Soft Tissue Sarcoma

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Overview

Sarcoma is a type of cancer that happens when the cells present in bones and muscles begin to divide abnormally. The two most common forms are bone and soft tissue sarcomas.

Soft tissue sarcomas begin in the muscles, fats, lymph vessels, blood vessels, nerves and fat cells along with other kinds of tissues.

While they can occur anywhere in the body, the arms, legs and abdomen are the most prevalent sites.

Soft tissue sarcoma is one of the rare forms of cancer, and it can happen at any age.

Types

Oncologists have identified about 50 types of soft tissue sarcomas, which could arise from various cell types in different locations. Following are the types of soft tissue sarcomas that are commonly seen:

1. Angiosarcoma: This is a rare cancer type that begins in the lining of the lymph vessels and blood vessels. It is commonly seen forming in the skin of the head and neck region.
2. Dermatofibrosarcoma Protuberans: This cancer forms in the middle layer of the skin or dermis. Initially, it might look like a scar or bruise. However, as the disease progresses, lumps form beneath the skin layer. This cancer type is more common in the regions of the arms, trunk and legs.
3. Epithelioid Sarcoma: This is a type of soft tissue sarcoma that is both slow-growing and rare. Initially, it begins as one painless lump or nodule, and as the disease progresses, multiple nodules start forming. Epithelioid sarcoma usually forms in the regions of the arms, forearms, legs and feet. This cancer type could also form in the groin area, and it can behave aggressively.
4. Gastrointestinal Stromal Tumour (GIST): As the name says, these tumours form in the inner wall of the gastrointestinal tract.
5. Leiomyosarcoma: This type of soft tissue sarcoma forms in smooth muscle tissue. Although it can occur anywhere in the body, it is more common in the abdomen and uterus regions. It is more common among older adults.
6. Liposarcoma: Liposarcoma begins in the fatty tissue of the body. It is more common in older adults, and it usually occurs in the region of the limbs and abdomen.
7. Malignant Peripheral Nerve Sheath Tumours: This type of cancer develops in the lining of the nerves that branch out from the spinal cord to various body parts. This cancer type is aggressive, and the presence of a painful lump is one of the primary symptoms. These tumours usually form in the arms, trunk and legs.
8. Myxofibrosarcoma: This is a slow-growing type of soft tissue sarcoma, which is more common among people aged between 50 and 70. Painless lumps could be formed in the regions of arms and legs. This cancer type is more common among men than women.
9. Rhabdomyosarcoma: This is a rare type of soft tissue sarcoma that forms in the skeletal muscle tissue or connective tissues like tendons and ligaments. In some cases, it occurs in hollow organs such as the uterus and bladder. This cancer type can happen at any age. In some cases, it grows quickly and reaches the areas that are hard to access, which makes its treatment challenging.
10. Solitary Fibrous Tumour: This type of soft tissue sarcoma can occur anywhere in the body; nevertheless, it is more often seen arising from the tissues of the exterior lining of the lungs. These tumours could be both benign and malignant. Also, it is one of the slow-growing cancers.
11. Synovial Sarcoma: This cancer type is more common among children and young adults. It usually forms in the large joints, especially the knees.
12. Undifferentiated Pleomorphic Sarcoma: This is a fast-growing and rare type of soft tissue sarcoma. It usually starts in the arms and legs. This cancer type often spreads to other organs. In this type, the cancer cells grow in multiple shapes and sizes, and hence the name ‘pleomorphic’.
13. Kaposi’s Sarcoma: This type of soft tissue sarcoma forms in the blood vessels and lymph vessels. Kaposi’s sarcoma is characterised by purplish spots on the face, legs and feet. These lesions can also appear in the regions of the mouth, lymph nodes and groin. Usually, it is associated with the infection caused by the human immunodeficiency virus (HIV). This type is aggressive and will need immediate medical attention.

Symptoms

Most soft tissue sarcoma types usually begin in the leg, and the presence of a painless lump could be the first symptom of this condition. This cancer type can also form in the stomach and chest region. Soft tissue sarcomas can cause other symptoms too. If any of the following symptoms last for more than two weeks, one should immediately consult a doctor:

Presence of a lump
Coughing
Breathing difficulties
Abdominal pain that does not go away
Blood in the stool or vomit
Dark-coloured stools

Courses

There is no sufficient data on the exact causes of soft tissue sarcoma. Nevertheless, the researchers have identified a few factors that can increase one’s risk of developing soft tissue sarcoma:

Age: Although soft tissue sarcomas can affect all age groups, it is found to be more common among middle-aged and older people; the risk of developing this disease increases with age.
Certain Genetic Disorders: Having specific inherited genetic disorders, namely neurofibromatosis and Gardner syndrome, can increase your risk of developing soft tissue sarcomas.
History of Radiation Therapy: Those who have received radiation therapy for a different cancer type in the past are also at a higher risk of developing this disease.
Exposure to Harmful Chemicals: Exposure to certain harmful chemicals like phenoxyacetic herbicides, arsenic, dioxins and vinyl chlorides, etc., can increase the risk of soft tissue sarcoma.

