Soft Tissue Sarcoma: Symptoms, Causes, Types, Diagnosis and Treatment

20 Jul, 2023

What is Soft Tissue Sarcoma?

 Soft tissues in the human body support, connect and surround other organs and body parts. Some soft tissues are bone and cartilage, blood vessels, muscles, lymph vessels, tendons, fibrous tissues, tissues surrounding joints, and nerves. The cells of these soft tissues divide uncontrollably, resulting in soft tissue sarcoma. Although soft tissue sarcoma may occur in any soft tissues in the body, the abdomen, legs, arms, and retroperitoneum are mainly affected by soft tissue sarcoma.

How Common are soft tissue sarcomas?

 Soft tissue sarcoma is rare and occurs in about one to four of every 100,000 individuals worldwide. The Consolidated Report of the Population-Based Cancer Registries Incidence and Distribution of Cancer: 2012-14, published by ICMR, reported that the incidence among children varies between 3.6% in Delhi to 14.8% at Barshi among males and 3.7% at Bangalore to 9.5% at Bhopal among females.

Soft Tissue Sarcoma Types     

 Most Common Soft Tissue Sarcomas in Adults

  • Undifferentiated Pleomorphic Sarcoma

This soft tissue sarcoma in adults affects the arms, legs, or chest. It has the potential to spread rapidly to other body parts.           

  • Liposarcoma

This sarcoma type initiates in the fat cells and mostly affects the soft tissues of the abdomen and limb muscles but may also occur in other body parts.

  • Leiomyosarcoma

Smooth muscles are present in the bladder, abdomen, intestine, uterus, and blood vessels. Soft tissue sarcoma in adults that affects smooth muscles is known as leiomyosarcoma.

  • Most Common Soft Tissue Sarcoma Types in Teenagers and Young Adults

  • Rhabdomyosarcoma

This soft tissue sarcoma type usually affects the skeletal muscles (the muscles attached to bones and assist in body movement) and hollow organs, such as the uterus or bladder. However, it may also occur at other places in the body.

  • Ewing Sarcoma in Soft Tissue

This sarcoma type usually starts in the bone or in the soft tissues that surround the bone. The most common site is the pelvis or the leg bone. However, it may initiate in any bone.

  • Other Soft Tissue Sarcoma Types

  • Angiosarcoma

It occurs in the inner lining of the lymph and blood vessels. Although angiosarcoma may occur in any body organ, the most common sites are the spleen, breasts, liver, and skin.

  • Deramtofibrosarcoma Protuberans

This type of soft tissue sarcoma occurs in the dermis and may extend to the subcutaneous fat. In rare cases, fascia and the muscles may also be involved. The tumor is generally slow growing.

  • Epithelioid Sarcoma

This soft tissue sarcoma type is slow growing, and it usually affects the hands, arms, fingers, feet, and legs. In most cases, there is a small, firm lump or growth under the skin known as nodules.

  • Gastrointestinal Stromal Tumor

This soft tissue sarcoma type occurs in the gastrointestinal tract with symptoms of blood in the stool, abdominal pain, nausea, and vomiting. Although it may occur at any gastrointestinal tract site, the most common is the stomach and small intestine.

  • Kaposis Sarcoma

Kaposi’s sarcoma is characterized by the growth of lesions in the skin, mucus membranes lining the nose, mouth, throat, lymph nodes, and other body organs. The color of the lesions is generally purple, and they are comprised of cancer cells, white blood cells, red blood cells, and new blood vessels.

  • Myxofibrosarcoma

It is one of the most common types of sarcoma that usually occurs in the connective tissues of the legs and arms. It starts as a slow-growing lump under the skin and does not cause any soft tissue sarcoma symptoms at the initial stage of the soft tissue sarcoma.

  • Solitary Fibrous Tumor

This type of sarcoma generally affects older adults and occurs in the connective tissue that supports other organs. The most common site of solitary fibrous tumors is the lining of the lungs, known as the pleura.

  • Spindle Cell Neoplasm

Spindle cell neoplasm consists of spindle-shaped cells and mostly occurs in the legs, arms, and pelvis bones.

  • Synovial Sarcoma

Synovial sarcoma is also known as malignant synovioma and occurs in the leg, arm, or foot. Synovial sarcoma is also found near the joints, such as the ankle and wrists.  

Most Common Soft Tissue Sarcoma Symptoms

The most common soft tissue sarcoma symptoms are:

  • A new lump or a lump that grows fast in any area of the body.
  • A lump that causes discomfort and pain

  • Persistent abdominal pain that is getting worse

  • Black tarry stools occur due to bleeding in the stomach.

  • Blood in the stools to bleeding in the large intestine.

  • Nausea and vomiting

  • Unexplained weight loss

 Causes of Soft Tissue Sarcoma

Causes of soft tissue sarcoma are:

  • Inherited Syndromes: Causes of soft tissue sarcomas include inherited disorders, such as:

  • Li Fraumeni Syndrome

This condition occurs due to a defective TP53 gene. People with Li Fraumeni syndrome are at very high risk of cancers, such as brain tumors, leukemia, breast cancer, and sarcoma.

