Retroperitoneal Sarcoma (RPS)

What Is Retroperitoneal Sarcoma?

Sarcoma is a rare cancer that affects the connective tissues of the body, including the muscles, bones, nerves, cartilage, tendons, blood vessels, and fatty and fibrous tissues.

The retroperitoneum is home to retroperitoneal sarcomas. The retroperitoneum is positioned behind the abdominal lining, deep into the abdomen (tummy) and pelvis, and contains organs such as kidneys, pancreas, and bladder.

Who does Retroperitoneal Sarcoma Affect?

Although the median patient age is around fifty, retroperitoneal sarcomas can develop at any age and in both men and women.

How Does Retroperitoneal Sarcoma Affect Your Body

A fullness or heaviness in the abdomen may be felt by the patient. With large tumours, the patient may have shortness of breath when exercising, as well as reflux, constipation, and leg swelling. When retroperitoneal sarcomas press against a nerve, they can cause pain.

What Causes Retroperitoneal Sarcoma?

There is no known cause for any of these cancers. Retroperitoneal sarcoma can affect anyone. In fact, even though liposarcoma originates from fat, there is no clear association with a fatty diet or obesity leading to this type of cancer.

Symptoms of Retroperitoneal Sarcoma

A fullness or heaviness in the abdomen may be felt by the patient.  Symptoms can include:

• Abdominal mass
• Loss of appetite or weight loss
• Early satiety (feeling full after eating only a small amount)
• Blood in stools
• Lower extremity swelling
• Pain

With large tumours, the patient may have shortness of breath when exercising, as well as reflux, constipation, and leg swelling. When retroperitoneal sarcomas press against a nerve, they can cause pain.

What are the Stages or Progression of Retroperitoneal Sarcoma

The majority of retroperitoneal sarcomas are larger than 5cm in diameter and located deep within the body. As a result, when determining the stage of your disease, cancer's grade becomes more essential.

• Stage 1 - The cancer is low grade, tiny (less than 5cm), and has not spread to other parts of the body
• Stage 2 -  refers to any grade of cancer that has expanded to other parts of the body but has not spread to other sections of the body.
• Stage 3 - means high-grade cancer that has not spread to other parts of the body
• Stage 4 - A malignancy of any grade or size that has progressed to another section of the body

How is Retroperitoneal Sarcoma Diagnosed

Patients who suspect they have Retroperitoneal Sarcoma require a full 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 performs a comprehensive physical evaluation that can include:

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

Diagnostic Testing

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

• A scan -  is a procedure that uses ultrasound, x-ray, CT, or MRI to take photos of the inside of the body.
• A biopsy - is a process of extracting and analyzing a sample of tissue. The tissue is removed with a core (hollow) needle in core needle biopsies.

After a pathologist with sarcoma experience examines a tissue sample, a definitive diagnosis will be determined. A biopsy can also assist distinguish between a sarcoma and other illnesses that affect the abdomen. It can also assist in determining the best treatment for various retroperitoneal diseases.

Non-Surgical Treatment for Retroperitoneal Sarcoma

Treatment for your condition can begin immediately and can include:

• Radiotherapy
• To kill cancer cells, this treatment uses high-energy radiation beams. Retroperitoneal sarcomas are not commonly treated with it.
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• When a tumour is close to critical tissues, such as the organs in the retroperitoneum, radiation to the tumour can be employed before surgery to treat it. It can also be used to eliminate any cancer cells that remain after surgery. This treatment will not be done if the tumour is close to an organ and there is a possibility of the organ being harmed by radiation.
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• Chemotherapy

Anti-cancer medications are used to kill cancer cells in this treatment. Chemotherapy affects different subtypes of sarcoma in different ways. 

Chemotherapy can be used to treat retroperitoneal sarcomas depending on the cell types that make up the tumour. It's used in some subtypes to try to reduce a huge tumour so it may be operated on safely. Chemotherapy can sometimes make the tumour totally vanish. In this situation, radiotherapy is utilized to eliminate any remaining local cancer cells in the tumour region. 

Surgical Treatment for Retroperitoneal Sarcoma

The most common treatment for this form of sarcoma is surgery. When possible, the surgeon will remove the tumour as well as a portion of normal tissue around it. Taking a margin is the term for this. Surrounding organs may be touched or pressed by retroperitoneal sarcomas. In these circumstances, the surgeon will try to remove the tumour, as well as any nearby organs, all at once.

Depending on the size and location of your tumour, surgery may remove parts or all of the organs like the kidney, colon (bowel), pancreas, spleen, or bladder, which might affect your quality of life. If this is necessary, your surgeon will discuss the ramifications of removing the organ linked to or near to your tumour. Following surgery, a small number of people will require a colostomy or urostomy. Surgery is unlikely to be suggested in cases when the tumours cannot be entirely removed.

What if Retroperitoneal Sarcoma Goes Untreated?

The cells of sarcoma will continue to divide if it is not treated, and the sarcoma will grow in size. 

The sarcoma's growth results in a lump in the soft tissues. This puts pressure on any neighbouring body tissues or organs. Sarcoma cells from the original region may break away over time.