Unveiling sarcoma: understanding its risk factors and treatment options

In the vast landscape of cancer, sarcoma stands as a unique and complex disease. Unlike more well-known forms of cancer, such as breast or lung cancer, sarcoma is a rare and diverse group of malignant tumors that originate from connective tissues including over 500 subtypes.

What is Sarcoma?

Sarcoma is a type of cancer that develops in the cells of connective tissues, including bones, muscles, tendons, nerves, fat and blood vessels. It is categorized into two main types: soft tissue sarcoma and bone sarcoma. Soft tissue sarcomas primarily affect the soft tissues, such as muscles and tendons, while bone sarcomas arise in the bones themselves. Common sites for sarcoma include the retroperitoneum as well as the extremities (arms and legs).

Risk Factors

The exact causes of sarcoma are still not fully understood. However, certain risk factors have been identified:

1. Genetic factors: Some sarcomas may have a genetic component, such as certain inherited syndromes that predispose individuals to develop sarcomas, like Li-Fraumeni syndrome or neurofibromatosis.
2. Radiation exposure: Prior exposure to radiation therapy for other cancers or radiation accidents may increase the risk of developing sarcomas.
3. Environmental factors: Exposure to certain chemicals, such as vinyl chloride or dioxins, may be associated with an increased risk of sarcoma development.
4. Age and gender: Sarcoma can affect individuals of any age, but certain subtypes have a predilection for specific age groups. For example, Ewing sarcoma typically affects children and young adults, while leiomyosarcoma is more common in middle-aged and older adults. Additionally, some sarcomas may have a slight male predominance.

Treatment Options

“The treatment of sarcoma depends on several factors, including the type, stage and location of the tumor,” says E. Ramsay Camp, surgical oncologist with the Dan L Duncan Comprehensive Cancer Center. “A multidisciplinary approach involving oncologists, surgeons, radiation oncologists and other healthcare professionals is key to having a tailored care plan for the patient leading to the best possible outcomes. The importance of a well-trained, experienced team is essential to provided optimal care for each patient.”

Some treatment options include:

1. Surgery: Surgery plays a crucial role in the management of sarcoma. The primary aim is to remove the tumor while preserving surrounding healthy tissues whenever possible. For extremity sarcomas, treatments are designed to preserve limb function whenever possible. In contrast, depending on the location, retroperitoneal sarcomas often require removal of multiple organs to clear the tumor. The extent of the surgery depends on the tumor’s size, location and stage. In some cases, reconstructive surgery may be necessary after tumor removal.
2. Radiation therapy: Radiation therapy uses high-energy beams to kill cancer cells and shrink tumors. It can be administered before surgery (neoadjuvant) to shrink tumors or after surgery (adjuvant) to kill any remaining cancer cells or as a palliative measure to relieve symptoms. When combined with surgery, it can reduce the chance of tumor recurrence.
3. Chemotherapy: Chemotherapy involves the use of drugs to kill cancer cells throughout the body. It is often used in combination with surgery or radiation therapy. Chemotherapy may be administered before or after surgery, depending on the type and stage of sarcoma. Typically, chemotherapy is reserved for patients with metastatic disease (cancer that has spread through the body away from the original source) but can be used as part of the treatment plan for locally advanced tumors.
4. Targeted therapy: Some sarcomas have specific genetic alterations that can be targeted by certain drugs. Targeted therapy aims to disrupt the signaling pathways involved in tumor growth and progression, leading to more effective treatment.
5. Immunotherapy: Immunotherapy harnesses the body’s immune system to recognize and destroy cancer cells. While still under investigation, immunotherapy has shown promising results in some sarcomas, particularly those with specific immune-related features.

The Importance of Rehabilitation and Follow-up Care

After sarcoma treatment, rehabilitation plays a crucial role in helping patients regain strength, mobility and overall quality of life. Physical therapy, occupational therapy and psychosocial support can aid in the recovery process.

Regular follow-up visits and imaging scans are necessary to monitor for any recurrence or metastasis of sarcoma. Early detection of any potential signs or symptoms can lead to prompt intervention and improve outcomes.

By Tiffany Harston, communications associate with the Michael E. DeBakey Department of Surgery