Understanding Retroperitoneal Sarcoma: Symptoms and Treatment

Written by Medicover Team and Medically Reviewed by Dr Kovvuru Bhaskar Reddy , Oncologists

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1. Causes 2. Find a Doctor 3. Diagnosis 4. Treatment 5. FAQs

Retroperitoneal sarcoma is a rare type of soft tissue cancer that develops in the retroperitoneum, the space at the back of the abdominal cavity. This area contains vital structures such as the kidneys, pancreas, major blood vessels, and parts of the digestive system.

Because there is considerable space in the retroperitoneum, these tumors can grow quite large before causing noticeable symptoms. Their location near important organs and blood vessels can also make diagnosis and treatment complex.

What Are the Types of Retroperitoneal Sarcoma?

Retroperitoneal sarcoma includes several subtypes based on the type of tissue or cells where the cancer begins. Identifying the subtype is important because it can influence treatment and prognosis.

Liposarcoma: Develops from fatty tissue and is one of the most common types of retroperitoneal sarcoma.
Leiomyosarcoma: Begins in smooth muscle tissue and may arise from major blood vessels in the retroperitoneum.
Solitary Fibrous Tumor: Develops from connective tissue and may behave as a slow-growing or aggressive tumor.
Malignant Peripheral Nerve Sheath Tumor: A rare sarcoma that develops from the protective covering of peripheral nerves.
Undifferentiated Pleomorphic Sarcoma: An aggressive soft tissue cancer made up of abnormal cells that do not resemble a specific tissue type.

What Are the Symptoms of Retroperitoneal Sarcoma?

Retroperitoneal sarcoma symptoms are often nonspecific and may not appear until the tumor becomes large enough to press on nearby organs, nerves, or blood vessels.

Abdominal Pain or Discomfort: Persistent abdominal pain may develop as the tumor grows.
Abdominal Mass: A large tumor may sometimes be felt as a lump or swelling in the abdomen.
Unexplained Weight Loss: Some people may lose weight without changes in diet or physical activity.
Nausea and Vomiting: Pressure on digestive organs can cause nausea and vomiting.
Changes in Bowel Habits: Compression of the intestines may cause constipation or other changes in bowel movements.
Feeling Full Quickly: A large tumor may press on the stomach and cause early fullness after eating.
Leg Swelling: Pressure on major blood vessels may interfere with blood flow and cause swelling in one or both legs.

Because these symptoms can occur with many other conditions, persistent or unexplained abdominal symptoms should be medically evaluated.

What Causes Retroperitoneal Sarcoma?

The exact cause of most retroperitoneal sarcomas is unknown. The cancer develops when genetic changes cause cells in the soft tissues of the retroperitoneum to grow and divide uncontrollably, eventually forming a tumor.

Genetic Conditions

Certain inherited disorders can increase the risk of developing soft tissue sarcomas. These include Li-Fraumeni syndrome and neurofibromatosis type 1.

Previous Radiation Exposure

People who have previously received radiation therapy may have a small increased risk of developing a sarcoma in the treated area years later.

Chemical Exposure

Long-term exposure to certain industrial chemicals has been investigated as a possible risk factor for soft tissue sarcomas, although most people with retroperitoneal sarcoma have no identifiable environmental cause.

Spontaneous Genetic Changes

In many cases, the genetic changes that lead to retroperitoneal sarcoma occur spontaneously and are not inherited from a parent.

When to See a Doctor for Retroperitoneal Sarcoma?

Medical evaluation is important for persistent or unexplained abdominal symptoms, especially when they worsen over time. A oncologist usually treats retroperitoneal sarcoma, with support from radiation oncologists, radiologists, pathologists, and other specialists.

You should see a doctor if you have:

Persistent abdominal or back pain that does not improve
An unexplained abdominal lump, swelling, or increasing abdominal size
Unexplained weight loss, reduced appetite, or ongoing changes in bowel habits

Get medical help immediately if:

You develop severe or rapidly worsening abdominal pain
You have persistent vomiting or signs of intestinal obstruction
You experience sudden weakness, fainting, or severe difficulty breathing

These could be signs of a serious complication like organ compression, which needs urgent care.

No doctors found for Retroperitoneal Sarcoma in any location.

How Is Retroperitoneal Sarcoma Diagnosed?

