Soft Tissue Tumors

Background

Clinical Key

There are a wide variety of soft tissue tumors that can occur throughout the body which range from the most aggressive malignant sarcomas, to locally aggressive tumors, to more benign tumors.

Malignant soft tissue tumors, also called sarcomas, are cancers that begin in the muscle, fat, fibrous tissue, blood vessels, or other supporting tissue in the body. There are over 50 different types of soft tissue sarcomas. They are often found in the arms, legs, head and neck, or abdomen and occur more commonly in adolescents and young adults. Soft tissue sarcomas account for more than 11,400 new diagnoses and 4,300 deaths each year.

Intermediate grade soft tissue tumors have features in between sarcomas and benign tumors. They are often locally aggressive and rarely spread to distant sites. Desmoid tumors, also called desmoid fibromatosis, are one example of this category. These lesions usually grow slowly but are difficult to treat as they are locally invasive, preventing surgical resection without significant morbidity. Even in cases where the tumors are considered to be completely resected, there is a high recurrence rate. Radiation and systemic medical therapy is often used to treat these tumors, but these treatments are not highly effective and may result in local and systemic side effects.

Benign soft tissue tumors are non cancerous but also can occur in multiple areas of the body. The most common benign soft tissue tumor is the lipoma, comprised of fat. Others involve different types of soft tissues including arteriovenous malformations, which are composed of aberrant blood vessels and can cause local pain and even swelling of an entire extremity.

Treatment

Surgery is the most common treatment for soft tissue sarcomas. In some cases, radiation or chemotherapy might be used to shrink the tumor before surgery or kill any tumor cells left behind after surgery. Lymph nodes, although typically not a site of sarcoma spread, also might be removed and checked for signs of cancer. The exact treatment regimen depends on the size and stage of the sarcoma.

Soft tissue sarcomas may recur after surgery. In such cases, surgery is used again to remove the tumor. In rare cases, amputation is needed.

Immunotherapy uses the immune system to fight disease and is currently being investigated in the treatment of sarcomas.

Focused Ultrasound

Focused ultrasound may offer a noninvasive way to destroy soft tissue sarcomas. Using imaging guidance, the physician directs a focused beam of acoustic energy toward the tumor. This energy heats and kills tumor cells without damaging surrounding tissue. As a non-invasive procedure, focused ultrasound might offer the following benefits:
  • It offers shorter recovery time with fewer complications.
  • Because it does not use ionizing radiation, it can be repeated as often as needed.
  • Patients can avoid the long-term effects of radiation or chemotherapy.
  • Can be used in locations that are difficult or impossible to access with conventional surgery

Surgery, chemotherapy and/or radiation remain the standard of care for soft tissue sarcomas, and offer the only proven methods of achieving cure. However, for some locally aggressive tumors such as desmoids, the same methods used to treat sarcomas may cause too much morbidity. In these tumors, which do not metastasize, it would be valuable to have a means of achieving local control of tumor growth with causing minimal side effects. A recent article states that MRgFUS may be a feasible treatment option for desmoid tumors as 4/5 patients that had 1 year follow up had a decrease in size of their tumors. A more recent publication reports the results of a multicenter study in which 15 patients with desmoid tumors were successfully treated using MR guided focused ultrasound. Several cases of soft tissue sarcomas have also been treated with MRgFUS with success and thus MRgFUS may prove helpful in the treatment of sarcomas where there are limited treatment options or there is recurrence. In one patient, focused ultrasound was used to treat a patient with a recurrent synovial sarcoma. Other soft tissue lesions have been treated such as benign arterio-venous malformations of the lower extremity.

Clinical Trials

A clinical trial is currently enrolling patients at Stanford University, exploring the feasibility of focused ultrasound for the ablation of soft tissue tumors (benign and malignant) of the extremities.

See a list of clinical trials here

Treatment Sites

Please see a list of treatment sites here

Regulatory & Reimbursement

The Model JC Haifu system has received regulatory approval in Europe for treatment of soft tissue tumors, including sarcomas. At present, focused ultrasound is not universally reimbursed for treatment of soft tissue sarcomas.

Patient Advocacy

Several organizations worldwide support patients with soft tissue sarcomas. Among them are:

Sarcoma Alliance, which works to improve the lives of patients affected by sarcomas through better diagnostic accuracy, improved access to care, education, and support. For more information, visit www.sarcomaalliance.org.

Sarcoma Patients Euronet, which works with clinical experts, research scientists, industry, and others to improve treatment and care for sarcoma patients in Europe by increasing awareness and providing patients with support and information. For more information, visit www.sarcoma-patients.eu.

 

Notable Papers

Avedian R, Bitton R, Gold G, Butts-Pauly K, Ghanouni P. Is MR-guided High-intensity Focused Ultrasound a Feasible Treatment Modality for Desmoid Tumors? Clin Orthop Relat Res and Related Research. 2016 Mar;474(3):697-704. doi: 10.1007/s11999-015-4364-0. A Publication of The Association of Bone and Joint Surgeons

Ghanouni P, Butts Pauly K, Bitton R, Avedian R, Bucknor M,Gold G. MR guided focused ultrasound treatment of soft tissue tumors of the extremities — preliminary experience. J Ther Ultrasound. 2015; 3(Suppl 1): O69.

Hu X, Cai H, Zhou M, He H, Tian W, Hu Y, Chen L, Deng Y. New clinical application of high-intensity focused ultrasound: local control of synovial sarcoma. World J Surg Oncol 2013;11:265.

Keshavarzi A, Vaezy S, Nobel ML, Chi EY, Walker C, Martin RW, Fujimoto VY. Treatment of uterine leiomyosarcoma in a xenograft nude mouse model using high-intensity focused ultrasound: a potential treatment modality for recurrent pelvic disease. Gynecol Oncol 2002;86:344-50.

Click here for additional references from PubMed.

     

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