Diagnosis

As there are various types of soft tissue sarcomas, it becomes imperative to get the diagnosis right before proceeding with the treatment. There are multiple testing methods available for the detection and diagnosis of soft tissue sarcomas:

a. Physical Exam: Before anything, the doctor thoroughly assesses the patient’s medical history for underlying medical conditions. Later, the doctor will examine the patient for the signs of soft tissue sarcoma, which could be lumps and other anomalies.

b. Imaging Tests: If sarcoma is suspected, various imaging tests may be recommended. Imaging tests, such as ultrasound scans, MRI scans, PET CT scans, etc., help doctors in obtaining detailed information, such as the size of the tumour, its exact location and its stage.

The results from tests may also be used in staging the disease, planning the treatment and also, monitoring the treatment response shown by the patient.

c. Biopsy: During the biopsy, a small portion of the suspected tissue is collected and examined under a microscope for the presence of cancer cells. A biopsy is the only way to obtain a definitive diagnosis of soft tissue sarcoma.

The biopsy sample may be obtained either by inserting a needle or through surgery.

Treatment

There are various treatment options available for the management of soft tissue sarcomas.

a. Surgery: Surgery is the main line of treatment for soft tissue sarcomas. There are different surgical procedures to support the successful treatment of soft tissue sarcomas. The doctor may recommend the following surgical procedures depending on the severity of the disease:

Minimally Invasive Surgery: For certain soft tissue sarcoma types, minimally-invasive surgeries, such as laparoscopic surgery, robotic surgery, etc., is recommended by the doctor. During these procedures, tiny incisions are made in the skin to remove the tumour. These procedures cause fewer treatment complications and support faster recovery.
Limb-Sparing Surgery: This procedure is recommended for the sarcomas of the leg and arm regions. During this procedure, the tumour-affected bone or cartilage is removed. This tissue could later be replaced with a graft. Limb sparing surgery may be combined with radiation therapy or chemotherapy in order to ensure that all sarcoma cells are destroyed.
Amputation: In rare cases, amputation is recommended as a treatment option for soft tissue sarcomas, especially those formed in the arms and legs.

b. Radiation Therapy: Radiation therapy uses high-energy radiation beams in the form of X-rays or proton beams to destroy the tumour cells. Radiation therapy may be administered before the surgery (neoadjuvant) to shrink the tumour or after the surgery (adjuvant) to destroy the residual cancer cells.

c. Chemotherapy: This treatment approach destroys cancer cells by using powerful anticancer drugs. Chemotherapy may be administered orally, intravenously or intramuscularly. It is often combined with other treatments in order to enhance the efficacy of the overall treatment. Chemotherapy is found to work well for specific types of soft tissue sarcoma.

d. Targeted drug treatment: Targeted drug therapy works by identifying the specific vulnerabilities present on the cancer cells and attacking them. Targeted therapy acts more precisely than chemotherapy and thereby helps in reducing damage to healthy tissues. This treatment approach is found to be helpful in the management of gastrointestinal stromal tumours (GISTs).

Frequently Asked Questions

1. Are soft tissue sarcomas treatable?

Yes, soft tissue sarcomas are treatable, as we have many treatment options available today. However, for the clinical outcomes to be excellent, this disease has to be diagnosed in the early stages. In the early stages, soft tissue sarcomas can be treated successfully with less complex treatment plans.

However, as the disease progresses, much more complex treatment becomes necessary and the quality of clinical outcomes may also go down. Therefore, patients must not ignore any symptoms and immediately consult a doctor if they sense something abnormal.

2. Are soft tissue sarcomas hereditary?

No, soft tissue sarcomas are not found to be hereditary. However, having certain inherited genetic disorders like neurofibromatosis, retinoblastoma, Li-Fraumeni syndrome and Gardner’s syndrome can increase the risk of soft tissue sarcoma.

3. Do soft tissue sarcomas grow fast?

This depends on the grade of the tumour. Certain soft tissue sarcomas grow faster, while some grow at a slower rate. It also depends on the patient’s overall condition.

4. Can soft tissue sarcomas spread to other organs?

Yes, as the disease advances, the sarcoma can spread to other organs, such as the lymph nodes, the lungs, the liver, the skin, etc.

5. How should I take care of myself after getting treatment for soft tissue sarcoma?

Following are the few things that soft tissue sarcoma survivors should follow:

Always keep up your follow-up appointments
Adopt a healthier lifestyle
Ensure to maintain a healthy weight
Quit tobacco consumption
Reduce your alcohol intake