age at diagnosis. NB was detected by mass screening in 4 patients and prenatally in 2, and these patients were asymptomatic at diagnosis. The other 3 patients were diagnosed with clinical symptoms.

The primary lesion site was the adrenal glands in all the patients, and the metastatic sites were the liver in 8, skin in 2, and bone marrow in 1. Histopathological findings indicated a favorable prognosis in 8 patients; the DNA ploidy was diploid in 3 and hyperdiploid in 5. None of the patients showed MYCN amplification, and the risk was classified as low in 5 and intermediate in 3. Tumor extirpation and chemotherapy were performed in patients with mass-screening-detected NB, and surgery and chemotherapy were consequently inevitable in prenatally diagnosed patients. The patients who were asymptomatic at diagnosis were all alive, whereas 2 of the 3 patients who were symptomatic at diagnosis died of the disease despite aggressive treatments, and their overall survival was 33%.

Conclusion: Prompt and aggressive treatments are necessary for 4SNB patients who are symptomatic at diagnosis. Since prenatally diagnosed NBs, not those detected by mass screening, may progress, follow-up with close observation is extremely important, and the indication of treatments should be considered if required.