What is the recommended management approach for bone tumors?

Management of Bone Tumors

Bone tumors require centralized management by a multidisciplinary team at specialized centers to optimize patient outcomes and survival. 1

Diagnostic Approach

• Initial evaluation should include appropriate imaging: plain radiographs followed by CT scan as first-line investigation, with MRI indicated for suspected neurological complications (metastatic epidural spinal cord compression, radicular compression) 1
• In patients under 40 years with an aggressive, symptomatic bone lesion, referral to an orthopedic oncologist should be considered before further workup due to significant risk of primary bone malignancy 1
• In patients 40 years or older, workup for potential bone metastasis is recommended, including bone scan, chest x-ray, and CT of chest/abdomen/pelvis with contrast 1
• Biopsy must be performed at specialized centers by experienced surgeons, as inappropriate biopsy can compromise functional prognosis and sometimes survival outcomes 2
• Biopsy options include percutaneous needle biopsy under radiological control or open surgical biopsy (preferable for primary bone tumors, especially cartilaginous tumors) 2

Treatment Principles

Primary Bone Sarcomas

• Treatment requires a multidisciplinary approach involving pathologists, radiologists, surgeons, radiation and medical oncologists at specialized centers 1, 3
• Core treatment components include:
  1. Surgical resection (extensive, en bloc excision without breaching the tumor)
  2. Neoadjuvant/adjuvant chemotherapy for chemosensitive subtypes
  3. Radiation therapy for specific indications

Surgical Management

• Surgical excision is the mainstay of treatment for localized bone tumors 3
• Complete surgical excision requires removal of the affected bone segment and invaded soft tissues as a single block, without breaching the tumor, preserving a peripheral margin of healthy tissue 2
• Limb-sparing approaches are possible in approximately 80-90% of cases without compromising survival 2, 4
• Reconstruction options include osteosynthesis, bone grafts (autografts/allografts), prostheses, or composite reconstruction 2
• Amputation is indicated only when conservative resection is impossible, such as with massive tumors invading vessels and nerves, tumor infection, inappropriate biopsy, or local relapse 2

Chemotherapy

• Multiagent chemotherapy is essential for osteosarcoma and Ewing sarcoma 5, 4
• Standard approach involves neoadjuvant (pre-operative) chemotherapy followed by surgical resection and adjuvant (post-operative) chemotherapy 5
• Most effective chemotherapy agents for osteosarcoma include high-dose methotrexate, doxorubicin, cisplatin, and ifosfamide/etoposide 4
• For Ewing sarcoma, compressed VDC/IE (vincristine, doxorubicin, cyclophosphamide, ifosfamide, etoposide) is the preferred first-line treatment for medically fit patients 1

Radiation Therapy

• Reserved for cases with marginal or incomplete resection, or for definitive treatment of unresectable disease 5
• Specific indications for postoperative radiotherapy in Ewing sarcoma include positive surgical margins, poor response to chemotherapy, and large tumors in high-risk areas 1
• For chordoma, high-dose radiation therapy including proton or carbon ion beam RT is recommended, especially for skull base tumors 1

Specific Tumor Types

Osteosarcoma

• Multimodality treatment with neoadjuvant chemotherapy, surgery, and adjuvant chemotherapy has improved 5-year survival to 60-70% in localized disease 1
• Limb-sparing procedures are safe alternatives to amputation for many patients 4

Ewing Sarcoma

• Multimodality treatment improves 5-year survival to almost 80% in localized disease and 20-40% in metastatic disease 1
• Local treatment decisions require discussion between bone sarcoma MDT, patient, and family 1

Chondrosarcoma

• Primarily surgical management, as most subtypes are chemoresistant 1
• Doxorubicin and ifosfamide may be active in high-grade lesions 1

Giant Cell Tumor of Bone

• Treatment options include en bloc excision or intralesional curettage 1
• Denosumab is standard treatment for unresectable or metastatic disease 1

Chordoma

• En bloc R0 resection is standard treatment when feasible 1
• R1 surgery plus high-dose RT is recommended for skull base and upper cervical tract chordoma 1

Bone Metastases Management

• Management strategy based on fracture risk and neurological risk evaluation 1
• Modern management integrates focal treatments (radiotherapy, surgery, interventional radiology), orthoses, and antiresorptive and systemic oncological treatment 1
• Treatment choice depends on pain, neurological risk, bone instability, patient characteristics, and treatment objectives 1
• Multidisciplinary approach must include rehabilitation, systemic treatment, antiresorptive treatment, and supportive care 1

Follow-up and Surveillance

• Follow-up of high-grade tumors should include physical examination of tumor site, assessment of function, and possible complications of reconstruction 1
• Recommended intervals: every 3 months for first 2 years; every 6 months for years 3-5; every 6-12 months for years 5-10; every 0.5-2 years thereafter 1
• Imaging should include local imaging and chest X-ray/CT 1
• Long-term monitoring for toxic effects of chemotherapy, surgery, and radiation therapy should continue for >10 years after treatment 1

Common Pitfalls and Caveats

• Delayed referral to specialized centers can negatively impact outcomes 1, 3
• Inappropriate biopsy technique can compromise limb salvage options and survival 2
• Secondary cancers may arise in survivors of bone sarcomas, either related to or independent of irradiation 1
• Long-term cardiac evaluation is important as deterioration of cardiac function can occur decades after anthracycline treatment 1