Table of Contents

Understanding Soft Tissue Sarcoma

Soft tissue sarcoma is an uncommon cancer form originating in the body’s soft tissues, encompassing muscles, fat, blood vessels, nerves, tendons, and joint linings. These soft tissues play a vital role in supporting and connecting various body parts.

This cancer type can develop in any body part, though it predominantly appears in the arms, legs, and abdomen. Soft tissue sarcomas are diverse, with over 50 different subtypes, some predominantly affecting children, while others are more common in adults. The complexity and variety of these tumors often lead to challenges in accurate diagnosis, as they can be confused with other types of growths.

Treatment strategies for soft tissue sarcoma typically involve surgical intervention, supplemented by radiation therapy and chemotherapy when necessary. The choice of treatment hinges on the tumor’s size, type, location, and growth rate.

Soft tissue sarcoma is characterized by the formation of malignant cells within the body’s soft tissues, which include a mix of bone and cartilage, fibrous tissue, muscles, tendons, fat, blood vessels, lymph vessels, nerves, and synovial tissue. These sarcomas can emerge almost anywhere in the body, including the head, neck, trunk, and particularly in the arms, legs, abdomen, and retroperitoneum.

Each sarcoma subtype has unique cellular characteristics, visible under a microscope, depending on the specific soft tissue from which the cancer originates.

Soft tissue sarcoma can spread in three ways within the body:

  1. Tissue Spread: The cancer grows into nearby areas from its original site.
  2. Lymphatic Spread: The cancer enters the lymph system and travels through lymph vessels to other body parts.
  3. Blood Spread: The cancer gets into the bloodstream and travels through blood vessels to distant body parts.

Varieties of Soft Tissue Sarcoma

Soft tissue sarcoma encompasses a range of cancer types, each originating from various soft tissues within the body. Key examples include:

– Angiosarcoma: Originates in the cells of blood or lymph vessels.

– Gastrointestinal Stromal Tumors (GISTs): Arises in the connective tissues supporting the digestive system’s organs.

– Undifferentiated Pleomorphic Sarcoma: Develops in the soft tissues of the legs, arms, or chest, and is known for rapid spread to other body areas.

– Liposarcoma: Typically found in the abdominal area, though it can also occur in the thighs and other body parts.

– Leiomyosarcoma: Begins in smooth muscles located in the abdomen, bladder, uterus, intestines, and blood vessels.

Among teenagers and young adults, the most prevalent soft tissue sarcomas are:

– Rhabdomyosarcoma: A very rare form, with potential for recurrence. Approximately 70% of individuals with this diagnosis survive five years post-diagnosis.

– Ewing Sarcoma in Soft Tissue: Generally develops in tissues adjacent to bones. Annually, around 200 cases are diagnosed, primarily affecting individuals aged 10 to 20. The 5-year survival rate varies by age, being 76% for children under 15 and 59% for teenagers aged 15 to 19.

Understanding the Origins of Soft Tissue Sarcomas

What leads to the development of soft tissue sarcomas, and how do they vary?

The exact causes of most soft tissue sarcomas remain uncertain. These cancers begin with genetic mutations in the DNA of connective tissue cells. This DNA contains vital instructions for cellular functions. When mutated, these instructions get altered, causing cells to proliferate uncontrollably, bypassing the natural cell death cycle. This uncontrolled growth results in the formation of a tumor.

Depending on the sarcoma type, these cancerous cells may remain localized, leading to the tumor’s growth, or they may metastasize to other body areas. The specific cell type affected by the DNA changes determines the sarcoma subtype. For instance, angiosarcoma originates from blood vessel lining cells, while liposarcoma begins in fat cells.

Risk Factors for Developing Soft Tissue Sarcoma

What increases the likelihood of developing soft tissue sarcoma, and how do inherited disorders play a role?

Risk factors are elements that heighten the probability of contracting a disease, like soft tissue sarcoma. It’s important to note that having one or more risk factors doesn’t guarantee the development of sarcoma, and conversely, sarcoma can occur in individuals without any identifiable risk factors. If you’re concerned about your risk, discussing it with a healthcare professional is advisable.

