Retroperitoneal Sarcoma (RPS)

The retroperitoneum is the space between the peritoneum and the posterior abdominal wall that contains the kidneys and associated structures, the pancreas, and part of the aorta and inferior vena cava. 

Approximately 15 percent of soft tissue sarcomas arise in the retroperitoneum. One third of malignant tumors that arise in the retroperitoneum are sarcomas. Soft tissue sarcomas are rare, with approximately 8,600 new cases diagnosed annually in the United States—less than one percent of all newly diagnosed malignancies.

Evaluation and treatment of retroperitoneal sarcomas are challenging because RPS tumors are relatively rare and frequently present with advanced disease in an anatomically complex location. Retroperitoneal sarcomas smaller than five cm are rarely seen because they are not noted by the patient until they have become larger in size.

Survival rates are all affected by the large tumor size on presentation, the inability to achieve wide surgical margins and the limitations of treating retroperitoneal sarcoma with radiation and chemotherapy. 

Retroperitoneal Sarcoma (RPS) Topics

Risks Factors for Retroperitoneal Sarcoma (RPS)

Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for RPS include the following inherited disorders:

  • Nevoid basal cell carcinoma syndrome (Gorlin syndrome).
  • Familial adenomatous polyposis (FAP; Gardner syndrome).
  • Li-Fraumeni syndrome.
  • Tuberous sclerosis (Bourneville disease).
  • Neurofibromatosis type 1 (NF1; von Recklinghausen disease).
  • Werner syndrome (adult progeria).
Symptoms of Retroperitoneal Sarcoma (RPS)

Retroperitoneal sarcomas most commonly present as an abdominal mass, often without other symptoms. Although the median patient age is approximately fifty years, retroperitoneal sarcomas occur at any age and arise equally in women and men. When symptoms are present, they relate to the mass effect of the tumor or to local invasion.

Retroperitoneal may cause the following symptoms:

  • 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
Stages of Retroperitoneal Sarcoma (RPS)

After a retroperitoneal sarcoma has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body. This process is called staging. Learn more about the stages of soft tissue tumors.

Treatment for Retroperitoneal Sarcoma (RPS)

Surgery is the primary treatment for RPS. Surgical oncologists at BWH are among the national leaders in retroperitoneal sarcoma surgery; they are all on the faculty at Harvard Medical School.

What You Should Expect

You will receive a thorough diagnostic examination to determine your course of treatment. Careful monitoring and the involvement of an experienced surgical oncologist are important to the successful outcome for patients with RPS.

If you are having surgery or a procedure, you will likely be scheduled for a visit to the Weiner Center for Preoperative Evaluation for pre-operative information and tests.

The day of surgery, you will be cared for in the operating room by surgeons, anesthesiologists and nurses who specialize in surgery for patients with RPS. After surgery, you will recover in the post-surgical care unit where you will receive comprehensive care by an experienced surgical and nursing staff.

Learn more about your hospital stay and returning home.

Multidisciplinary Care

The Center for Sarcoma and Bone Oncology at Dana-Farber/Brigham and Women’s Cancer Center provides advanced and innovative multidisciplinary care for patients with sarcoma, including RPS. Our treatment team includes surgical oncologists, medical and radiation oncologists, plastic surgeons, nutritionists, pathologists and anesthesiologists. In addition, patients have full access to BWH’s world-renowned academic medical community with its diverse specialists and state-of-the-art facilities.

Resources

Visit the Kessler Health Education Library in the Bretholtz Center for Patients and Families to access computers and knowledgeable staff.

Visit the Weiner Center for Preoperative Evaluation.

Access a complete directory of patient and family services.

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