Pleomorphic Sarcoma
Pleomorphic sarcoma is an aggressive type of soft tissue tumor characterized by cells that vary widely in shape and size. Soft tissue sarcomas are relatively rare, accounting for less than 1% of all new cancers reported each year. In the United States, approximately 3.4 new soft tissue sarcoma cases are diagnosed per 100,000 people annually.
Although soft tissue sarcomas are uncommon, pleomorphic sarcoma—also known as undifferentiated pleomorphic sarcoma—is among the more frequently diagnosed soft tissue sarcoma subtypes in adults and makes up a substantial portion of nonbone sarcomas.
Pleomorphic sarcoma most often arises in the soft tissues of the arms, legs or abdomen, though it can develop anywhere in the body. Because it tends to grow quickly and can spread, a prompt evaluation and treatment are essential to support the best possible outcome and quality of life.
What are the causes and risk factors for pleomorphic sarcoma?
Researchers have not yet identified the precise cause of pleomorphic sarcoma, and most cases develop sporadically with no clear explanation. Although many patients have no known risk factors, certain inherited conditions, environmental exposures and prior cancer treatments have been associated with a higher likelihood of developing soft tissue sarcomas. Scientists believe these factors may influence cellular growth and repair processes, creating an environment in which cancer can develop.
Commonly recognized risk factors for soft tissue sarcomas include:
- Genetic conditions such as Li-Fraumeni syndrome and neurofibromatosis type 1 (NF1)
- Previous radiation therapy, particularly when delivered to the area where the tumor later forms
- Exposure to harmful chemicals, including industrial toxins and herbicides linked to soft tissue changes
- Chronic swelling or lymphedema, which can affect tissue health over time
- Increasing age, as pleomorphic sarcoma occurs more often in older adults
Is pleomorphic sarcoma preventable?
There is no known way to prevent pleomorphic sarcoma. Genetic risk factors cannot be modified, and most patients have no identifiable contributing factors linked to their diagnosis. Reducing unnecessary radiation exposure and avoiding environmental carcinogens when possible may help lower risk, but these steps do not guarantee prevention. Early recognition and prompt medical evaluation remain essential.
What are the signs and symptoms of pleomorphic sarcoma?
Pleomorphic sarcoma symptoms can differ based on the size and location of the tumor. An early-stage soft tissue sarcoma may cause little to no discomfort, which can delay diagnosis. As the cancer progresses, it might begin to interfere with normal function, leading to more noticeable changes.
What are the early warning signs of pleomorphic sarcoma?
Early signs of pleomorphic sarcoma often involve subtle changes near the tumor site. The patient may notice a painless mass or swelling that gradually increases in size. Some people also experience mild discomfort or a sensation of fullness in the affected limb or body region. Because many benign conditions present similarly, a prompt evaluation by a medical professional is important if any unusual changes occur.
What are the most common symptoms of pleomorphic sarcoma?
Many patients with pleomorphic sarcoma begin to experience symptoms as the tumor grows. These issues can range widely depending on the cancer site, but often include:
- A growing lump or swelling
- Pain or tenderness near the mass
- Reduced mobility or stiffness in the affected area of the body
- Numbness or tingling sensations if the tumor presses on a nearby nerve
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Schedule an AppointmentWhat are the symptoms of advanced pleomorphic sarcoma?
Advanced cancer may disrupt the surrounding tissues or spread to distant organs. Symptoms of late-stage pleomorphic sarcoma may include:
- Significant pain or functional limitations
- Noticeable enlargement of the mass
- Fatigue or unexplained weight loss
- Breathing difficulties, if the cancer spreads to the lungs
How is pleomorphic sarcoma diagnosed?
The diagnostic process for pleomorphic sarcoma typically involves a combination of medical history, physical examination, imaging studies and tissue analysis. Because soft tissue tumors are relatively uncommon, a comprehensive evaluation by a sarcoma specialist is recommended.
What laboratory tests are used to diagnose pleomorphic sarcoma?
Lab testing alone cannot confirm a diagnosis of pleomorphic sarcoma, but blood work can help the physician rule out other conditions and evaluate the patient’s overall health before treatment begins. Common blood tests include:
- Complete blood count (CBC), which measures blood cell levels
- Metabolic panels, which assess the function of certain organs, such as the liver and kidneys
Additionally, the physician may order specialized lab testing if a hereditary cancer syndrome is suspected, such as blood tests that can detect inherited genetic mutations linked to increased cancer risk.
What imaging tests are used to diagnose pleomorphic sarcoma?
Imaging studies can help the physician pinpoint the tumor’s location, size and relationship to nearby structures. Common options include:
- Magnetic resonance imaging (MRI) to obtain detailed images of soft tissues
- Ultrasound to assess superficial soft tissue masses
- Computed tomography (CT) scans to evaluate deeper structures or check for possible cancer spread
- Positron emission tomography (PET) scans to identify metabolic activity suggestive of cancer
What procedures are used to diagnose pleomorphic sarcoma?
