Soft Tissue Sarcoma

1 What is Soft Tissue Sarcoma?

A soft tissue sarcoma is a rare form of cancer that develops in the soft tissues of your body, such as muscle, fat, blood vessels, nerves, tendons and the lining of your joints.

Soft tissues connect, support and surround other body structures. Of the different types, some soft tissue sarcoma is common in children while some in adults.

Although soft tissue sarcoma can develop in any parts of the body, it mostly affects abdomen, arms, and the legs.

2 Symptoms

Usually, no signs and symptoms are experienced at an early stage of soft tissue sarcoma. The symptoms may appear as sarcoma becomes advanced.

You may experience following signs and symptoms:

  • An evident swelling
  • Pain, usually experienced when excessive growth compresses nerves or muscles.

Seek medical attention if you have troublesome signs and symptoms.

3 Causes

In most of the cases, the cause of soft tissue sarcoma is not clear. Genetic alteration in DNA (mutation) of cells results in abnormal growth and production of cells.

Out-of-control cell growth causes tumors that can spread to either neighboring organs or other parts of the body.

Soft tissue carcinomas are classified on the basis of the types of cells that mutate.

The different types of soft tissue carcinomas are:

  • Alveolar soft part sarcoma
  • Angiosarcoma
  • Clear cell sarcoma
  • Dermatofibrosarcoma protuberans
  • Desmoplastic Small round cell tumor
  • Epitheloid hemangioendothelioma
  • Epithelioid sarcoma
  • Extrarenal rhabdoid tumor
  • Fibrosarcoma
  • Gastrointestinal stromal tumor
  • Hemangiosarcoma
  • Infantile fibrosarcoma
  • Inflammatory myofibroblastic tumor
  • Kaposi's sarcoma
  • Leiomyosarcoma
  • Liposarcoma
  • Lymphangiosarcoma
  • Malignant fibrous histiocytoma
  • Malignant peripheral nerve sheath tumor
  • Myofibrosarcoma,
  • Myxofibrosarcoma
  • Neurofibrosarcoma
  • Perivascular epitheliod cell tumor (PEComa)
  • Rhabdomyosarcoma
  • Synovial sarcoma
  • Undifferentiated, unclassified soft tissue sarcomas

4 Making a Diagnosis

If soft tissue sarcoma is likely to be the cause of your symptoms, you may be referred to oncologist to receive a diagnosis.
Getting prepared for the visit can optimize the therapy and help make the visit more fruitful. List out all the symptoms. Write down your key medical information.

Write down the names of all your medications, vitamins or supplements. Ask a friend or a family member to accompany you during the visit. Make a list of the questions to ask your doctor.

Some typical questions can be:

  • Do my symptoms indicate cancer?
  • Are there other possible causes for my symptoms?
  • Are there any confirmatory tests that I need?
  • What type of sarcoma do I have? What stage is it?
  • What are my treatment options and side effects of each option?
  • Is there any surgical procedure that can remove cancer?
  • What's my prognosis?
  • Should I get additional treatments such as chemotherapy or radiation therapy either before or after an operation?
  • Is the surgeon experienced in this specific type of cancer operation?
  • What your does doctor want to know?

A clear talk with your doctor can optimize the therapy and improve the outcomes. Prepare yourself to answer some essential questions from your doctor.

Your doctor might ask you typical questions like:

  • When did you first notice your signs and symptoms?
  • Are you experiencing pain?
  • Does any factor improve or worsen your symptoms?
  • Do you have any family history of cancer?
  • If so, do you know what type of cancer?

Your doctor is likely to perform following tests for diagnosis of soft tissue sarcoma:

Imaging tests: Imaging tests such as X-rays, computerized tomography (CT) scans, magnetic resonance imaging (MRI) and positron emission tomography (PET) may be recommended to look for the area where sarcoma is suspected of developing.

Biopsy: A sample of tissue from the affected area is removed and analyzed in the laboratory. The biopsy sample is usually obtained by using long, thin needle. A biopsy sample may be collected while your doctor is performing surgery.

The biopsy sample is then analyzed by the pathologist (the specialist who identifies the disease by studying body tissues) to diagnose soft tissue sarcoma.

5 Treatment

The selection of treatment for your soft tissue sarcoma depends on upon the size, type and location of your cancer.

Surgery

Soft tissue sarcomas along with some healthy neighboring tissues may be are removed surgically. Arm or leg, if affected by sarcoma, may need to be cut off.

However, your doctors will put all their efforts to prevent amputation. For instance, reducing the size of the tumor by chemotherapy and radiation may help to avoid amputation.

The sarcoma that has spread to other parts of the body may be taken out by surgical means. 

Radiation therapy

It uses ionizing radiations like X-rays to destroy cancer cells. Radiation therapy, when given prior to surgery, reduces tumor size and facilitates surgery.

Radiation given after surgery helps to kill cancer cells that might have survived the surgery.

Chemotherapy

The chemicals used in chemotherapy kill the cancer cells. Chemotherapy can be given either orally (pills) or intravenously (injections).

Out of different types of soft tissue carcinoma, some forms of carcinoma may be susceptible to chemotherapy. For example, rhabdomyosarcoma is responsive to chemotherapy.

Targeted drug therapy

Targeted drugs, as the name suggests, target specific abnormality in sarcoma cell, unlike the chemotherapy drugs.

Targeted drugs that may be used for the treatment of gastrointestinal stromal tumor are imatinib, sunitinib, regorafenib, and pazopanib. Many new targeted drugs are being studied.

6 Lifestyle and Coping

Lifestyle modifications are necessary in order to cope with soft tissue sarcoma.

Being diagnosed with soft tissue sarcoma can be a devastating and life-changing event.

Following measures may help you to deal with the stress and anxiety:

  • Learn about soft tissue sarcoma: Keep yourself updated on Soft tissue sarcoma. Knowing more about sarcoma can boost your confidence and help to make appropriate decisions for your treatment.
  • Stay close to family and friends: Talk to people who are ready to listen and help you.
  • Join support groups: Join a support group in your community or online. Being with people who are similar to you may give you a better insight of life and help you maintain a positive outlook towards your condition.

7 Risks and Complications

There are several risks associated with soft tissue sarcoma, which include:

  • Inherited syndromes: You are more likely to develop soft tissue sarcoma if you have certain hereditary syndromes, such as hereditary retinoblastoma, Li-Fraumeni syndrome, familial adenomatous polyposis, neurofibromatosis, tuberous sclerosis and Werner syndrome.
  • Chemical exposure: Exposure to toxic chemicals, such as herbicides, arsenic, and dioxin, is associated with higher risk of developing soft tissue carcinomas.
  • Radiation exposure: If you have previously undergone radiation therapy for other cancers, you are at an increased risk of having soft tissue sarcoma.

8 Related Clinical Trials

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