Search

Soft Tissue Sarcoma: What You Need to Know About this Rare Cancer

Last updated date: 18-Jul-2022

CancerGeneral HealthMedanta HospitalsSarcoma
Medanta Hospital

13 mins read

Overview

Sarcoma

Soft tissue sarcomas account for <1% of all adult tumors and 15% of pediatric tumors. They are a complex, heterogeneous group of tumors that can occur in any part of the body, making them diagnostically challenging.

Sarcomas can spread to other parts of the body while seriously damaging soft tissue or bone. Treatment for soft tissue sarcoma depends on the type, procedure, size, and other factors, including whether your body is suitable for chemotherapy or radiation. Let's take a deeper look at this rare disease.

 

What is the meaning of Sarcoma?

Synovial Sarcoma is one of the rarest types of cancer. It usually starts in the thighs, knees, feet, or arms. It is usually diagnosed after a lump or some related pain.

Synovial sarcoma has a male to female ratio of 1.2:1. It can be caused by a number of risk factors. But most are caused by inherited conditions.

 

What is Soft Tissue Sarcoma?

Soft Tissue Sarcoma

Our soft tissues are made up of muscles, ligaments, ligaments, fat, blood vessels, and other tissues. They connect and surround organs while supporting other structures of our bodies.

Soft tissue tumors may be harmless. Sarcoma is a type of cancer that begins in tissues and can be found anywhere in the body. However, they are commonly found in the head, arms, neck, abdomen, and legs.

Soft tissue sarcomas (STS) are a type of cancers that can afflict people of all ages and arise from any part of the body. These tumors can appear in a variety of ways, from aggressive metastatic angiosarcomas to benign lipomas. Imaging investigations and biopsies are needed to assess them.

 

Epidemiology

Soft tissue sarcomas are uncommon, with only 12,020 new cases and 4740 fatalities recorded in the United States in 2014. They account for around 1% of all cancer cases in the United States and about 2% of all cancer-related fatalities. As previously stated, they are classified based on where they occur in the body and which extremities they affect, with trunk STS being more prevalent than intraperitoneal and retroperitoneal instances.

When it comes to the extremities, they are more common in the proximal limb, with the thigh accounting for 44 percent of occurrences. Rhabdomyosarcoma, hemangioma, neurofibroma, and alveolar sarcoma are more common in children and young adults, and their age at diagnosis and histological subtype are frequently associated. 

 

What are the risk factors for soft tissue sarcoma?

risk factors for soft tissue sarcoma

The exact cause of what causes most soft tissue sarcomas is still under research. However, there are some risk factors identified as well. Most of these risk factors are inherited conditions due to gene mutations and affect genes in the cells of soft tissues.

Medical research indicates the following are risk factors for developing soft tissue sarcoma:

  1. Radiation during treatment for other cancers: Patients may develop sarcoma during treatment for breast cancer or lymphoma. However, it accounts for less than 5% of all sarcomas. The time between radiation exposure and sarcoma diagnosis is about 10 years.

  2. Familial cancer syndromes: They are disorders caused by gene mutations. People are often born with it, and it may increase the chances of developing soft tissue sarcoma. The main types of familial cancer syndromes that can cause soft tissue sarcoma are:
    • Neurofibromatosis is Also known as von Recklinghausen's disease. It forms the subcutaneous nerve. 5% of people with Neurofibromatosis will develop sarcoma.

    • Gardner Syndrome, which causes polyps in the colon and colon. It increases the risk of colon cancer as well and can cause problems outside the colon.

    • Li-Fraumeni syndrome, in which the susceptibility to cancer of the breast, brain, or blood is high. People with this disease are highly susceptible to radiation and may eventually develop sarcoma in new parts of the body while being treated.

    • Retinoblastoma It is the most common type of eye cancer in children.

    • Werner Syndrome is a condition that causes children to develop age-related diseases such as cataracts, changes in the skin, arteries. (clogged arteries) and an increased risk of soft tissue sarcoma.

  3. Exposure to cancer-causing chemicals In particular, vinyl chloride is at high risk for developing sarcoma. Exposure to polysilicic hydrocarbons, asbestos, and dioxins can also increase the risk.

