From the Guidelines
Pediatric bone tumors should be managed with a multidisciplinary approach, including imaging and biopsy for diagnosis, and treatment typically involves a combination of surgery, chemotherapy, and sometimes radiation therapy, with the goal of improving morbidity, mortality, and quality of life outcomes. The most common types of pediatric bone tumors are osteosarcoma and Ewing sarcoma, with osteosarcoma typically affecting teenagers during growth spurts and commonly occurring in the metaphysis of long bones, particularly around the knee, as noted in the study by 1. Ewing sarcoma can affect any bone but frequently involves the pelvis, femur, and tibia.
Diagnosis and Treatment
Diagnosis requires a multidisciplinary approach including imaging (X-rays, MRI, CT scans) and biopsy for definitive diagnosis, as recommended by 1. For osteosarcoma, standard chemotherapy includes methotrexate, doxorubicin, and cisplatin (MAP regimen), usually given for about 10 weeks before surgery and continued afterward for several months. Ewing sarcoma treatment includes vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide, with radiation often used for local control, as stated in 1 and 1.
Surgical Options
Surgical options range from limb-sparing procedures to amputation depending on tumor location and extent. Early detection is crucial for better outcomes, so any persistent bone pain, swelling, or unexplained fractures in children should prompt immediate medical evaluation, as emphasized by 1 and 1. Long-term follow-up is essential as these patients require monitoring for recurrence, metastasis, and treatment-related complications including growth disturbances, secondary malignancies, and organ toxicity from chemotherapy.
Key Considerations
- The biopsy of a suspected primary malignant bone tumor should be carried out at a reference center for bone sarcomas, with a primary biopsy under the supervision of a surgical team who will carry out the definitive tumor resection or by a dedicated interventional radiologist, as noted in 1.
- The entire affected bone should be evaluated radiologically prior to biopsy, and pulmonary metastases should be evaluated by CT scan, as recommended by 1 and 1.
- Approximately 20% of patients have detectable metastases at diagnosis, most commonly in the lungs and/or bone/bone marrow, with adverse prognostic factors including metastatic disease, pelvic localization, tumor diameter >8-10 cm, age >15 years, elevated serum LDH, and poor histological response to preoperative chemotherapy, as stated in 1 and 1.
Outcome Improvement
Improving outcomes in pediatric bone tumors requires early detection, prompt referral to a specialized center, and a multidisciplinary approach to treatment, with a focus on minimizing morbidity, mortality, and treatment-related complications, as emphasized by the most recent and highest quality study 1.
From the Research
Overview of Paediatric Bone Tumors
- Paediatric bone tumors are rare, accounting for 3%-5% of all pediatric malignancies 2
- Osteosarcoma (OS) and the Ewing sarcoma family of tumors (ESFT) are the most common primary pediatric bone malignancies, comprising 90% of malignant primary bone tumors in children and adolescents 3, 4, 2
Diagnosis of Paediatric Bone Tumors
- Fine-needle aspiration biopsy (FNAB) can be used in the initial diagnosis of pediatric bone and soft tissue tumors, with a high degree of accuracy when combined with ancillary studies 5
- Imaging techniques such as radiography, computed tomography, and magnetic resonance imaging are essential for establishing tumor invasion and staging of malignant lesions 2, 6
- Open biopsies have a higher diagnostic accuracy than percutaneous biopsies, especially for primary site biopsies 3
Characteristics of Paediatric Bone Tumors
- Specific characteristics such as tumor margin, pattern of bone destruction, periosteal reaction, presence or absence of soft tissue mass, lesion location, and type of matrix present must be considered to distinguish between benign and malignant tumors 6
- Osteosarcoma and Ewing sarcoma have distinct genetic and molecular characteristics, which can aid in diagnosis and treatment 4
Treatment and Prognosis
- Despite significant improvements in survival rates for other pediatric malignancies, the treatment and prognosis for pediatric bone tumors have remained largely unchanged over the past 3 decades 4
- Current challenges and emerging drug targets are being researched to improve treatment outcomes for pediatric bone tumors 4