Abstract
Special NBs (with opsomyoclonus or OMC; IV-S) have an excellent prognosis (85% 2 year survival) vs. conventional NBs (29% 2 y.s.) in our patient pool; this was inexplicable by standard prognostic factors alone. We studied tumor biopsies prior to therapy from 46 special (12 Stage II-OMC, 34 IV-S) and 48 conventional (12 Stage II, 36 Stage III or IV) NBs to detect discerning tumor-associated antigens via immunoperoxidase technique. A unique and variable pattern of tumor stromal differentiation was identified in all NBs, consisting of the relative occurrence of S100 protein-positive (Schwann-type) versus ferritin (FER) positive satellite cells around tumor vessels. In any NB, the presence of any S100 cells vs. only FER cells significantly improved survival (S100-94% 2 y.s.; FER-29% 2 y.s.) (p<0.001). With decrease in FER in NBs, a significant (p=0.03) curvilinear increase in tumor stromal lymphoid aggregates occurred. Special NBs contained twice as many S100 cells (p<0.002), one-half as many FER cells (p<0.001) and 4 times as many lymphoid aggregates (p<0.005) as conventional NBs. Stromal perivascular satellite cell differentiation (Schwann-type or S100 vs. FER) is a new and significant prognostic factor in both special and all NBs; a favorable differentiation pattern (↑S100/↓FER cells) may be linked to enhanced immune response.
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Qualman, S., Auyama, C., Shimada, H. et al. CONVENTIONAL VERSUS SPECIAL NEUROBLASTOMA (NB): FAVORABLE PROGNOSIS CORRELATES WITH STROMAL DIFFERENTIATION. Pediatr Res 21 (Suppl 4), 305 (1987). https://doi.org/10.1203/00006450-198704010-00825
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DOI: https://doi.org/10.1203/00006450-198704010-00825