Stage II and III Soft Tissue Sarcoma: Limbs, Outer Trunk, Head or Neck

Patients diagnosed with stage II or III soft tissue sarcoma (STS) are treated differently depending on the size of the primary tumor, the tumor grade, and whether or not the sarcoma has spread to the local lymph nodes.

  • Stage IIA tumor are 5cm or smaller, have not spread to lymph nodes or more distant sites, and are either intermediate or high grade
  • Stage IIB tumors are larger than 5cm, have not spread to lymph nodes and are intermediate grade
  • Stage III tumors are larger than 5cm, have not spread to lymph nodes and are high grade or any size or grade and spread to lymph nodes.

Sarcomas are relatively uncommon cancers and individuals diagnosed with a STS should consider being evaluated by a doctor that specializes in the management of STS.

For individuals whose cancer has not spread to lymph nodes, surgery remains the cornerstone of treatment. For some individuals with limited stage IIA STS, surgery may be the only treatment required. For patients with stage II/III STS, radiation therapy may be used before surgery to shrink the cancer and/or after surgery to reduce the risk of recurrence. Surgical techniques used in STS may include the following:

  • Wide Local Excision (WLE): WLE involves removal of the sarcoma with some surrounding normal tissue to ensure complete excision can be used to treat sarcomas of the trunk, abdomen, head, and neck.
  • Mohs microsurgery: This type of surgery is designed to remove as little normal tissue as possible and is typically used where a good cosmetic result is desired such as the face or skin. The sarcoma is cut from the skin in thin layers, examining each layer under a microscope during the surgery until no more cancer cells are present.
  • Limb-sparing surgery: Arms and legs are common sites for STS and surgical procedures that avoid amputation are important. Radiation or chemotherapy is often given prior to wide excision to avoid amputation.
  • Lymphadenectomy: Lymph nodes are often sampled or removed during surgery to ensure the sarcoma has not spread.

Radiation therapy given prior to surgery is called neoadjuvant therapy and after surgery is referred to as adjuvant therapy.  Radiation therapy uses high-energy x-rays to kill cancer cells and is considered a local therapy. It can only kill cancer cells within the exact area it is delivered. Learn more about various radiation techniques here.

Stage IIB or III STS patients may have adjuvant therapy with a combination of radiation therapy and/or chemotherapy, and in some cases for patients who have had neoadjuvant radiation therapy, adjuvant therapy in the form of a radiation therapy boost might also be a treatment option. Although some patients with stage IIB or III STS may receive chemotherapy, there is limited evidence at this time regarding the benefits of chemotherapy in this setting. Patients should also consider participation in a clinical trial evaluating new treatments as they become available.

For stage II/III STS patients who are not initially eligible for surgery, treatment options may include radiation therapy, chemoradiation, chemotherapy, or isolated limb chemotherapy with the hope of debulking their cancer, making surgery an option.

Sarcoma Centers & Second Opinions

find a specialist2Because sarcomas are uncommon it is important to find physicians who have experience with this disease. In the United States there are several locations that have physicians and teams dedicated to advancing the treatment of sarcoma. Patients with sarcoma should at least consider getting a second opinion at one of these centers. Click here to learn more about second opinions and click here to view a comprehensive list of sarcoma centers in the United States.

Genetic Assessment

Li-Fraumeni syndrome is a hereditary cancer predisposition syndrome associated with soft tissue sarcoma. Genetic assessment may be suggested for some patients with STS. Learn more about genetic testing here.

Strategies to Improve Soft Tissue Sarcoma Treatment

The development of more effective treatment for STS requires that new and innovative therapies be evaluated with cancer patients in clinical trials. Patients may gain access to better treatments by participating in a clinical trial. Participation in a clinical trial also contributes to the cancer community’s understanding of optimal cancer care and may lead to better standard treatments. Patients who are interested in participating in a clinical trial should discuss the risks and benefits of clinical trials with their physician. Learn more about clinical trials here.

Adjuvant or Neoadjuvant Therapy:  In 2015 the US Food and Drug Administration (FDA) approved two new drugs, Halaven® (eribulin mesylate) and Yondelis® (trabectedin), for the treatment of advanced STS.  Votrient® (pazopanib) was approved by the FDA for the treatment of advanced STS in 2012. Clinical trials are ongoing in order to determine how best to use these drugs, whether they can improve outcomes in the adjuvant/neoadjuvant setting, and whether combining them with other drugs can further improve patient outcomes.

Yondelis is a chemotherapy agent that works by blocking certain types of proteins (transcription factors) that are involved in the copying and sending of tumor cell DNA. Clinical trial results have demonstrated that Yondelis improves outcomes compared to the standard agent, dacarbazine, in patients with advanced liposarcoma or leiomyosarcoma that has recurred or progressed following prior therapies.1

Halaven is a chemotherapy agent that works by causing abnormalities in microtubule formation in cells in order to prevent cell replication. Halaven was approved by the FDA based on clinical trial results directly comparing it to dacarbazine in STS patients with advanced liposarcoma. Overall survival almost doubled to 15.6 months for patients treated with Halaven, compared to only 8.4 months for patients treated with dacarbazine.2

Votrient is an oral medication known as a multi-targeted tyrosine kinase inhibitor. The drug works by interfering with the growth of new blood vessels that sarcomas need to survive. Clinical trial results have demonstrated that Votrient delays the time to cancer progression and increases overall survival in patients with advanced STS.3

Hyperthermia: Applying heat to the blood supply of an extremity affected by sarcoma has been utilized to increase the effectiveness of chemotherapy or radiation therapy. In this procedure, the blood supply to the affected limb is isolated and heated before returning it to the body. This treatment is usually accompanied by intra-arterial infusion of chemotherapy. The theory underlying this treatment is sound, and encouraging results have been reported. However, the technique is difficult to perform and there have been no randomized trials comparing the effectiveness of hyperthermia and chemotherapy to conventional chemotherapy treatment. Heat can also be applied directly to cancer with the use of microwaves, but the advantages of this approach are not clear.

Isolated Limb Perfusion:  Isolated limb perfusion is a procedure that sends chemotherapy directly to an arm or leg in which the cancer has formed. The flow of blood to and from the limb is temporarily stopped with a tourniquet and chemotherapy drugs are put directly into the blood of the limb sending a high dose of the drug directly to the sarcoma.

Care after Treatment

Appropriate follow-up care is important following treatment for stage II or stage III STS. Speak with your doctor to discuss your follow-up care plan which may include rehabilitation, physical exams and imaging.

Reference

1 Demetri G, von Mehren M, Jones R, et al. Efficacy and Safety of Trabectedin or Dacarbazine for Metastatic Liposarcoma or Leiomyosarcoma After Failure of Conventional Chemotherapy: Results of a Phase III Randomized Multicenter Clinical Trial. Journal of Clinical Oncology. 2015.62.4734.

2 United States Food and Drug Administration. FDA approves first drug to show survival benefit in liposarcoma. Available at: http://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm483714.htm. Accessed January 28, 2016.

3 FDA approves Votrient for advanced soft tissue sarcoma [FDA News Release]. U.S. Food and Drug Administration website. Available at: http://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm302065.htm