The Adolescent and Young Adult with Cancer: State of the Art - Bone Tumors

Abstract

Primary malignant bone tumors in the pediatric to young adult populations are relatively uncommon and account for about 6 % of all cancers in those less than 20 years old [1] and 3 % of all cancers in adolescents and young adults (AYA) within the age range of 15 to 29 years [2]. Osteosarcoma (OS) and Ewing's sarcoma (ES) comprise the majority of malignant bone tumors. The approach to treatment for both tumors consists of local control measures (surgery or radiation) as well as systemic therapy with high-dose chemotherapy. Despite earlier advances, there have been no substantial improvements in outcomes over the past several decades, particularly for patients with metastatic disease. This review summarizes the major advances in the treatment of OS and ES and the standard therapies available today, current active clinical trials, and areas of investigation into molecularly targeted therapies.

Sox2 antagonizes the Hippo pathway to maintain stemness in cancer cells

“…We have shown that this stem cell transcription factor maintains CSCs in osteosarcomas 12 , the most common bone malignancy in childhood and adolescence 13 . Molecular genetic analysis of sporadic and hereditary osteosarcomas in humans demonstrated that inactivation of the tumor suppressors Rb and p53 plays a role in their development 14,15 .…”

Serum microRNA-17 functions as a prognostic biomarker in osteosarcoma

“…Further advances in therapeutic strategies for OS, including wide tumor excision, neoadjuvant and adjuvant chemotherapy, and radiotherapy, have resulted in significantly improved clinical outcomes and long-term survival rates (2,6–9). However, >30% of patients with OS survive for <5 years and succumb due to pulmonary metastases following diagnosis (10,11). Therefore, it is critical to identify OS effector molecules, novel therapeutic strategies, and signaling pathways regulating OS growth and metastasis.…”

How PET/MR Can Add Value for Children with Cancer

“…Overestimating tumor size can lead to unnecessary surgical resections of too much normal tissue, affect the approach for limb-sparing surgery and lead to unsatisfactory long-term outcomes [70, 73]. Conversely, incomplete tumor resection can impact prognosis and post-surgical care [74–76]: In Ewing sarcomas, a wide surgical margin (R0) with normal tissue around the lesion does not require additional local control while a marginal excision (R1), which includes tumor cells at the cut surface, must be treated by radiotherapy and/or intensified chemotherapy [77]. Recent evidence shows that 18 F-FDG-PET can help differentiating tumor tissue and peri-lesional edema: The primary tumor shows marked 18 F-FDG uptake while peri-lesional edema shows little or no 18 F-FDG uptake [78] (Fig.…”