What is the initial treatment approach for patients diagnosed with sarcoma?

Initial Treatment Approach for Sarcoma

The initial treatment approach for patients diagnosed with sarcoma is surgery with wide excision or compartmental resection as the main treatment for localized disease, followed by adjuvant radiation therapy in cases of high-grade sarcomas. 1

Diagnosis and Staging

Before initiating treatment, proper diagnosis and staging are essential:

• Patients with suspected sarcoma should be referred to specialized sarcoma centers with multidisciplinary expertise 1
• MRI is the main imaging modality for soft tissue tumors, while CT is useful for calcified lesions and retroperitoneal tumors 1
• Multiple core needle biopsies (≥16G) are the standard approach to diagnosis 1
• Staging includes physical examination and appropriate radiological techniques, with CT scan recommended to exclude lung metastases in operable patients 1
• Tumor size (≤5 cm vs >5 cm), location (superficial vs deep), and histological grade are key staging parameters 1

Treatment Algorithm for Localized Disease

Surgery

• Wide excision or compartmental resection is the standard approach, including removal of the cutaneous scar and biopsy tract 1
• The width of resection may be decreased when resistant anatomic planes (muscle fasciae, periosteum, perineurium) are not infiltrated 1
• Complete surgical excision with negative margins is the goal to minimize local recurrence 1

Adjuvant Therapy

• Adjuvant radiation therapy is recommended after wide excision of high-grade sarcomas 1
• Preoperative chemotherapy is not standard practice for operable patients but can be considered with radiotherapy for borderline resectable tumors 1
• Adjuvant chemotherapy is not routinely recommended but may be considered in younger patients with large, high-grade tumors 1

Non-Resectable Localized Disease

• For non-resectable tumors confined to an extremity, options include:
  • Chemotherapy with or without radiotherapy 1
  • Isolated hyperthermic limb perfusion with chemotherapy and/or cytokines 1
  • Regional hyperthermia combined with chemotherapy 1

Treatment of Metastatic Disease

• Chemotherapy is the standard treatment for metastatic disease 1
• Doxorubicin with or without ifosfamide is commonly used as first-line therapy 1
• For isolated lung metastases:
  • Metachronous (disease-free interval ≥1 year) resectable lung metastases without extrapulmonary disease are managed with surgery if complete excision is feasible 1
  • Synchronous lung metastases are treated with chemotherapy first, with surgery considered for responding disease 1

Special Considerations

• Soft tissue sarcoma requires a multidisciplinary approach by an experienced team 1
• Treatment response for metastatic disease should be evaluated after 2-3 cycles of chemotherapy 1
• Regular follow-up is recommended with physical examination every 3 months, MRI of the primary tumor site twice yearly for the first 2-3 years, and chest imaging based on tumor grade 1

Common Pitfalls and Caveats

• Inadequate surgical margins increase the risk of local recurrence; referral to specialized sarcoma centers is crucial 1
• Frozen-section technique for immediate diagnosis should be avoided as it may not allow complete diagnosis, especially when preoperative treatment is planned 1
• Biopsy should be performed in a way that the biopsy pathway and scar can be safely removed during definitive surgery 1
• Histological subtypes vary widely in their response to systemic therapy; proper pathological diagnosis is essential for treatment planning 1, 2
• Delaying referral to specialized centers may compromise optimal treatment outcomes 1