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A retroperitoneal sarcoma is a tumor that originates in the retroperitoneum, the space at the back of the abdominal cavity near the kidneys and pancreas. These soft tissue sarcomas can develop in fat, smooth muscle, fibrous tissue, or nerves. Often, they grow undetected for long periods, gradually increasing in size—sometimes reaching up to 30 pounds. Approximately 80% of retroperitoneal sarcomas are malignant, though the condition itself is relatively rare.

Retroperitoneal sarcomas occur when cells in the soft tissues of the retroperitoneum undergo genetic changes, leading to uncontrolled cell growth and tumor formation. While the exact cause of these DNA mutations remains unknown, they result in an excess of abnormal cells that cluster together to form a tumor.

Retroperitoneal Sarcoma Risk Factors

Scientists identify several risk factors that may contribute to 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 hazardous substances like asbestos, vinyl chloride, arsenic, herbicides, and thorotrast
  • Prior radiation therapy for medical treatment

Although fatty tissue may be involved in some cases, researchers have not conclusively linked retroperitoneal sarcoma to a fatty diet, excess body weight, or obesity.

Symptoms

Retroperitoneal sarcoma often presents vague and nonspecific symptoms. Many individuals first notice a sensation of heaviness or fullness in the abdomen. As the tumor grows, additional symptoms may include:

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

Diagnosing Retroperitoneal Sarcoma and Treating

Physicians use imaging tests to detect and assess the tumor’s size and location. They typically order a computed tomography (CT) scan, magnetic resonance imaging (MRI) scan, or ultrasound. After imaging, they perform a needle biopsy to confirm the diagnosis.

Surgical removal remains the primary treatment for retroperitoneal sarcoma. In some cases, physicians recommend chemotherapy or radiation therapy after surgery to improve outcomes and enhance the patient’s quality of life.

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Resource: https://www.moffitt.org/cancers/sarcoma/diagnosis-treatment/types/retroperitoneal-sarcoma