A definitive diagnosis of pleomorphic sarcoma requires a procedure to collect tissue for microscopic examination by a pathologist, who can identify cancer cells. Options may include:
- Core needle biopsy to remove a small tissue sample
- Incisional biopsy if a larger sample is needed
- Surgical biopsy, if less invasive procedures are not feasible
How is pleomorphic sarcoma treated?
Treatment for pleomorphic sarcoma is highly individualized and may involve one or more therapies depending on the tumor’s location, grade and extent. The goal is to remove or control the cancer while preserving function and quality of life.
How is surgery used to treat pleomorphic sarcoma?
Surgery is often the primary treatment for pleomorphic sarcoma. The goal is to remove the entire tumor along with a thin margin of surrounding healthy tissue, which can reduce the risk of recurrence by helping to ensure that any microscopic cancer cells near the tumor site are also removed. In most cases, limb-sparing techniques have replaced amputation, and reconstructive procedures can be used when needed to preserve or restore function.
How is radiation therapy used to treat pleomorphic sarcoma?
Radiation therapy may be administered before surgery to shrink the tumor, which can improve the chance of complete removal and help preserve nearby structures. It may also be recommended after surgery to eliminate any remaining microscopic cancer cells and lower the risk of pleomorphic sarcoma recurrence.
If surgery is not an option due to the tumor’s critical location or a patient’s overall health, radiation therapy can serve as the primary treatment to slow or control cancer growth. Modern radiation delivery techniques are designed to target tumors with a high level of precision, allowing for the administration of an effective dose while limiting exposure to surrounding healthy tissues and reducing potential side effects.
How is targeted therapy used to treat pleomorphic sarcoma?
Targeted therapy is designed to focus on the specific molecular features that drive tumor growth. By interfering with these unique pathways, targeted medications can disrupt the processes that allow sarcoma cells to survive and multiply. This approach is a key component of precision medicine, which involves tailoring cancer treatment to the individual characteristics of each tumor.
While targeted therapy is not appropriate for every patient with pleomorphic sarcoma, it may be considered if a tumor shows certain genetic mutations or biologic markers that make it more likely to respond. It may also be an option for a patient whose cancer does not improve with standard treatments or returns after prior therapy. As research advances, new targeted and precision-based treatments are being developed to more effectively match therapy to each tumor’s unique profile.
How is chemotherapy used to treat pleomorphic sarcoma?
Chemotherapy uses powerful medications that enter the bloodstream and circulate throughout the body to destroy cancer cells wherever they may be found. For pleomorphic sarcoma, chemotherapy may be recommended as part of a comprehensive treatment plan if the tumor’s characteristics suggest that systemic therapy could be beneficial or the risk of cancer spread is high.
Chemotherapy for pleomorphic sarcoma may be given before surgery to shrink the tumor and improve the chance of complete removal. It can also be used after surgery to help reduce the risk of recurrence by targeting microscopic cancer cells that may remain in nearby tissues. For advanced pleomorphic sarcoma that has spread beyond its original location, chemotherapy may help slow disease progression, shrink the tumor or ease symptoms.
The specific chemotherapy regimen will be tailored to the patient’s overall health, the tumor’s behavior and how the cancer responds over time. Ongoing evaluation will allow the healthcare team to adjust the treatment strategy as needed to help ensure optimal effectiveness.
How is immunotherapy used to treat pleomorphic sarcoma?
Immunotherapy works by strengthening the body’s natural immune response so it can better recognize and attack cancer cells. For pleomorphic sarcoma, immunotherapy may be considered in select cases, particularly when the tumor displays certain biologic features that make it more likely to respond to immune-based treatment. Options may include checkpoint inhibitors, which can help the immune system overcome signals that normally prevent it from targeting cancer, as well as other emerging approaches currently under study in clinical trials.
Not all patients with pleomorphic sarcoma are candidates for immunotherapy. Eligibility is determined based on the tumor’s molecular profile and behavior and the patient’s overall health. When appropriate, immunotherapy may offer an additional option for a patient whose cancer does not respond to standard treatments or returns after prior therapy. Through ongoing research, scientists continue to explore new immunotherapy strategies to better support patients with pleomorphic sarcoma.
Are there other treatment options for pleomorphic sarcoma?
Some patients may choose to participate in a clinical trial designed to evaluate new or emerging therapies for pleomorphic sarcoma. Clinical trials provide access to innovative treatments that are not yet widely available and may be appropriate for a patient seeking additional options beyond the standard of care.
In addition to medical therapies, supportive care can play an essential role throughout the treatment journey. Services such as physical therapy, nutritional counseling, pain management and emotional support can help the patient manage symptoms, maintain strength and achieve the best possible quality of life. These resources are valuable at every stage of care, whether used alongside active treatment or during recovery.
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If you would like to learn more about pleomorphic sarcoma, you can request an appointment with a specialist in Moffitt’s nationally recognized Sarcoma Program by calling 1-888-663-3488 or completing our new patient registration form online. We do not require referrals.
