Sarcoma: Causes, Symptoms, and Treatment

What Is Sarcoma?

Sarcoma is the general term used for a broad group of cancers that affects the bones and soft tissues. Soft tissue sarcomas can affect any tissue that connects, support or protect other organs of the body such as the skin, blood vessels, connective tissues, lining of the joints, muscles and fat. 

What Is the Prevalence of Sarcoma?

Sarcomas are more common in children as compared to adults. According to the American Society of Clinical Oncology, sarcomas account for 15% of cancers in children as compared to only 1% of the adult cancers. Its prevalence in the Indian population is estimated to be about 0.9%. Studies carried out in the Indian population suggests sarcoma to most commonly affect children between 10-14 years of age (4.1 per million population).

What Are the Various Types of Sarcomas?

There are more than 70 different types of sarcoma that can affect humans, including

  • Leiomyosarcoma: This type of sarcoma affects the smooth muscle tissues, such as the abdomen, uterus, blood vessels and the skin.

  • Liposarcoma: This type of sarcoma occurs in the fatty tissues. Liposarcoma generally originates from the back of the knee, thighs, or the back of the abdomen.

  • Fibrosarcoma: Fibroblasts are the cells of the connective tissues. Fibrosarcoma develops in these fibroblasts.

  • Kaposi’s Sarcoma: Kaposi Sarcoma may affect any part of the body but usually occurs on the skin. It is caused due to herpesvirus 8 infection, also called Kaposi's sarcoma-associated herpesvirus.

  • Chondrosarcoma: Chondrosarcoma develops in the cartilage-producing cells of the bone. It constitutes approximately 30% of the skeletal system cancers.

  • Ewing Sarcoma: Usually affects the young population between the age of 10 to 20 years. This sarcoma develops in the bones or the tissues surrounding the bones.

  • Osteosarcoma: It occurs in the bone-forming cells resulting in immature bone formation. Males are more affected as compared to females.

What Are the Signs and Symptoms?

Symptoms of sarcoma depend upon the site of occurrence and its severity. 

Signs and symptoms of soft tissue sarcoma:

  • Presence of lump and swelling which is usually painless

  • Pain if the tumour affects nerves, muscles, or surrounding tissues

  • A gastric tumour may lead to blood in the stools

  • Abdominal pain

  • Weight loss

Signs and symptoms of osteosarcoma:

  • Pain in the bones which tends to worsen at night.

  • Weak and brittle bones that may break with a small injury

  • The patient may limp if the tumour affects the bone of one of the legs

  • Bone swelling

What Are the Causes of Sarcoma?

Although the exact cause of sarcoma is not known certain factors increase the risk of its occurrence. These include:

  • Radiation therapy: Radiation therapy used in the treatment of cancer 

  • Chemical Exposure: Exposure to various chemicals such as dioxins, and vinyl chloride 

  • Viral infection: Viral infection caused by Human Herpesvirus 8 

  • Genetic syndromes: Various inherited syndromes such as familial retinoblastoma may be passed on to the children and increase the risk of sarcoma

How Is Sarcoma Diagnosed?

There is no standard screening test for sarcoma available till date. 

A diagnosis is usually confirmed by a doctor through the following techniques:

  • Physical examination: The doctor will perform a comprehensive evaluation for any lump or swelling and ask the patient about the signs and symptoms. 

  • Imaging techniques: Imaging techniques such as Ultrasound, X-ray, PET scan, CT scan, bone scan, and MRI are usually recommended or sarcoma diagnosis

  • Biopsy: A small part of the suspicious tissue is taken and analyzed in the laboratory. A biopsy helps to determine the presence and extent of cancer.  

  • Genetic testing: Patients who have a strong history of soft tissue sarcoma in their family are at a higher risk of sarcoma. For these patients, genetic testing plays a major role in early screening. Genetic testing is a procedure in which mutations in a particular gene are identified using modern molecular technologies.

What Are the Treatment Options for Sarcoma?

Treatment for sarcoma includes:

  • Surgery: Surgery is done to remove cancer cells. Surgery may be accompanied by radiation therapy or chemotherapy. Small low-grade sarcomas can be managed with surgery alone.

  • Chemotherapy: Various drugs are administered to the patients that kill cancer cells. It may be used in combination with radiation therapy or surgery. 

  • Radiation Therapy: High energy beams of X-rays or photons are guided onto the cancer cells to kill them.

  • Immunotherapy: Immunotherapy helps target the immune cells to fight against the cancerous cells.  

  • Targeted Therapy: Targeted therapies involves drugs that directly target the cancerous cells without affecting the healthy cells.

  • Ablation Therapy: In ablation therapy, the cancer cells are killed either by heat generation through electricity or freezing the cells using cold liquid.

What Are the Different Sarcoma Stages and What Are the Treatment Options Available?

  • Stage 1: Early-stage, where the cancer is restricted to the tissue of origin. Surgery ± Radiation therapy is usually the choice of treatment.  

  • Stage 2: High grade, where cancer can grow quickly and spread to other tissues. Surgery + Radiation therapy is usually used to treat patients in this stage. 

  • Stage 3: Cancer is high grade and larger in this stage. Surgery + Radiation ± Chemotherapy is usually required to stop its further growth. 

  • Stage 4: Also known as the metastatic stage, cancer spreads to other parts in the body. The treatment plan usually includes medications and palliative care.

 

Dr. Poonam Patil 

Consultant – Medical Oncology

Manipal Hospitals Bangalore – Old Airport Road

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