Diagnosing retroperitoneal sarcoma usually involves imaging studies, a biopsy, and laboratory evaluation. Accurate diagnosis is important because treatment depends on the tumor's location, size, subtype, and extent of spread.

Computed Tomography (CT) Scan

A CT scan is commonly used to identify the tumor, measure its size, and determine its relationship with nearby organs and major blood vessels.

Magnetic Resonance Imaging (MRI)

MRI provides detailed images of soft tissues and may help doctors assess whether the tumor involves nearby muscles, nerves, blood vessels, or organs.

Ultrasound

Ultrasound may help identify an abdominal mass, although CT or MRI is usually needed for a more detailed assessment.

Biopsy

A biopsy is essential for confirming the diagnosis. A tissue sample is usually obtained with an image-guided core needle and examined by a pathologist to identify the exact type and grade of sarcoma.

Additional Tests

Further imaging tests may be performed to determine whether the cancer has spread to other parts of the body and to help doctors plan treatment.

How Is Retroperitoneal Sarcoma Treated?

Treatment for retroperitoneal sarcoma depends on the tumor's size, location, subtype, grade, and whether it has spread. Care is usually provided by a multidisciplinary team experienced in treating sarcomas.

Surgery

Surgery is the primary treatment for retroperitoneal sarcoma when the tumor can be removed. The goal is to remove the entire tumor with a margin of healthy tissue to reduce the risk of recurrence.

Because these tumors can grow close to or involve nearby structures, adjacent organs may sometimes need to be removed along with the tumor. Treatment at a specialized centre with an experienced surgical team is important because these procedures can be complex.

Radiation Therapy

Radiation therapy may be considered for selected patients to improve local control of the cancer. It may be given before surgery to target the tumor while it is still clearly visible or, less commonly, after surgery.

Advanced radiation techniques can help target the tumor while limiting exposure to nearby healthy organs and tissues.

Chemotherapy

Chemotherapy is not routinely effective for every type of retroperitoneal sarcoma. It may be considered for certain sarcoma subtypes, aggressive tumors, or cancer that has spread to distant parts of the body.

Targeted Therapy

Some patients may benefit from medicines that target specific genetic or molecular features of the tumor. Molecular testing can help determine whether targeted treatment may be appropriate.

Clinical Trials

Clinical trials may provide access to new medicines, treatment combinations, or other approaches being studied for advanced or recurrent retroperitoneal sarcoma.

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What Are the Complications of Retroperitoneal Sarcoma?

Retroperitoneal sarcoma can cause complications as the tumor grows or returns after treatment. The type and severity of complications depend on the tumor's location and the structures it affects.

Organ Compression: A large tumor can press on the kidneys, intestines, pancreas, or other abdominal organs.
Blood Vessel Compression: Pressure on major veins can cause leg swelling and circulation problems.
Intestinal Obstruction: Tumor growth may block the intestines and cause severe abdominal pain, vomiting, and constipation.
Local Recurrence: Retroperitoneal sarcoma has a significant risk of returning in the same area after treatment.
Metastasis: Some aggressive sarcomas can spread to distant organs, particularly the lungs or liver.

What Is the Prognosis for Retroperitoneal Sarcoma?

The prognosis for retroperitoneal sarcoma depends on several factors, including the tumor's size, grade, histological subtype, extent of spread, and whether it can be completely removed with surgery.

Retroperitoneal sarcomas have a higher risk of local recurrence than many other soft tissue sarcomas. Regular follow-up with physical examinations and imaging tests is important after treatment to detect recurrence or spread as early as possible.

Advances in surgical techniques, imaging, radiation therapy, and personalized cancer treatment continue to improve outcomes for some patients.

How Is Retroperitoneal Sarcoma Different From Other Soft Tissue Sarcomas?

Retroperitoneal sarcoma differs from other soft tissue sarcomas mainly because of its location. Tumors in the arms or legs may be detected earlier and can often be treated with limb-sparing surgery, while retroperitoneal tumors may grow to a large size before causing symptoms.

The close proximity of retroperitoneal sarcomas to the kidneys, intestines, pancreas, nerves, and major blood vessels also makes surgical treatment more complex. These differences make specialized multidisciplinary care particularly important for people with retroperitoneal sarcoma.