A retroperitoneal sarcoma is a tumor that originates in the retroperitoneum, an area at the back of the abdominal cavity near the kidneys and pancreas. These soft tissue sarcomas can form in fat, smooth muscle, fibrous tissue or nerves. In many cases, the tumor goes undetected and gradually grows over a long period, often reaching a massive size. A retroperitoneal sarcoma can weigh up to 30 pounds.

Up to 80% of retroperitoneal sarcomas are cancerous. Overall, the condition is relatively rare.

Retroperitoneal sarcoma causes

A retroperitoneal sarcoma develops when cells in the soft tissues of the retroperitoneum undergo harmful changes that cause them to grow uncontrollably, creating an overabundance of abnormal cells that bind together and form a tumor. The exact causes of the cellular DNA changes that lead to retroperitoneal sarcoma are unknown.

"My first experience it sounded like there was no hope. And when I came to Moffitt it was a totally different experience where there was hope, you know. There was going to be a silver lining at the end of all of this. And I was going to win."

Kevin, Sarcoma Survivor

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Retroperitoneal sarcoma risk factors

Scientists believe several risk factors may play a role in the development of retroperitoneal sarcoma, including:

Certain inherited cancer syndromes, such as nevoid basal cell carcinoma syndrome, familial adenomatous polyposis (FAP), Li-Fraumeni syndrome, tuberous sclerosis, neurofibromatosis type 1 (NF1) and Werner syndrome
Exposure to certain hazardous substances, such as asbestos, vinyl chloride, arsenic, herbicides and thorotrast
Prior radiation therapy for medical treatment

Although some retroperitoneal sarcomas develop in fatty tissue, researchers have not conclusively linked the condition to a fatty diet, excess body weight or obesity.

Retroperitoneal sarcoma symptoms

The symptoms of retroperitoneal sarcoma can be vague and nonspecific. The first warning sign may be a sensation of heaviness or fullness in the abdomen.

As the tumor grows, additional symptoms may occur, such as:

Abdominal pain and bloating
Back pain
Shortness of breath with physical exertion
Acid reflux
Constipation
Leg swelling
Nausea and vomiting
Unintended weight loss
Fatigue

Retroperitoneal sarcoma diagnostic testing

To visualize the tumor and evaluate its size and location, a physician will typically order images, such as a computed tomography (CT) scan, magnetic resonance imaging (MRI) scan or ultrasound. Usually, the imaging tests are followed by a needle biopsy. A biopsy is necessary to reach a conclusive diagnosis.

Retroperitoneal sarcoma treatment

The main form of treatment for retroperitoneal sarcoma is surgical removal of the tumor. In some cases, chemotherapy or radiation therapy may be administered after surgery to help ensure the best possible outcome and quality of life.

Benefit from world-class care at Moffitt Cancer Center

If you would like to learn more about retroperitoneal sarcoma, you can request an appointment with a specialist in Moffitt’s comprehensive Sarcoma Program by calling 1-888-663-3488 or submitting a new patient registration form online. We do not require referrals.

Types

Retroperitoneal Sarcoma