Abstract
Insulin-like growth factor-binding protein 2 (IGFBP2) is a tumor-associated protein measurable in patients’ biopsies and blood samples. Increased IGFBP2 expression correlates with tumor severity in rhabdomyosarcoma (RMS). Thus, we examined the plasmatic IGFBP2 levels in 114 RMS patients and 15 healthy controls by ELISA assay in order to evaluate its value as a plasma biomarker for RMS. Additionally, we looked for the presence of a humoral response against IGBFP2 protein measurable by the production of anti-IGFBP2 autoantibodies. We demonstrated that both circulating IGFBP2 protein and autoantibodies were significantly higher in RMS patients with respect to controls and their combination showed a better discriminative capacity. IGFBP2 protein identified metastatic patients with worse event-free survival, whereas both IGFBP2 and anti-IGFBP2 antibodies negatively correlated with overall survival. Our study suggests that IGFBP2 and anti-IGFBP2 antibodies are useful for diagnostic and prognostic purposes, mainly as independent negative prognostic markers in metastatic patients. This is the first study that reports a specific humoral response in RMS plasma samples and proves the value of blood-based biomarkers in improving risk assessment and outcome of metastatic RMS patients.
Highlights
Rhabdomyosarcoma (RMS) is an aggressive pediatric tumor arising from soft tissues and sharing aspects with skeletal muscle lineage. a rare disease, RMS comprises about three percent of childhood cancers and is the most common soft tissue sarcoma in children, with an overall incidence rate of approximately4.5 patients per million individuals aged less than 20 years [1]
The 5-years event-free survival (EFS) and overall survival (OS) of the whole cohort of patients was 46% and 58.8 %, respectively
In childhood RMS, treatment and prognosis vary widely according to a series of clinical characteristics such as histology and fusion status, having fusion-positive RMS patients a worse outcome compared to fusion-negative ones [24,25,26]
Summary
Rhabdomyosarcoma (RMS) is an aggressive pediatric tumor arising from soft tissues and sharing aspects with skeletal muscle lineage. a rare disease, RMS comprises about three percent of childhood cancers and is the most common soft tissue sarcoma in children, with an overall incidence rate of approximately4.5 patients per million individuals aged less than 20 years [1]. Rhabdomyosarcoma (RMS) is an aggressive pediatric tumor arising from soft tissues and sharing aspects with skeletal muscle lineage. RMS comprises about three percent of childhood cancers and is the most common soft tissue sarcoma in children, with an overall incidence rate of approximately. ERMS occurs in children under 10 years age, with a bimodal age distribution with the largest peak between 0–5 years. It typically arises in favorable sites such as head and neck, the genitourinary tract and the retroperitoneum. ERMS has a relatively favorable prognosis, with 5-year survival rate of 70–80%, while
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