Abstract

Ewing sarcoma (EWS) is a paediatric aggressive malignant tumour of bones and soft tissues. Multidisciplinary chemotherapies, surgical resection, and radiation represent the only strategies counteracting the disease, however spreading and relapse of disease still remain a clinical issue. Circulating tumour cells (CTCs) are an important feature of EWS but the prognostic significance has not been, yet, clarified. CTCs have been found both in patients with localized disease and in those who recur or metastasize. The identification of markers that can detect recurrences and metastasis remains an important challenge for research. Unfortunately, even most of patients with localized cancer relapsed and the reason has not yet been fully understood. In this clinical study on EWS patients, we evaluated the expression of CD99 antigen and beta-3 adrenergic receptor (β3-AR) on CTCs and bioptic derived cells by flow cytometry. The preliminary data revealed a higher β3-AR expression on cells derived from metastatic or relapsed patients, suggesting a role for the β3-AR as a possible predictive maker of disease recurrence in both patients with metastatic and localized disease.

Highlights

  • Ewing sarcoma (EWS) is a highly aggressive and metabolically active malignant tumour of soft tissue and bones, that primarily occurs in adolescents and young adults [1,2]

  • The results show the expression of β3-AR on the surface of CD45− CD99+ peripheral blood (PB) cells, confirming the presence of β3-AR on tumour cells derived from Ewing sarcoma patients

  • In order to gain insight on the significance of analysed markers, biological samples were divided into two different group: Metastatic those derived from patients affected by metastatic disease at onset and non-metastatic those derived from patients with localized disease at diagnosis

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Summary

Introduction

Ewing sarcoma (EWS) is a highly aggressive and metabolically active malignant tumour of soft tissue and bones, that primarily occurs in adolescents and young adults [1,2]. EWS is the second most common paediatric malignant bone tumour that develops in osseous sites and in extra-skeletal soft tissues and represents 2% of all childhood cancers [4,5]. The estimated five-year survival in cases of localized disease, i.e., non-metastatic cases, is between 50% and 70% [6,7]. Even after undergoing intensive multi-modality treatment (that include allogeneic haematopoietic stem cell transplantation) the prognosis for patients with metastatic or recurrent disease remains unfavourable [5,9]. Current researches have focused on identifying and targeting specific pathways and genes that would have a crucial role in the development of therapeutic resistance

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