About Sarcomas

What is a Sarcoma?

When the word sarcoma is part of the name of a disease, it means the tumor is malignant (cancer).

A sarcoma is a type of cancer that starts in tissues like bone or muscle. Bone and soft tissue sarcomas are the main types of sarcoma.

Soft tissue sarcomas can develop in soft tissues like fat, muscle, nerves, fibrous tissues, blood vessels, or deep skin tissues. They can be found in any part of the body.

Most of Sarcomas start in the arms or legs. They can also be found in the trunk, head and neck area, internal organs, and the area in back of the abdominal cavity (known as the retroperitoneum).

Who Does a Sarcoma Affect?

Sarcoma can affect people of all ages, depending on its sub-type, and people with compromised immune systems such as HIV infected individuals and organ transplant recipients with immunosuppression. 

How Does a Sarcoma Occur?

Sarcoma development is still poorly misunderstood but it involves unregulated growth and formation of mutated cells due to mutations in cell DNA that result in tumor formation.

Causes of Sarcoma?

The causes of Sarcoma are still not completely understood certain risk factors for Sarcoma development include:

• Genetic predisposition (family history)
• Exposure to carcinogenic chemicals
• Exposure to certain viruses such as Herpes Virus 8, for example
• Lymphedema (chronic swelling)

Symptoms of Sarcoma?

The symptoms for Sarcoma vary from type to type and are specific to each sub – type. Some common symptoms include:

• Bone pain
• Brittle bone resulting in an unexpected fracture with little or no injury
• Weight loss
• Abdominal pain
• A deep lump that can be appreciated through the skin

Types of Sarcoma

There are multiple subtypes of this group of cancer, some of the more known ones are:

• Angiosarcoma
• Ewing Sarcoma
• Kaposi’s Sarcoma
• Soft tissue sarcoma
• Osteosarcoma
• Synovial sarcoma
  

How is a Sarcoma Diagnosed

Patients who suspect they have a Sarcoma require a fully diagnosis this can include:

Medical History

Your doctor will ask questions about:

• Current symptoms and what if any activities aggravate your condition, or how injury was sustained,
• Obtain a medical history including family or genetic links etc
• Determine any related former conditions diseases and treatments,
• Record health metrics (weight, mobility, blood pressure, heart rate, etc)
• Confirm medications, past and present
• Record habits, diet, exercise and other related actions
• Understand any risk factors or related complications or links

Physical Examination

Your doctor perform comprehensive physical evaluation that can include:

• Examining the affected area,
• Evaluate any swelling, pain, bruising or other features, etc
• Determine impact of condition such as range or motion, pain, diet, lifestyle,
• Record levels or severity of any symptoms

Diagnostic Testing

Once your doctor has completed the physical examination further tests maybe required. These tests can help your doctor determine or eliminate possible causes or impacts, or complete the diagnostic process and can include:

• Pathology - blood, or tissue samples via biopsy
• X-Ray, CT Scan and PET Scans
• Ultrasound -
• Magnetic Resonance Imaging (MRI)

Once a final diagnosis has been completed your doctor can discuss with you and recommend any treatment options. Often surgery is required but various therapies such as radiation therapy and chemotherapy may be prescribed to reduce cancer's size so that it can be removed without the need for amputation or excess surgical damage.

Sarcoma Staging

Staging Dermal Sarcoma using TNM system:

• The extent of the tumor (T): How large is the cancer?
• The spread to nearby lymph nodes (N): Has the cancer spread to nearby lymph nodes?
• The spread (metastasis) to distant sites (M): Has the cancer spread to distant organs such as the lungs?
• The grade (G) of the cancer: How much do the sarcoma cells look like normal cells?

A prognosis is dependant on many factors some important ones are:

• The type of sarcoma you have
• The location of the cancer
• The stage (extent) of the cancer
• Your age and overall health
• How well the cancer responds to treatment

How is a Sarcoma Treated?

There are a few treatment options when it comes to Sarcomas, and the doctor can recommend the best possible option based on the patient’s age and extent of cancer spread, on a case by case basis. Treatment can range from:

• Performing surgery to remove the tumor and resect surrounding normal tissue.
• Radiation therapy that can deliver precisely aimed radiation beams such as protons and X-Rays at the cancer cells. It is sometimes used in conjunction to surgery.
• Chemotherapy which is not a common option for this type of cancer, but is sometimes used if the cancer has spread.
• Immunotherapy, which supports the immune system to better help the body fight cancer once it has spread.

Beyond these more traditional treatment options, there are also clinical trials that are being conducted to design more efficient and effective detection and treatment methods. 

Radiation therapy

Radiation therapy kills cancer cells by delivering high-powered beams of energy, such as X-rays or protons.

Radiation therapy can be provided in the following ways:

• Radiation from an External Beam. This sort of radiation is produced by a machine that revolves around you as you lie on a table. The radiation is directed to certain locations on your body by the machine.
• Radiation Treatment was given During Surgery (IORT). IORT is utilised after cancer has been removed following surgery. The radiation is focused on the area where the cancer was previously located. If the cancer is placed in a region where total removal of cancer during surgery is difficult, IORT may be advised.

Chemotherapy

Chemotherapy is a medicinal treatment that kills cancer cells by using chemicals. It can be taken as a pill, injected into a vein (intravenously), or both. Chemotherapy is most commonly used to treat undifferentiated pleomorphic sarcoma that returns or spreads to other parts of the body after the first treatment.

Targeted Drug Therapy

Targeted medication treatments are designed to target specific abnormalities found in cancer cells. Targeted medication treatments can kill cancer cells by inhibiting these aberrations. Targeted therapy medications may be coupled with chemotherapy in the treatment of undifferentiated pleomorphic sarcoma.

Immunotherapy

Immunotherapy is a type of cancer treatment that makes use of your immune system. Because cancer cells create proteins that help them conceal the body's natural ability to fight them, your body's disease-fighting immune system may not attack your cancer. Immunotherapy works by interfering with the immune system's natural processes to enable it to more effectively fight the cancer growth. Immunotherapy is often reserved for patients with advanced cancer.

Surgical Treatment for Sarcoma

The surgeons goal is to eliminate the malignancy as well as a margin (at least 1 to 2 cm) of healthy tissue around the affected tissue with the least amount of unnecessary tissue damage as possible. This is designed to ensure that all Sarcoma or cancer cells are removed completely.

Surgeons prefer to utilize limb-sparing surgeries when cancer affects the arms and legs. However, in some circumstances, the damaged arm or leg may need to be amputated.

When the removed tissue is looked at under a microscope, the doctor will check to see if cancer is growing at the edges (margins) of the specimen. If cancer cells are present at the edges, the tissue removed is said to have positive margins. This means that cancer cells may have been left behind. When cancer cells are left after surgery, the patient may need more treatment − such as radiation or another operation.

Most tumour can be resected with surrounding normal tissue and the wound closed together. For larger tumours, there can be a large defect, this can be covered with skin (skin graft) or from tissue elsewhere in the body (pedicled or free flap) which is done jointly with a Plastic Surgeon.

Sometimes, an amputation can’t be avoided. It might be the only way to remove all of the cancer. Other times, critical nerves, muscles, bone, and blood vessels would have to be removed along with the cancer. If removing this tissue would mean leaving a limb that can’t function well or would result in chronic pain, amputation may be the best option.

What If Sarcoma Is Untreated?

If left untreated, the prognosis of a sarcoma malignancy is based on its sub-type and extent ranging from being dangerous and fatal to being cured with a simple resection surgery.