Key inherited disorders that elevate the risk of soft tissue sarcoma include:

– Li-Fraumeni Syndrome: Linked to mutations in the TP53 gene.

– Von Recklinghausen Disease: Associated with neurofibromatosis type 1 due to NF1 mutations.

– Gardner Syndrome: Tied to mutations in the APC gene.

– Nevoid Basal Cell Carcinoma Syndrome (Gorlin Syndrome): Related to PTCH1 gene mutations.

– Tuberous Sclerosis (Bourneville Disease): Resulting from mutations in the TSC1 or TSC2 genes.

– Werner Syndrome (Adult Progeria): Caused by WRN gene mutations.

Additional risk factors include:

– Previous Radiation Therapy: Increases the likelihood of sarcoma development.

– Prolonged Lymphedema: Persistent swelling in arms or legs can contribute to risk.

– Exposure to Certain Chemicals: Such as Thorotrast (thorium dioxide), vinyl chloride, and arsenic.

– Infections: Being infected with HIV or human herpesvirus 8 can also increase the risk.

Identifying Signs and Symptoms of Soft Tissue Sarcoma

What are the indicators of soft tissue sarcoma?

One common indicator of soft tissue sarcoma is the presence of a lump or swelling in the body’s soft tissues. Often, a sarcoma manifests as a lump that’s not painful, typically found under the skin, frequently on an arm or leg. When sarcomas develop in the abdominal area, they might not show any noticeable signs until they grow significantly large. As the sarcoma enlarges, exerting pressure on surrounding organs, nerves, muscles, or blood vessels, symptoms can include:

– Experiencing Pain: Caused by the growing mass.

– Difficulty Breathing: Particularly if the sarcoma is in the abdomen and pressing against respiratory organs.

Diagnosing Soft Tissue Sarcoma: Essential Steps and Tests

How is soft tissue sarcoma identified?

To diagnose soft tissue sarcoma, medical professionals primarily use a biopsy, with the method depending on the tumor’s size and location. The common biopsy methods include:

– Core Needle Biopsy: Extracting tissue with a wide needle, often guided by ultrasound, CT scan, or MRI.

– Incisional Biopsy: Removing part of a lump or tissue, especially when core needle biopsy results are inconclusive.

Post-biopsy, several tests are conducted on the extracted tissue:

– Immunohistochemistry: This lab test uses antibodies to detect specific markers in the tissue sample, helping in cancer diagnosis and differentiation.

– Flow Cytometry: Measures cell characteristics in a sample, such as cell count and the presence of markers on the cell surface.

– Molecular Profiling: Identifies genes, proteins, or molecules in a tissue sample that may indicate cancer.

– Microscopy (Light and Electron): Observes cells under microscopes for any cellular changes.

– Cytogenetic Analysis: Examines chromosomes in cells for abnormalities indicative of cancer.

– FISH (Fluorescence In Situ Hybridization): Uses fluorescent DNA to identify genes or chromosomes in cells.

To stage the cancer, your doctor might perform these tests:

– Chest X-ray: Captures images of organs and bones inside the chest.

– Blood Chemistry Studies: Analyzes blood for substances indicating disease.

– Complete Blood Count (CBC): Assesses the number of various blood cells and hemoglobin content.

– CT Scan: Creates detailed internal body images.

– MRI: Generates detailed images using magnets and radio waves.

– PET Scan: Detects malignant cells by how they metabolize a radioactive sugar solution.


Each test contributes to a comprehensive understanding of the sarcoma’s characteristics, aiding in developing an effective treatment strategy.

Determining Treatment and Prognosis for Soft Tissue Sarcoma

What influences the treatment approach and recovery outlook for soft tissue sarcoma?

The decision-making for treating soft tissue sarcoma and predicting its recovery outcome hinges on various factors, including:

– Sarcoma Type: The specific variety of soft tissue sarcoma being treated.

