1 research outputs found
Tumor expression of survivin, p53, cyclin D1, osteopontin and fibronectin in predicting the response to neo-adjuvant chemotherapy in children with advanced malignant peripheral nerve sheath tumor
Purpose Selected cell-cycle regulators and extracellular matrix proteins were found to play roles in malignant peripheral
nerve sheath tumor (MPNST) biology. We aimed to analyze whether initial tumor tissue expressions of survivin, p53, cyclin
D1, osteopontin (OPN) and fibronectin (FN) correlate with the response to neo-adjuvant CHT (naCHT) in children with
advanced inoperable MPNST.
Methods The study included 26 children with MPNST (M/F 14/12, median age 130 months) treated in Polish centers of
pediatric oncology between 1992 and 2013. Tissue expression of markers was studied immunohistochemically in the manually
performed tissue microarrays and assessed semi-quantitatively as low and high, based on the rate of positive cells and
staining intensity.
Results Good response to naCHT was noted in 47.6%, while poor-in 52.4% of patients. The response to naCHT was
influenced negatively by the presence of neurofibromatosis NF1 and high initial tumor tissue expression of OPN, survivin,
p53 and cyclin D1. Patients with high tumor expression of either OPN, survivin or p53 and those with simultaneous high
expression of ≥ 3 of the markers, responded significantly worse to naCHT, than patients, in whom expression of ≤ 2 markers
were detected at diagnosis. Nearly, 85% of patients expressing ≥ 3 markers, responded poor to CHT; while 87.5% of children,
expressing ≤ 2 markers, were good responders.
Conclusion The initial tumor tissue expression of OPN, survivin, p53 and cyclin D1 may serve as markers to predict response
to naCHT in pediatric advanced MPNST. Future studies in more numerous group of patients are needed to confirm these
preliminary results