Purpose: A retrospective study was conducted to investigate the relati
onship between CD44 expression in neuroblastoma and related tumors and
other known prognostic indicators. Materials and Methods: Immunostain
ing of CD44 was done on surgical specimens of 55 cases (42 patients) o
f neuroblastoma (NB) and ganglioneuroblastoma (GNB) and nine cases of
ganglioneuroma. The percentage of positive tumor cells was scored semi
quantitatively (0-4+) by two observers. CD44 expression was then corre
lated with survival, age, stage, and N-myc amplification. Results: Fif
ty-seven percent of the patients with NE or GNB had heterogeneous posi
tive staining (2-1+) on their diagnostic specimens. Twenty-four percen
t of the patients had no staining for CD44, and 19% had 1+ staining. I
n the 17 cases with N-myc analysis, an inverse relationship was demons
trated between N-myc and CD44 expression by univariate analysis. Lack
of expression of CD44 was highly associated with poor survival (p = 0.
0002). When assessing the joint effects of age, stage, and CD44 in mul
tivariate analysis, the effect of CD44 remains significant (p = 0.028)
and appears to be independent of age and stage. Conclusion: Our data
suggest a relationship between CD44 and N-myc amplification. Absence o
r low expression of CD44 correlates with poor survival and may be a bi
ologic marker of tumor aggressiveness. CD44 appears to be an independe
nt prognostic marker and deserves continued investigation in prospecti
ve studies of neuroblastoma.