  • Neurofibromatosis

It is also known as von Recklinghausen disease and caused due to mutation in the NF1 and NF2 genes. Approximately 5% of people with neurofibromatosis develop sarcomas.

  • Gardner Syndrome

Gardner syndrome is caused by a defective APC gene. People with this condition have many polyps in their colon and are at increased risk of colon cancer.

  • Retinoblastoma

It is characterized by cancer of the eye in children and is caused due to mutation in the RB1 gene. People with retinoblastoma are at increased risk for developing sarcomas.

  • Werner Syndrome

This condition is caused due to the defective RECQL2 gene. People with Werner Syndrome are at increased risk for developing certain cancers, including soft tissue sarcoma.

  • Chemical Exposure

 People exposed to certain chemicals for a significant period are at increased risk for soft tissue sarcoma. The chemicals that increase the soft tissue sarcoma risk are arsenic, herbicides, and dioxin.

  • Radiation Exposure

Although general exposure to radiation accounts for less than 5% of sarcomas, exposure to radiation therapy for treating other cancer increases the risk of developing sarcomas. After radiation therapy, if sarcoma recurs, it is often at the site of radiation exposures (lymph nodes or breasts). The average time gap between radiation therapy and sarcoma development is ten years.

How is it Diagnosed?   

  • Physical Exam

The doctor may perform a comprehensive physical examination of the individual to determine the presence of a lump or other causes of symptoms. The patients may also be asked about their medical history, any prior exposure to radiation therapy, or the presence of any genetic disorder. The doctor may also inquire about the family history.

  • X-Ray

Once the doctor suspects the presence of a malignant lump, the patient is asked to undergo imaging testing. X-ray for soft tissue sarcoma is usually the first test ordered after the lump is detected. A chest x-ray may also be performed to test if sarcoma cancer has spread to the lungs.

  • Computed Tomography

CT scan provides detailed and high-quality cross-sectional images of the body. CT scan is recommended if the doctor suspects the presence of soft tissue sarcoma in the chest, abdomen, and back of the abdomen. This imaging test is also conducted to detect if sarcoma cancer has spread to the liver, lungs, or other organs. The patients may undergo a contrast CT scan in cases with high metastatic potential. CT scan also assists pathologists in performing biopsies (CT-guided biopsy).

  • Magnetic Resonance Imaging (MRI)

MRI scan involves using strong magnets and radio waves to create images of the body organs. MRI provides more detailed images compared to CT scans while evaluating sarcomas of the leg and arms. It detects the size and extent of sarcomas. The doctor usually recommends an MRI scan in case the X-ray scan shows abnormal growth.

  • PET Scan

A PET scan uses radioactive sugar, which is injected into the blood and used for the diagnosis of soft tissue sarcoma. The tumor cells are fast-growing cells and thus take up radioactive sugar, which is then detected by a scanner. PET scans help in determining the spread of sarcoma cancer to other body parts. It may also be combined with a CT scan (PET/CT).

  • Ultrasound

Ultrasound for soft tissue sarcoma uses sound waves for developing images of the target organ for diagnosis of soft tissue sarcoma. The test is usually conducted to determine the characteristics of the tumor, whether the tumor is solid or fluid-filled. The test is not usually ordered if the patient has already undergone MRI or CT scan.

  • Biopsy

If the doctor is highly suspicious about sarcoma, he may advise the patients to undergo a biopsy. A biopsy is often a confirmatory diagnosis of soft tissue sarcoma that involves obtaining a sample of abnormal tissues and sending it to the laboratory for further evaluation. There are different types of biopsies, such as core needle biopsy or fine needle aspiration, and the choice of biopsy depends upon the location and size of the tumor. Soft tissue mass biopsy can also be done through core needle biopsy or incisional biopsy. 

  • Core Needle Biopsy

Core needle biopsy is obtaining a sample of abnormal tissue using a long, hollow needle.

  • Incisional Biopsy

When the sample of the abnormal tissue cannot be withdrawn through needle biopsy, the doctor makes a small incision to remove the lump or abnormal tissue. 

 Stages of Soft Tissue Sarcoma 

  • Stage 1

This stage of soft tissue sarcoma is divided into stages 1A and 1B. Stage 1A is characterized by a tumor, which is less than 5 cm, while stage 1B tumor is greater than 5 cm. The tumor is of low grade or unknown grade in both the sub-stages.

  • Stage 2

The tumor at stage 2 is less than 5 cm and is of either mid or high grade.

  • Stage 3

This stage of soft tissue sarcoma is divided into stage 3A and stage 3B. Stage 3A is characterized by a tumor larger than 5 cm but not greater than 10 cm, while stage 3B tumor is greater than 10 cm. The tumor grade is mid or high in the stage 3 sub-stages.

  • Stage 4

The stage 4 soft tissue sarcoma is indicated by a tumor of any size, any grade, and spread to other organs.