– Tumor Attributes: This encompasses the tumor’s size, its grade (a measure of how much the cancer cells resemble normal cells and how quickly they are likely to grow and spread), and its stage (extent of cancer in the body).

– Tumor Location: The part of the body where the tumor is situated.

– Surgical Outcomes: Whether the tumor could be entirely removed through surgery.

– Patient Health Factors: This includes the age and overall health condition of the patient.

– Cancer Recurrence: Whether the cancer has returned after initial treatment.

Smaller, low-grade tumors, particularly those located in the trunk, arms, or legs, often respond well to surgical treatment alone. On the other hand, high-grade sarcomas, known for their aggressive nature and higher likelihood of metastasis, require more comprehensive treatment strategies.

Treatment Options for Soft Tissue Sarcoma

Patients with soft tissue sarcoma have access to various treatment modalities. Some are well-established, while others are under investigation in clinical trials. Trials play a crucial role in advancing treatment by evaluating the effectiveness of new methods. When new treatments outperform existing ones, they may become the new standard. Participation in clinical trials is an option that patients might consider.

Surgical Treatment

Surgery is a primary treatment for soft tissue sarcoma, especially for smaller, low-grade tumors. Surgical techniques include:

– Mohs Micrographic Surgery: This method involves sequentially removing and examining thin layers of the tumor until no cancer cells are detected.

– Wide Local Excision: Involves removing the tumor along with a margin of normal tissue. This approach is typical for tumors in sensitive areas like the head, neck, abdomen, and trunk.

– Limb-Sparing Surgery: Aimed at removing the tumor in an arm or leg without amputation, preserving the limb’s function and appearance. Pre-surgical radiation or chemotherapy may be used to shrink the tumor.

– Amputation: Rarely used, it involves removing part or all of an arm or leg.

– Lymphadenectomy: Removal of lymph nodes to check for cancer spread.

Radiation Therapy

This treatment uses high-energy rays or particles to kill cancer cells. Types include:

– External Radiation Therapy: A machine directs radiation towards the cancer from outside the body.

– Intensity-Modulated Radiation Therapy (IMRT): A 3-D approach that reduces damage to nearby healthy tissue.

– Internal Radiation Therapy: Involves placing radioactive material close to or inside the cancer.


Involves drugs to stop the growth of cancer cells, either by killing them or inhibiting their division.

Targeted Therapy

This treatment identifies and attacks specific cancer cells. Types include tyrosine kinase inhibitors like pazopanib, imatinib, sunitinib, and regorafenib, and histone methyltransferase inhibitors like tazemetostat.


This utilizes the body’s immune system to fight cancer. Types include immune checkpoint inhibitors like CTLA-4 and PD-1/PD-L1 inhibitors.

Clinical trials often offer patients access to new therapies and contribute to advancing future cancer treatments. Patients may experience side effects from treatments, and it’s important to discuss these with healthcare providers. Participation in a clinical trial can be a significant treatment choice for some patients.

Stage I Soft Tissue Sarcoma Treatment Approaches

For Stage I soft tissue sarcoma, treatments may include:

– Surgical procedures such as Mohs microsurgery for minor skin sarcomas, wide local excision, or limb-saving surgeries.

– Pre- and/or post-surgical radiation therapy.

– Intense radiation therapy for non-surgical tumor removal cases.

Managing Stage II and III Soft Tissue Sarcoma without Lymph Node Involvement

Treatments for Stage II and III soft tissue sarcoma, excluding lymph node involvement, can involve:

– Surgical tumor removal via wide local excision or limb-sparing surgery.

– Radiation therapy pre or post-surgery.

– Combined pre-surgical radiation or chemotherapy, sometimes followed by post-surgical radiation.

– Intense radiation for tumors that are surgically inoperable.

Advanced Stage III Soft Tissue Sarcoma with Lymph Node Spread

For advanced Stage III soft tissue sarcoma with lymph node metastasis, treatment options are:

– Wide local excision surgery paired with lymphadenectomy, possibly followed by radiation.

