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

Journal of the Korean Pediatric Society 1998;41(5):684-694.
Published online May 15, 1998.
A Clinical Study of Survival Rate and Prognostic Factors in Advanced Neurobalstoma in Children
Seung Yang, Ho Joon Im, Nam Su Kim, Hahng Lee
Department of Pediatrics, College of Medicine, Hanyang University, Seoul, Korea
진행된 소아 신경모세포종의 치료성적 및 예후인자에 관한 연구
양승, 임호준, 김남수, 이항
한양대학교 의과대학 소아과학교실
Correspondence: 
Seung Yang, Email: 1
Abstract
Purpose
: Despite the use of improved therapeutic modalities, long-term survival remains poor in patients in stage Ⅲ or Ⅳ 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 Ⅲ or Ⅳ 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 Ⅲ, 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 Ⅲ and 20% for stage Ⅳ. The age <2 years, mediastinal primary, low serum NSE, and low serum ferritin were associated with better outcome. In stage Ⅲ, progression of disease was observed in only two, both with unfavorable prognostic factors. In stage Ⅳ, 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 Ⅲ including certain unfavorable prognostic factors and for stage Ⅳ, which might need more effective innovative therapies, such as autologous bone marrow transplantation or peripheral blood stem cell transplantation.
Key Words: Neuroblastoma, Survival, Prognostic factors


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