PROGNOSTIC-SIGNIFICANCE OF AGE, MYCN ONCOGENE AMPLIFICATION, TUMOR-CELL PLOIDY, AND HISTOLOGY IN 110 INFANTS WITH STAGE D(S) NEUROBLASTOMA - THE PEDIATRIC-ONCOLOGY-GROUP EXPERIENCE - A PEDIATRIC-ONCOLOGY-GROUPSTUDY
Hm. Katzenstein et al., PROGNOSTIC-SIGNIFICANCE OF AGE, MYCN ONCOGENE AMPLIFICATION, TUMOR-CELL PLOIDY, AND HISTOLOGY IN 110 INFANTS WITH STAGE D(S) NEUROBLASTOMA - THE PEDIATRIC-ONCOLOGY-GROUP EXPERIENCE - A PEDIATRIC-ONCOLOGY-GROUPSTUDY, Journal of clinical oncology, 16(6), 1998, pp. 2007-2017
Purpose: Although a high rate of spontaneous regression is observed in
infants with stage D(S) neuroblastoma (NB), survival is not uniform.
To determine the prognostic relevance of age at diagnosis, therapy, an
d tumor biology in infants with stage D(S) NE, we reviewed the Pediatr
ic Oncology Group (POC) experience. Patients and Methods: A review of
patients diagnosed with stage D(S) NE registered on FOG protocols was
performed. Survival according to age at diagnosis, treatment and tumor
biology was determined. Results: Between 1987 and 1996, 110 infants w
ith stage D(S) NE had an estimated 3-year survival rate of 85% +/- 4%;
survival rate was 71% +/- 8% for infants 2 months of age or younger a
nd 68% +/- 12%, 44% +/- 33%, and 33% +/- 19% for patients with diploid
, MYCN-amplified, and unfavorable histology tumors, respectively. Surv
ival rates were similar for patients who received adjuvant chemotherap
y versus those who did not (82% +/- 5% v 93% +/- 6%, respectively; P =
.187). Furthermore, there was no statistical difference in survival r
ate for patients who underwent complete resection of their primary tum
or compared with those who underwent partial resection or biopsy only
(90% +/- 5% v78% +/- 7%, respectively; P = .083). Conclusion: Our revi
ew confirmed that the survival of infants with stage D(S) NE is excell
ent. However, subsets of patients with poor prognosis can be identifie
d by young age and unfavorable biologic factors. More effective therap
y is needed for the group of stage D(S) infants who show unfavorable c
linical and biologic features.