The Diagnosis and Treatment of Osteosarcoma and Ewing's Sarcoma in Children and Adolescents

Abstract

Background: Osteosarcoma and Ewing's sarcoma in children and adolescents require age-specific interdisciplinary diagnosis and treatment to achieve optimal therapeutic outcomes.

Methods: The diagnosis and treatment of malignant bone tumors in childhood and adolescence are presented in the light of publications retrieved by a selective search, pertinent guidelines, and the authors' extensive experience in an interdisciplinary cancer center.

Results: Bone sarcomas make up approximately 5% of all malignancies in children and adolescents; the most common types are Ewing's sarcoma and osteosarcoma. Patients are often not referred to a specialized center until long after the onset of symptoms, as they and their physicians rarely consider the possibility of a bone tumor, and the symptoms are often trivialized. Bone pain of unknown origin, swelling, and functional limitations should be investigated with conventional x-rays. Lesions of unclear origin should be biopsied after a meticulous clinical and radiologic evaluation. Multimodal treatment consists of neo - adjuvant chemotherapy, limb-preserving resection if possible, and radiotherapy where indicated. In multicenter studies, patients with osteosarcoma achieve event-free survival in 64% of cases if their disease is localized, and 28% if it is metastatic; the corresponding figures for patients with Ewing's sarcoma are 80% and 27%, respectively.

Conclusion: With implementation of the current treatment recommendations, most children and adolescents with malignant bone tumors can be treated successfully with curative intent. These patients should be referred to a sarcoma center for diagnosis and treatment.

Figure:

Ewing’s sarcoma in an 11-year-old boy with an approximately 2-month history of pain in the right femur a, b) Initial X-rays of the right femur show a periosteal reaction in the medial region. c, d) T1-weighted magnetic resonance imaging (MRI) indicates an intraosseous lesion with a periosteal and soft tissue reaction. e, f) Following neoadjuvant treatment as recommended by the German Society for Pediatric Oncology and Hematology (GPOH) in the Ewing registry, wide resection and biological reconstruction with allogeneic femoral diaphysis, fibula with vascular pedicle, and plate osteosynthesis were performed.