Liposarcoma

Liposarcoma is a rare type of sarcoma that develops from lipocytes (fat cells). It can occur in any part of the body, but is most commonly found in the muscles of the limbs (arms and legs) or in the abdomen. Liposarcoma is the most common type of soft tissue sarcoma in adults.

Liposarcoma is more common in males, and is generally diagnosed between the ages of 40-60. However, it can develop at any age. Paediatric cases of liposarcoma are generally diagnosed in a patients’ teen years or in their early 20’s.

Types of Liposarcoma

There are four types of liposarcoma, which are classified by their cellular appearance under the microscope, and the genetic mutations involved.

Well-differentiated liposarcoma

The well-differentiated subtype is the most common type of liposarcoma. This type generally forms in the limbs or in the retroperitoneum, and is often associated with a mutation on chromosome 12. It is usually low-grade and less aggressive than other types of liposarcomas.

Dedifferentiated liposarcoma

Dedifferentiated liposarcomas are the second most common subtype of liposarcoma. It often develops as the result of a well-differentiated liposarcoma, and usually occurs in the limbs or retroperitoneum. It tends to be more aggressive than the well-differentiated subtype, and has a higher metastasis rate.

Myxoid liposarcoma

Myxoid liposarcoma is a less common form of liposarcoma. It is often the result of a genetic mutation that affects chromosomes 12-16. Myxoid liposarcomas are more common in the paediatric age groups, and usually develops in the limbs. It is usually a low-grade cancer.

Round cell liposarcoma

Round cell liposarcoma is a high-grade form of myxoid liposarcoma. This type generally forms in the leg. This subtype is generally more aggressive and may have a less favourable prognosis.

Pleomorphic liposarcoma

Pleomorphic liposarcomas are the rarest form of liposarcomas. This subtype usually develops in the limbs, and is more common in older populations. It is often high-grade with high recurrence and metastasis rates, and often spreads to the lungs. It is associated with mutations in the RB (retinoblastoma) tumour suppressor gene.

Treatment

If liposarcoma is detected, it will be staged and graded based on size, metastasis, and how the cancer cells look under the microscope. Staging and grading helps your doctors determine the best treatment for you.

Cancers can be staged using the TNM staging system:

  • T (tumour) indicates the size and depth of the tumour.
  • N (node) indicates whether the cancer has spread to nearby lymph nodes.
  • M (metastasis) indicates whether the cancer has spread to other parts of the body.

This system can also be used in combination with a numerical value, from stage 0-IV:

  • Stage 0: this stage describes cancer cells in the place of origin (or ‘in situ’) that have not spread to nearby tissue.
  • Stage I: cancer cells have begun to spread to nearby tissue. It is not deeply embedded into nearby tissue and had not spread to lymph nodes. This stage is also known as early-stage cancer.
  • Stage II: cancer cells have grown deeper into nearby tissue. Lymph nodes may or may not be affected. This is also known as localised cancer.
  • Stage III: the cancer has become larger and has grown deeper into nearby tissue. Lymph nodes are generally affected at this stage. This is also known as localised cancer.
  • Stage IV: the cancer has spread to other tissues and organs in the body. This is also known as advanced or metastatic cancer.

Cancers can also be graded based on the rate of growth and how likely they are to spread:

  • Grade I: cancer cells present as slightly abnormal and are usually slow growing. This is also known as a low-grade tumour.
  • Grade II: cancer cells present as abnormal and grow faster than grade-I tumours. This is also known as an intermediate-grade tumour.
  • Grade III: cancer cells present as very abnormal and grow quickly. This is also known as a high-grade tumour.

Once your tumour has been staged and graded, your doctor may recommend genetic testing, which analyses your tumour DNA and can help determine which treatment has the greatest chance of success. They will then discuss the most appropriate treatment option for you.

Treatment is dependent on several factors, including location, stage of disease and overall health. In adult patients, your tumour will be staged and graded to help determine the best treatment option for you. There is currently no standard staging system for children with liposarcomas.

The treatment options for adults and children with liposarcomas are similar. These options may include:

  • Surgery, potentially including:
    • Tumour resection (depending on the location).
    • Amputation (in extreme cases only).
  • Radiation therapy.
  • Chemotherapy.
  • Targeted therapy.
  • Immunotherapy.
  • Clinical trials.
  • Palliative care.

Risk factors

Some of the risk factors associated with liposarcoma include:

  • Prior radiation treatment.
  • A family history of cancer.
  • Prior damage to the lymphatic system.
  • Exposure to certain hazardous chemicals, such as vinyl chloride.
  • Certain genetic syndromes.

Not everyone with these risk factors will develop the disease, and some people who have the disease may have none of these risk factors. See your general practitioner (GP) if you are concerned.

Symptoms

In the early stages of liposarcoma, there may be no signs of the disease. As the cancer progresses, some of the following symptoms may appear.

Symptoms of liposarcoma in the limbs include:

  • A growing lump under the skin.
  • Pain and swelling in the affected region.
  • Weakness of the affected limb.
  • Numbness in the affected region.

Symptoms of liposarcoma in the abdomen include:

  • Abdominal pain, cramping and/or swelling.
  • Loss of appetite/feeling full after little food.
  • Constipation.
  • Blood in stool.
  • Black/tarry stool.
  • Unexplained weight loss.
  • Blood in vomit.

Not everyone with the symptoms above will have cancer, but see your GP if you are concerned.

Diagnosis

If your doctor suspects you have a liposarcoma, they may order the following tests to confirm the diagnosis and refer you to a specialist for treatment:

  • Physical examination.
  • Imaging tests, potentially including:
    • X-ray.
    • MRI (magnetic resonance imaging).
    • CT (computed tomography) scan.
    • PET (positron emission tomography) scan.
  • Blood tests.
  • Biopsy.

References

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