A Clinical Study of Survival Rate and Prognostic Factors in Advanced Neurobalstoma in Children

Yang, Seung; Im, Ho Joon; Kim, Nam Su; Lee, Hahng

Abstract

PURPOSE: Despite the use of improved therapeutic modalities, long-term survival remains poor in patients in stage lll or lV of neurobalstoma and for over 1 year olds at diagnosis. We evaluated the long-term survival rate and related prognostic factors in advanced neuroblastoma.
METHODS
Twenty-nine children diagnosed with stage lll or lV neuroblastoma after 1 year of age, between May 1987 and December 1996, were enrolled. Therapy included surgery, radiotherapy, and chemotherapy with cyclophosphamide, DTIC, and vincristine (CDV) for stage lll, and CDV or intensified regimen for stage IV.
RESULTS
Of the 25 patients, 18 male and 7 female, 23 (92%) were<5 years of age at diagnosis. The most commonly appearing initial presentation was abdominal mass, which was observed in 14 patients. Abdominal primary was found in 21 patients (84%) including 17 (68%) of adrenal origin and mediastinal primary in 4 (16%). The 5Y-EFS was 44% in all study patients with 80% for stage lll and 20% for stage lV. The age< years, mediastinal primary, low serum NSE, and low serum ferritin were associated with better outcome. In stage lll, progression of disease was observed in only two, both with unfavorable prognostic factors. In stage lV, no difference in long-term survival was noted between those treated with CDV regmen and those with intensified chemotherapeutic regimen.
CONCLUSIONS
Long-term event free survival (EFS) rate for stage III was 80% with poor outcome for stage lll including certain unfavorable prognostic factors and for stage lV, which might need more effective innovative therapies, such as autologous bone marrow transplantation or peripheral blood stem cell transplantation.