Soft Tissue Sarcoma Treatment

 Oncologists have extensive knowledge about how to treat soft tissue sarcoma. Some of the soft tissue sarcoma treatment options are:

  • Chemotherapy

Chemotherapy for soft tissue sarcoma involves using drugs that kill the rapidly dividing cells in the body, including the sarcoma cancer cells. The chemotherapy drugs may be delivered orally or through injection. Chemotherapy for soft tissue sarcoma may be a primary treatment or administered as adjuvant therapy, depending upon the sarcoma type and stage of the soft tissue sarcoma. Oncologists may prescribe a combination of chemotherapy drugs for the management of sarcomas. Chemotherapy for soft tissue sarcoma may also be administered through isolated limb perfusion to treat sarcoma of the limb.

  • Radiation Therapy

Soft tissue sarcoma radiation therapy uses high-energy radiation to destroy sarcoma cancer cells. Soft tissue sarcoma radiotherapy may be combined with chemotherapy (chemoradiation) and surgery (adjuvant and neoadjuvant). The types of soft tissue sarcoma radiotherapy are external beam radiation, intraoperative radiation therapy, proton beam radiation therapy, and brachytherapy.

  • Targeted Therapy

There are certain proteins and enzymes present in sarcoma cancer that assist their abnormal growth and division. Targeted therapy specifically targets these enzymes and proteins and prevents the growth and division of sarcoma cancer cells. Targeted therapy usually has fewer side effects than chemotherapy for soft tissue sarcoma due to its specificity to cancer cells.

  • Immunotherapy

Immunotherapy uses the bodys immune system to kill sarcoma cancer cells. Certain processes in the cancer cells help them evade the immune system, resulting in their proliferation. Immunotherapy interferes with these processes and makes the malignant cells more vulnerable to the immune system.

Factors that Affect Soft Tissue Sarcoma Treatment

 Certain factors affect the type of soft tissue sarcoma treatment and its frequency in managing soft tissue sarcoma. These factors include:

  • Sarcoma type

Fast-growing tumors require more aggressive treatment compared to slow-growing tumors.

  • Size, Grade, and stage of the soft tissue sarcoma

 Tumors diagnosed at a later stage require advanced treatment delivered more frequently.           

  • Location of Tumor in the Body

 If the location of the tumor is such that it cannot be completely removed through soft tissue sarcoma surgery, the oncologists may use chemotherapy or radiation therapy in combination with surgery.

  • Patients Age

 As cancer treatment results in significant side effects, the age of the patients plays an important role in choosing the optimal treatment for soft tissue sarcoma.

When to See an Oncologist?           

Individuals should book an appointment with oncologists if they experience the following:

  • Lump
  • Abdominal pain
  • Nausea and vomiting
  • Unexplained weight loss
  • Loss of appetite
  • Blood in stool
  • Black tarry stools
  • Joint pain


Soft tissue sarcoma occurs in the connective tissues, such as bones, cartilage, muscles, blood vessels, lymph vessels, and nerves. These are rare types of tumors. The soft tissue sarcoma symptoms are lump that causes discomfort, nausea, vomiting, and abdominal pain. Diagnosis of soft tissue sarcoma is through physical examination, and the patients may be advised to undergo imaging tests. Soft tissue sarcoma treatment is through chemotherapy, radiation therapy, immunotherapy, and targeted therapy.


  • How serious is soft tissue sarcoma?

Soft tissue sarcoma is a serious condition and should be diagnosed early and treated effectively to prevent it from spreading to other organs.

  • Is soft tissue sarcomas cancer curable?

Soft tissue sarcoma may be curable if it is diagnosed early and can be completely removed. However, it is not usually possible to cure soft tissue sarcoma at advanced stages when it spreads to other organs.

  • Where does soft tissue sarcoma start?

Soft tissue sarcoma initiates in the connective tissues present in various parts of the body. The connective tissues are bone and cartilage, blood vessels, lymph vessels, tissues surrounding joints, tendons, muscles, fibrous tissues, and nerves.

  • Can blood tests detect soft tissue sarcoma?

No blood test detects soft tissue sarcoma. However, blood tests may help in determining the health of various organs, such as the liver, where soft tissue sarcoma may be suspected to metastasize.

  • Does soft tissue sarcoma grow fast?

The speed of growth of soft tissue sarcoma significantly varies within subtypes. However, soft tissue sarcoma is usually fast-growing, and the lump may grow into a noticeable size within a few weeks or months.

  • Can soft tissue sarcoma come back?

Soft tissue sarcoma can potentially return even after being completely removed through soft tissue sarcoma surgery. It may occur at the initial location or other body organs. The average time for soft tissue sarcoma recurrence is two to five years after soft tissue sarcoma surgery.

  • How does soft tissue sarcoma affect the body?

Soft tissue sarcoma symptoms depend upon the pace of occurrence. Some common effects of soft tissue sarcoma include lump with pain, abdominal pain, nausea and vomiting, and unexplained weight loss. Some patients may also experience constipation and a feeling of fullness.

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