  4. Lymphatic system damage caused by saliva (Clear fluid that contains cells from the immune system) accumulates caused by damaged lymph nodes during radiation therapy. It can result in swelling and is known as lymphedema.

 

What are the Types of Soft Tissue Sarcoma?

Despite having more than 30 species, soft tissue sarcoma is a broad category depending on the stem tissue. They are:

  • Muscle tissue
  • Fibrous tissue
  • Peripheral nerve tissue
  • Joint tissue
  • Blood vessels and lymph

The most commonly identified types are Pleomorphic Sarcoma (UPS), Gastrointestinal Stromal Tumor (GIST), Liposarcoma, Leiomyosarcoma, Ewing's Sarcoma, and Rheumatic Sarcoma. However, in India the two main types are Ewing's Sarcoma and Synovial Sarcoma.

 

What is Ewing's Sarcoma?

Ewing's sarcoma is a rare cancer. It is a tumor in the bone or soft tissue. It occurs in central areas such as the pelvis, femur, humerus, ribs, and cervical vertebrae. Symptoms include lumps in the legs and arms that grow over weeks or months.

Ewing's sarcoma occurs in adolescents and young adults with a 1:6 male/female ratio. Due to its complex nature, it is often difficult to diagnose.

 

What are the symptoms of soft tissue sarcoma?

symptoms of soft tissue sarcoma

 Soft tissue sarcoma may show no early signs or symptoms due to its complex nature. However, here are some symptoms you want to be aware of:

  1. New lumps of increased size
  2. Persistent and degenerative pain in the abdomen
  3. Blood in your vomit or stool
  4. Sticky black intestine (due to bleeding inside the stomach)

However, lumps and bumps on the body do not necessarily mean sarcoma. It is best to consult a medical professional if you are experiencing any of the above.

 

How is soft tissue sarcoma diagnosed?

soft tissue sarcoma diagnosis

The magnitude of the mass on physical examination, as well as the potential for neurovascular involvement, are factors in preoperative imaging and biopsy. As with STS of the extremities, the doctor must also assess the possibility of nodal involvement or distant metastases, as well as the relative respectability and potential postoperative functional limitations.

Numerous tests are used to identify soft tissue sarcomas. It usually starts with medical professionals determining whether the tumor is cancerous or benign and has spread in the body.

After examining the lump, in particular, a computed tomography (CT) scan or magnetic resonance imaging (MRI) scan is done to obtain a detailed view of the tumor.
To confirm the diagnosis and determine the tumor type, a biopsy is done. A biopsy sample can be obtained by removing tissue from the lump with a needle. In some cases, tissue samples may be obtained during surgery. The tissue sample is examined under a microscope by a pathologist. In addition to diagnosis, this specialist can determine the use of sarcoma by estimating the number of dividing cells (mitoses) in the sample. Cancer with many mitoses has a worse prognosis and may require treatment. proactive
If a malignant soft tissue tumor (sarcoma) diagnosis is made, the next step is to determine the cancer's staging, which is a measure of how far the cancer has spread. The procedure will depend on:

  • Tumor size
  • Tumor grade (Cells divide rapidly and abnormally, look abnormal under a microscope.)
  • Are cancer cells in nearby lymph nodes?
  • Has the cancer spread beyond the original site to other parts of the body? Positron emission tomography (PET-CT) may be done to identify the spread of the tumor. (preparation)

PET scans give a more accurate picture of where the cancer is. Because PET looks at the entire body, it's helpful when her doctor thinks the cancer may have spread to other parts of the body.

Treatment is advised only after the procedure, type and other important factors have been considered.

 

What are the Stages of Soft Tissue Sarcoma?

Your doctor first determines the type of sarcoma you have, then grades it based on how your cancer may have worked or has spread to other parts of your body. followed by the stage that was on

Sarcomas are graded on a G1 to G3 scale, with G1 being low grade and almost similar to normal cells, G2 or medium grade, and G3, or high-grade tumors, which may easily spread to other parts of your body.

The common stages of soft tissue sarcoma are:

Stage 1: The tumor is small and low grade.

Stage 2: The tumor is small. but with a higher grade

Stage 3: The tumor is larger and higher grade.

Stage 4: The cancer has spread to other parts of the body.

