Common Musculoskeletal Tumors of Childhood and Adolescence

Abstract

Osteosarcoma, Ewing sarcoma, and rhabdomyosarcoma are the most common malignant musculoskeletal tumors in children and adolescents. Today, most patients can be cured. Numerous factors have contributed to improved outcome for these patients over the past several decades. These include multidisciplinary care involving oncologists, radiation oncologists, surgeons, pathologists, and radiologists and enrollment of patients in clinical trials. Better understanding of molecular mechanisms of disease have resulted in studies using molecular targets in addition to standard chemotherapeutic agents, which hopefully will lead to better outcomes in the future. Moreover, new orthopedic techniques and devices as well as new technologies in radiation oncology hold promise for better local control of primary tumors and the potential for fewer late adverse effects. Despite this progress, patients must undergo lifelong follow-up for possible late effects of intense chemotherapy and radiation therapy. We review the diagnosis, prognosis, staging, multidisciplinary therapy, new directions in therapy, and long-term complications of treatment for these tumors. For this review, we searched MEDLINE using the terms rhabdomyosarcoma, osteosarcoma, Ewing sarcoma, biology, and humans and limited the search to articles from 2000 to September 2011. Additional references found in these articles were utilized as appropriate, as well as references from the background information in current therapeutic studies of the Children's Oncology Group. The same database and time frame were searched for articles written by leading authorities in the field.

Osteosarcoma: improvement in survival limited to high-grade patients only

“…Tumor response to preoperative chemotherapy has previously been shown to represent the most important prognostic factor (Arndt and Crist 1999;Davis et al 1994). Other possible prognostic impact factors include older (Saeter et al 1997) or younger age (French Bone Tumor Study Group 1988), patient gender (Saeter et al 1997), tumor size (Bieling et al 1996) and proximal location (Goorin et al 1987;Provisor et al 1997), high alkaline phosphatase (Bacci et al 1993;Meyers et al 1992), and high lactate dehydrogenase (Bacci et al 1994;Meyers et al 1992) …”

Chronic Recurrent Multifocal Osteomyelitis

“…Ewing sarcomas, the second most common primary bone malignancy in childhood, are undifferentiated neoplasms that may occur in almost any bone and cause localized pain and swelling [63]. Molecular genetic studies looking for particular chromosomal translocations and/or their fusion transcripts are usually required to confirm the diagnosis.…”

Metformin inhibits the proliferation, metastasis, and cancer stem-like sphere formation in osteosarcoma MG63 cells in vitro

“…Osteosarcoma (OS) is the most frequent primary malignant bone neoplasm which is characterized by high local aggressiveness and a tendency to metastasize [1]. In spite of the use of neoadjuvant chemotherapy and improvements in surgical technology that have brought prominent promotion in survival rate, a significant number of relapse or metastasis still occur, especially to the lungs and distant bones [2,3].…”