What is the standard treatment approach for a patient diagnosed with sarcoma?

Standard Treatment Approach for Sarcoma

Surgery is the standard treatment for all patients with adult-type, localized soft tissue sarcomas, complemented by radiation therapy for high-grade, deep tumors with a diameter of >5 cm. 1

Diagnosis and Initial Assessment

• All patients with suspected sarcoma should be referred to a specialist Sarcoma Multidisciplinary Team (MDT) before any treatment 1
• Diagnostic workup should include:
  • Pre-treatment histopathological diagnosis by percutaneous core biopsy, reviewed by a specialist sarcoma pathologist 1
  • Cross-sectional imaging of the primary tumor (usually MRI) 1
  • Chest CT scan to assess for lung metastases 1
  • Additional staging may be required depending on sarcoma subtype and location 1

Treatment Algorithm for Localized Disease

Surgery

• Wide excision is the standard surgical procedure, removing the tumor with a margin of normal tissue 1
• Surgery should be performed by a surgeon with appropriate training in sarcoma treatment 1
• The primary aim is complete excision with negative margins 1
• In rare situations, amputation may be necessary to achieve local control 1
• Plastic surgical reconstruction may be required as part of limb-conserving surgery 1

Radiation Therapy

• Adjuvant radiation therapy is recommended for:
  • High-grade sarcomas after wide excision 1
  • Deep tumors with diameter >5 cm 1
• Radiation therapy may be administered:
  • Postoperatively: 50-60 Gy with fractions of 1.8-2 Gy, with possible boosts up to 66 Gy 1
  • Preoperatively: typically using a dose of 50 Gy 1
• Radiation therapy is optional for deep lesions ≤5 cm or low-grade tumors 1

Chemotherapy

• Not standard practice for all patients with localized disease 1
• May be considered in specific situations:
  • High-risk patients (G2-3, deep, >5 cm tumors) 1
  • Younger patients with large and high-grade tumors 1
  • Patients with borderline resectable tumors (preoperative, with radiotherapy) 1
• When used, doxorubicin with or without ifosfamide is commonly administered 1, 2, 3

Treatment for Metastatic Disease

• Chemotherapy is the standard treatment for metastatic disease 1
• Doxorubicin with or without ifosfamide is the most commonly used regimen 1, 2, 3, 4
• Response evaluation should be performed after 2-3 cycles with appropriate imaging 1
• Surgery should be considered for completely resectable lung metastases 1

Follow-up

• Early detection of recurrence may influence the possibility of curative therapy 1
• Recommended follow-up includes:
  • Clinical examination every 3 months 1
  • MRI of the primary tumor site twice yearly for 2-3 years, then annually 1
  • For high-grade tumors: chest X-ray every 3-4 months for 2-3 years, twice yearly up to 5 years, then annually 1

Special Considerations

• Inadvertent surgery without preoperative diagnosis may require re-excision if margins are positive 1
• Non-resectable tumors confined to an extremity may be treated with chemotherapy with/without radiotherapy or isolated hyperthermic limb perfusion as an alternative to amputation 1
• Treatment approach should consider the specific sarcoma subtype, as some are more chemosensitive than others 1
• Bone sarcomas (osteosarcoma, Ewing sarcoma) typically require neoadjuvant chemotherapy before local treatment 1

Common Pitfalls to Avoid

• Failure to refer patients to specialist sarcoma centers, which can lead to suboptimal management 1
• Performing surgery without proper preoperative diagnosis and staging 1
• Inadequate surgical margins, which increase risk of local recurrence 1
• Treating all sarcoma subtypes with the same approach, despite their heterogeneity 5, 6
• Delaying referral of patients with suspicious masses (any unexplained lump that is increasing in size should be considered for ultrasound within 2 weeks) 1