Tumor size is usually measured in centimeters (cm) or inches. A common food item that can be used to denote tumor size in cm. Including: 

  • Nuts (1 cm), 
  • Peanuts (2 cm),
  • Grapes (3 cm), 
  • Walnuts (4 cm),
  • Lemons (5 cm or 2 in),
  • Eggs (6 cm)
  • Peaches (7 cm) and
  • Grapefruit (10 cm or 4 in.)

 

Treatment options for soft tissue sarcoma

treatment for sarcoma

 There are different approaches to treating different types of soft tissue sarcoma. It is often the combination of treatment that is most suitable for the patient. However, the four types of standard treatments are:

  • Surgery
  • Radiation
  • Chemotherapy
  • Isolated regional therapy

 

Lipomatous Tumors

Adipocytic tumors are benign tumors that can form in any section of the body and induce symptoms mostly through mass impact. They are normally encapsulated, homogeneous, and free of nodularity or septations, albeit they may include calcifications or bleeding due to trauma. Excision beyond the tumor's capsule is used to treat it.

There is some clinical overlap with liposarcoma, the most common soft tissue sarcoma and a potentially malignant type. Liposarcomas are tumors that are larger than 10 cm in diameter, have extensive internal septations, and contain less than 75 percent adipose tissue. Retroperitoneal sarcomas account for 45 percent of all sarcomas.

Liposarcoma is treated with surgical excision with broad margins. Local recurrence is a regular occurrence. Complete excision of retroperitoneal liposarcomas is the aim. Well-differentiated liposarcomas often have a low risk of distant metastases, but de-differentiated extremities liposarcomas benefit from neoadjuvant radiation treatment.

 

Trunk and Extremity Sarcoma

The balance between retaining limb function and tumor management is delicate. Chemoresistant cancers are common, and investigations on the effectiveness of neoadjuvant and adjuvant chemotherapy have shown mixed findings. In terms of sufficient resection, some studies have suggested a surgical margin of 1 cm.

 

Desmoid Tumors

Fibroblastic tumors in an uncommon type. Around 80% of cases are spontaneous, whereas the rest are linked to familial adenomatous polyposis (FAP). Pregnancy and past trauma are linked in few occurrences. Women are 2 to 3 times more likely than males to develop them, and they are most commonly diagnosed between the ages of 30 and 40. They might come from the extremities, the intraperitoneal space, the belly, or the chest wall.

They tend to grow slowly, although they may be rather aggressive. The WNT signaling pathway is used by them. They might range in clinical appearance from an asymptomatic hard mass to a painful mass causing intestinal blockages or ischemia. They normally seem homogeneous and solid on radiography, with a clear or infiltrating border. These tumors have a significant recurrence rate even after excision.

 

Angiosarcoma

A malignant tumor that arises from the endothelium lining of blood arteries and can appear in almost any part of the body. Soft tissue sarcomas account for 2% of all cancers, with 40% of them being caused by radiation. They most commonly affect the scalp, head, neck (scalp), and viscera, and they usually strike in the seventh or eighth decade of life. Regional lymphatic nodal involvement is common.

They might be well-differentiated or poorly differentiated histologically. Again, surgical excision with negative margins is the goal of treatment. Tumors that are greater than 5 cm in diameter and show epitheliod are thought to have a bad prognosis. These tumors are frequently locally progressed and beyond excision at the time of presentation; nevertheless, chemoradiation has been shown to have some benefit.

 

Retroperitoneal and Visceral Sarcomas

Retroperitoneal sarcomas account for around 15% of all soft tissue sarcomas, with an average tumor size of 15 cm at presentation. The average age at presentation is 54, with a male to female ratio of 50:50. Depending on the size and location of the tumor, there are a number of clinical manifestations. Many are asymptomatic and only found by chance.

Abdominal discomfort, weight loss, early satiety, nausea, emesis, back or flank pain, paresthesia, and weakness are all possible symptoms. CT is the preferred method of investigation. Gross resection, with or without neoadjuvant or adjuvant chemoradiation, is the preferred therapy. Liposarcoma is the most common subtype. The Gastrointestinal stromal tumor is the most common intraperitoneal subtype. Approximately 70% of patients will recur despite good surgical resection.

