Survival from non-stage 4 neuroblastoma without cytotoxic therapy: An analysis of clinical and biological markers Conference Paper


Authors: Cheung, N. K. V.; Kushner, B. H.; LaQuaglia, M. P.; Kramer, K.; Ambros, P.; Ambros, I.; Ladanyi, M.; Eddy, J.; Bonilla, M. A.; Gerald, W.
Title: Survival from non-stage 4 neuroblastoma without cytotoxic therapy: An analysis of clinical and biological markers
Conference Title: Advances in Neuroblastoma Research - 1996 (ANR 6)
Abstract: The clinical characteristics of 43 patients (pts) and the biological features of their non-stage 4 neuroblastoma (11, 3, 15, 7 and 7 with stages 1, 2A, 2B, 3 and 4S, respectively) all managed initially without cytotoxic therapy at Memorial Sloan-Kettering Cancer Center are summarised. We staged patients by the International Neuroblastoma Staging System and measured their urine and serum tumour markers. Tumour MYCN copy number, chromosomal ploidy, chromosome 1p deletion, Shimada histopathology, trk-A and CD44 expression were analysed. Among patients with localised tumour (n = 36), 13 had residual disease after initial surgery, 19 had regional lymph node invasion and 6 had epidural involvement (2 of 6 being paraplegic). All 7 stage 4S patients had liver tumours, 3 had bone marrow involvement and 3 had lymph node involvement. The most common adverse biological markers were unfavourable histopathology (9140 evaluable tumours) and diploidy (7/39 tumours tested). At a median follow-up of 50+ months, 42 patients are alive and well (5 with evidence of disease), and I patient in remission died of encephalopathy. Progressive/recurrent disease occurred in 12 patients, 1 stage 2A, 2 stage 2B, 4 stage 3 and S stage 4S. Chemotherapy was eventually used in 4 patients: a 3-year-old stage 2B patient who developed stage 4; a 2-year-old whose recurrent tumour had poor-risk biological markers; a 1-year-old whose recurrent stage 3 disease infiltrated a vertebral body and a stage 4S infant with respiratory impairment from progressive hepatomegaly. Three of the treated patients had diploid tumours. We conclude that non-stage 4 is of itself a strong predictor of a favourable outcome. Diploidy, unfavourable histopathology and unresectable tumours were associated with disease progression. However, evolution of local-regional tumour into distant metastatic stage 4 disease is not typical of neuroblastoma.
Keywords: cancer survival; child; clinical article; human tissue; treatment outcome; child, preschool; histopathology; conference paper; cancer staging; follow-up studies; neoplasm staging; technetium 99m; antigen expression; reverse transcription polymerase chain reaction; tumor markers, biological; recurrence; childhood cancer; infant; neuroblastoma; disease progression; tyrosine kinase receptor; (3 iodobenzyl)guanidine i 131; hermes antigen; diploidy; mycn; cd44; ploidies; neurotrophin receptor; humans; prognosis; human; male; female; priority journal; trk-a; local-regional; progression histopathology
Journal Title European Journal of Cancer
Volume: 33
Issue: 12
Conference Dates: 1996 May 22-25
Conference Location: Philadelphia, PA
ISBN: 0959-8049
Publisher: Elsevier Inc.  
Date Published: 1997-10-01
Start Page: 2117
End Page: 2120
Language: English
DOI: 10.1016/s0959-8049(97)00281-5
PUBMED: 9516865
PROVIDER: scopus
DOI/URL:
Notes: Conference Paper -- Export Date: 17 March 2017 -- Source: Scopus
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MSK Authors
  1. Brian Kushner
    190 Kushner
  2. Nai-Kong Cheung
    439 Cheung
  3. Kim Kramer
    168 Kramer
  4. William L Gerald
    367 Gerald
  5. Marc Ladanyi
    861 Ladanyi