Survivin expression and its potential clinical significance in gastrointestinal stromal sarcoma.
ABSTRACT This study was designed to determine the level of survivin expression and its clinical significance as a prognostic factor in gastrointestinal stromal sarcoma (GIST). Twenty patients (12 males and 8 females) ranging in age from 25 to 72, with a median age of 53 were evaluated. Failure of TKI treatment was higher in the survivin-positive group (p=0.06). The rate of metastasis was significantly higher in the survivin positive group vs. the negative group (80% vs. 30%, p=0.18). The median overall survival (OS) time was 114 (range 29-199)months, and the median disease-free survival (DFS) time was 88 (range 40-135) months. The median progression-free survival (PFS) time was 40 (range 24-55) months. Further, a comparison of patients with survivin positive versus negative tumors, revealed no significant difference for OS, DFS, and PFS (p=0.45, p=0.19, p=0.55, respectively), number of mitoses in 50 HPF (p=0.14), and tumor size (p=0.94). In conclusion, survivin was highly expressed in GISTs, although we found no correlation between survivin expression and PFS, DFS and OS, survivin may be a predictive marker in GISTs for disease progression. We believe that additional studies are warranted to determine the clinical significance of survivin expression as a prognostic or predictive marker in patients with GIST.
- SourceAvailable from: Riccardo Turrini[show abstract] [hide abstract]
ABSTRACT: Malignant peripheral nerve sheath tumors (MPNST) are very aggressive malignancies comprising approximately 5-10% of all soft tissue sarcomas. In this study, we focused on pediatric MPNST arising in the first 2 decades of life, as they represent one the most frequent non-rhabdomyosarcomatous soft tissue sarcomas in children. In MPNST, several genetic alterations affect the chromosomal region 17q encompassing the BIRC5/SURVIVIN gene. As cancer-specific expression of survivin has been found to be an effective marker for cancer detection and outcome prediction, we analyzed survivin expression in 35 tumor samples derived from young patients affected by sporadic and neurofibromatosis type 1-associated MPNST. Survivin mRNA and protein expression were assessed by Real-Time PCR and immunohistochemical staining, respectively, while gene amplification was analyzed by FISH. Data were correlated with the clinicopathological characteristics of patients. Survivin mRNA was overexpressed in pediatric MPNST and associated to a copy number gain of BIRC5; furthermore, increased levels of transcripts correlated with a higher FNCLCC tumor grade (grade 1 and 2 vs. 3, p = 0.0067), and with a lower survival probability (Log-rank test, p = 0.0038). Overall, these data support the concept that survivin can be regarded as a useful prognostic marker for pediatric MPNST and a promising target for therapeutic interventions.PLoS ONE 01/2013; 8(11):e80456. · 3.73 Impact Factor