 

Gastrointestinal Stromal Tumor (GIST)

The most prevalent kind of visceral soft tissue sarcoma is GIST tumors. The majority of them occur seldom. They arise in the gastrointestinal myenteric plexus' interstitial cells of Cajal and can appear anywhere throughout the GI tract. The stomach, small bowel, and rectum are the most common sites.

They operate as the bowel's pacemaker cells. GIST tumors feature a CD117 (also known as KIT gene) marker, which codes for the c-kit tyrosine kinase transmembrane receptor. Traditionally, a smooth muscle spindle cell neoplasm. They can be asymptomatic to symptomatic in clinical terms, including pain, nausea, hematemesis, and gastrointestinal (GI) blood loss.

They present endoscopically as a smooth submucosal tumor that can obstruct the visceral lumen. It looks well encapsulated on radiographs, with patchy necrosis inside the tumor causing heterogeneous regions of enhancement. Metastases are prevalent, with the liver and peritoneal surface being typical locations.

Localized lesions are transported to the operating theatre for full excision and empiric lymphadenectomy with negative margins. Imatinib is used as adjuvant therapy after resection and as neoadjuvant therapy for unresectable or locally advanced illness.

 

Leiomyosarcoma

Smooth muscle tumors that can arise from any section of the body are malignant. It is the second most prevalent subtype of soft tissue sarcoma. They happen in the sixth and seventh centuries. In women, the retroperitoneum and uterus are the most prevalent sites, although in men, they can occur everywhere. They are diverse, well-circumscribed tumors having cystic or necrotic center portions on gross examination.

 

Prognosis for Soft Tissue Sarcoma

Soft Tissue Sarcoma

In general, people with local soft tissue sarcomas have a very good prognosis with high cure rates. The main feature of an excellent prognosis is that the tumor is completely removed by surgery and has not spread beyond the margins of the tumor. Children tend to have a better prognosis than adults for both local and metastatic tumors.

Curative resection can be performed on confined and early-stage lesions with high long-term survival, although recurrences are common. Even after 10 to 15 years, the danger of recurrence exists, and patients must be monitored indefinitely.

Recurrences are most common in the first five years. A cure is not feasible for patients with advanced illness, and the median survival time varies according on the subtype. Chemotherapy has a low response rate in the majority of sarcomas . The patient's histological subtype, grade, and response all influence the outcome.

 

Complications

The tumor has several consequences, including migration of the sarcoma into surrounding tissues and neurovascular impairment.

  • Neurotoxicity, infertility, heart toxicity, infertility, and bone thinning caused by chemotherapy
  • Disfigurement, incapacity, discomfort, and poor cosmesis can all result after surgery.
  • Radiation can cause skin to become dry, leathery, avascular, and slow to heal.
  • There's also the possibility of secondary cancers developing.



Soft tissue sarcoma Prevention  

There is no known way to prevent sarcomas, however, as HIV infection appears to increase the risk of certain sarcomas. You should avoid behaviors that may lead to HIV infection.
If your occupation is exposed to substances that can cause soft tissue sarcomas, use appropriate protective equipment to minimize your exposure.

Thanks to advances in medical science and ongoing research and development in the treatment of sarcoma, soft tissue sarcoma, although a rare type of cancer, may be treated if detected early. Overall, the 5-year survival rate for soft tissue sarcoma is greater than 65%. It is important to understand that not everyone has risk factors for developing soft tissue sarcoma, especially due to its rare nature. However, talk to your doctor if you notice a visible lump or unmanageable pain.

 

Conclusion

Sarcomas are a diverse category of uncommon cancers that mostly start in the embryonic mesoderm. They usually show as an asymptomatic mass in an extremity, although they can appear anywhere in the body, especially in the trunk, retroperitoneum, and head and neck. Pretreatment radiologic imaging is essential for determining the local extent of a tumor, staging the illness, directing biopsies, and assisting with diagnosis. The recommended biopsy technique for soft tissue sarcomas is core-needle biopsy.

Despite advances in local control rates with extensive local resections and radiation treatment, metastasis and mortality remain a substantial concern in 50% of high-risk soft tissue sarcoma patients. The lungs are the most common location of metastasis, and it usually happens two to three years after treatment is finished. Progress in understanding the molecular features of these malignancies should lead to molecularly based medicines that may be included into regular treatment techniques in the near future.

Articles

Other Articles