From the Guidelines
The treatment options for soft tissue sarcoma are limited, and the prognosis is generally poor, with a median survival of approximately 12-18 months from diagnosis of metastases 1.
Treatment Options
- Surgery: may be considered to target symptomatic metastases or in an attempt to prolong the remission period 1
- Radiotherapy: may be used to control symptoms such as pain or dyspnoea, or to target symptomatic metastases 1
- Systemic treatment: may involve a combination of various strategies, including chemotherapy and targeted therapy, often used in a stepwise fashion 1
- Palliative care: is essential to improve the quality of life of patients with advanced disease, and may involve medications for pain or other complications, as well as supportive care 1
Prognosis
The prognosis for soft tissue sarcoma is generally poor, with a median survival of approximately 12-18 months from diagnosis of metastases 1. However, some patients may have a more indolent course, and periods without active treatment may be appropriate 1. The overall survival correlates with the absence of disease progression, not the degree of response 1.
Diagnosis and Staging
The diagnosis and staging of soft tissue sarcoma require a multidisciplinary approach, involving pathologists, radiologists, surgeons, radiation therapists, medical oncologists, and pediatric oncologists, as applicable 1. Magnetic resonance imaging (MRI) is the main imaging modality, and multiple core needle biopsies are the standard approach to diagnosis 1. The histological diagnosis should be made according to the World Health Organization (WHO) classification, and a pathological expert second opinion is strongly recommended in all cases 1.
Future Directions
There is a clear unmet need for more effective systemic therapies for patients with advanced/metastatic sarcomas 1. Targeting therapies to tumors and protecting normal soft tissues and bones from damage is an area of ongoing research 1. The discovery of neurotrophic tyrosine receptor kinase (NTRK) gene fusions as pan-tumor oncogenic drivers has provided new precision medicine-based treatment options for a subset of patients with sarcoma 1.
From the Research
Treatment Options for Soft Tissue Sarcoma
- Surgical resection is the mainstay of therapy for localized disease 2, 3, 4
- Neoadjuvant chemotherapy and/or regional hyperthermia may be used to maximize tumor shrinkage before surgery and to treat micrometastases 3, 4
- For metastatic disease, chemotherapy with anthracyclines remains the backbone of therapy 5, 3, 4
- Targeted therapies, such as trabectedin, eribulin, and pazopanib, have expanded the therapeutic armamentarium for metastatic STS 5, 3
- Histology-directed treatment is crucial for certain subtypes of STS, which are highly sensitive to targeted therapy and relatively insensitive to chemotherapy 5, 3
- Immune checkpoint inhibitors have proven to be effective for patients with alveolar soft part sarcoma 4
- NTRK-inhibitors should be evaluated in patients with NTRK-fusions 4
Prognosis for Soft Tissue Sarcoma
- The prognosis is excellent for patients diagnosed at an early stage and treated by adequate surgery 2
- Unresectable or advanced metastatic diseases shrink the overall survival at 5 years dramatically to less than 10% 2
- Overall prognosis is poor for patients with metastatic STS, despite significant progress in treatment options 5
- The use of adjuvant imatinib therapy in patients with completely resected localized GIST with a high risk of recurrence has significantly improved overall survival 3
Management of Complex Clinical Scenarios
- Limited metastatic disease, local recurrence, fungating STSs, and unplanned or incomplete resections of STSs present unique challenges for the multidisciplinary care team 6
- A multidisciplinary team approach is essential for treating patients in these challenging scenarios, with a recent focus on the integration of plastic and reconstructive surgery into the treatment algorithm 6
- Limb-salvage resection or amputation, as well as some combination of radiation therapy and/or ablative interventional radiology techniques, may be used to manage complex STS clinical scenarios 6