– Surgery combined with neoadjuvant (pre-surgery) or adjuvant (post-surgery) therapies, including chemotherapy or radiation.

Stage IV Soft Tissue Sarcoma Management

Treatment for Stage IV soft tissue sarcoma typically includes:

– Chemotherapy options.

– Surgical intervention to remove metastasized cancer in the lungs.

– Targeted therapy using tyrosine kinase inhibitors like pazopanib or imatinib.

Recurrent Soft Tissue Sarcoma Treatment Strategies

For recurrent soft tissue sarcoma, possible treatments involve:

– Surgical wide local excision, potentially combined with radiation.

– Amputation, though rarely performed.

– Surgical removal of recurred cancer in the lungs.

– Targeted therapy with tyrosine kinase inhibitors such as pazopanib or imatinib.

– Chemotherapy applications.

– Enrollment in clinical trials for immune checkpoint inhibitors like pembrolizumab, nivolumab, or ipilimumab.

Getting Ready for Your Medical Consultation

If you’re experiencing symptoms that are concerning, it’s important to schedule an appointment with your primary healthcare provider. Should they suspect soft tissue sarcoma, you will likely be referred to an oncologist, a specialist in cancer treatment. Given the rarity of soft tissue sarcoma, it’s beneficial to seek care from professionals experienced in treating this specific cancer, often found in academic or specialized cancer centers.

Before your appointment, it’s helpful to:

– Document all symptoms, even those that may not seem related to your primary concern.

– Compile a comprehensive list of all medications, vitamins, or supplements you are taking.

– Consider bringing a family member or friend along. They can help remember details from the appointment that you might overlook.

– Prepare a list of questions to maximize your appointment time. Prioritize these questions from most to least important.

Essential questions to ask about soft tissue sarcoma include:

– Confirmation of cancer diagnosis.

– Alternative causes for symptoms.

– Necessary diagnostic tests and any special preparations needed.

– Type and stage of the sarcoma.

– Available treatment options and recommendations.

– Possibility of complete cancer removal.

– Potential side effects of the proposed treatments.

– Availability of clinical trials.

– Managing other health conditions alongside cancer.

– Prognosis.

– Access to informational resources, such as brochures or recommended websites.

– Need for consultations with other specialists.

During your appointment, be prepared to discuss:

– The onset of your symptoms.

– Presence and nature of any pain.

– Factors that alleviate or aggravate your symptoms.

– Any family history of cancer, including specific types.

This proactive approach can ensure a more efficient and informative consultation, helping you and your doctor to better understand and manage your health situation.

Outlook / Prognosis

The curability of soft tissue sarcoma largely depends on the stage at which it is diagnosed and treated. The primary mode of treatment typically involves surgical removal of the tumors. If the surgery successfully removes the tumor before it has had a chance to grow and spread, there’s a possibility that the condition can be considered cured. However, it’s important to note that soft tissue sarcomas have a tendency to recur, even after surgery and other forms of treatment.

When discussing survival rates, it’s crucial to understand that these figures are based on the collective experiences of large patient groups and may not directly apply to every individual case. For personalized information regarding survival rates, it’s best to consult with a healthcare provider. In general, about 65% of adults diagnosed with soft tissue sarcoma are alive five years post-diagnosis.

For children and adolescents, survival rates vary depending on the specific type of soft tissue sarcoma. In cases of rhabdomyosarcoma, approximately 70% of patients survive five years following their diagnosis. The survival rates for Ewing sarcoma of soft tissue also fluctuate. Statistically, 76% of children under 15 years of age with soft tissue sarcoma survive for at least five years after their diagnosis, while the figure stands at around 59% for adolescents aged between 15 to 19 years.

It’s essential to approach these statistics with the understanding that they are general estimates and may not precisely reflect an individual’s prognosis. For more accurate and personalized information, it is always advisable to speak directly with a healthcare professional.