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WHAT IS SARCOMA?

Sarcoma is a rare type of cancer that originates in the connective tissues of the body. This includes tissues such as bones, muscles, fat, blood vessels, nerves, and deep skin tissues. Unlike carcinomas, which arise from epithelial cells, sarcomas develop from mesenchymal cells, which are responsible for forming connective tissues.

Sarcomas are broadly categorised into two main types:

Soft Tissue Sarcoma Bone Sarcoma
This type can develop in various soft tissues, including muscles, fat, blood vessels, nerves, tendons, and the lining of joints. There are many subtypes of soft tissue sarcoma, each with distinct characteristics and behaviours.

Some common subtypes include:

  • Rhabdomyosarcoma: A cancer that develops from skeletal muscle cells, often seen in children and young adults.
  • Liposarcoma: Arises from fat cells and can occur in various locations in the body.
  • Leiomyosarcoma: Develops from smooth muscle tissue, which is found in organs like the stomach and uterus.
  • Angiosarcoma: A rare type that originates from the cells lining blood vessels.
  • Gastrointestinal stromal tumour (GIST): Begins in the gastrointestinal tract, usually in the stomach or small intestine.
  • Ewing Sarcoma: Primarily affects children and young adults, often found in the bones or soft tissues.
  • Desmoplastic Round Cell Tumour: A rare and aggressive type of soft tissue sarcoma.
This type specifically arises in the bones.

Common types include:

  • Osteosarcoma: The most common type of bone sarcoma, typically affecting the long bones of the arms and legs.
  • Chondrosarcoma: Develops from cartilage cells and can occur in various locations, including the pelvis and ribs.
  • Ewing Sarcoma: Also classified as a bone sarcoma, it primarily affects children and young adults.

The symptoms of sarcoma can vary significantly based on the tumour’s location and size.

Common signs include:

  • A noticeable lump or swelling in the affected area.
  • Pain that may worsen over time, particularly if the tumour is pressing on nerves or muscles.
  • Limited range of motion if the tumour is near a joint.
  • Fractures or persistent bone pain in cases of bone sarcomas.

Sarcoma is a complex type of cancer with various risk factors that may increase an individual’s likelihood of developing the disease. While having one or more of these risk factors does not guarantee that a person will develop sarcoma, they can contribute to the overall risk. Here are some key risk factors associated with sarcoma:

  • Genetic Factors
    Inherited Syndromes: Certain genetic conditions can significantly increase the risk of developing sarcomas. These include neurofibromatosis type 1, Li-Fraumeni syndrome, retinoblastoma and tuberous sclerosis.
  • Previous Radiation Exposure
    Individuals who have undergone radiation therapy for other cancers, such as retinoblastoma, lymphoma, or breast cancer, may have an increased risk of developing sarcomas in the treated areas. This is particularly relevant for soft tissue sarcomas.
  • Chemical Exposure
    Exposure to certain toxic chemicals, such as herbicides, pesticides, and specifically Agent Orange, has been linked to an increased risk of developing sarcomas. Occupational exposure to these substances may also play a role.
  • Age and Gender
    Sarcomas can occur at any age, but certain types are more common in specific age groups. For example, osteosarcoma is often diagnosed in children and young adults, while other types, like liposarcoma, are more frequently seen in older adults. Additionally, some studies suggest that certain sarcomas may be more prevalent in one gender over the other.
  • Chronic Conditions
    Individuals with chronic conditions, such as lymphoedema (swelling due to lymphatic system blockage), may have a higher risk of developing soft tissue sarcomas.

Types

Sarcomas are broadly categorised into two main types:

Soft Tissue Sarcoma

This type can develop in various soft tissues, including muscles, fat, blood vessels, nerves, tendons, and the lining of joints. There are many subtypes of soft tissue sarcoma, each with distinct characteristics and behaviours.

Some common subtypes include:

  • Rhabdomyosarcoma: A cancer that develops from skeletal muscle cells, often seen in children and young adults.
  • Liposarcoma: Arises from fat cells and can occur in various locations in the body.
  • Leiomyosarcoma: Develops from smooth muscle tissue, which is found in organs like the stomach and uterus.
  • Angiosarcoma: A rare type that originates from the cells lining blood vessels.
  • Gastrointestinal stromal tumour (GIST): Begins in the gastrointestinal tract, usually in the stomach or small intestine.
  • Ewing Sarcoma: Primarily affects children and young adults, often found in the bones or soft tissues.
  • Desmoplastic Round Cell Tumour: A rare and aggressive type of soft tissue sarcoma.

Bone Sarcoma

This type specifically arises in the bones.

Common types include:

  • Osteosarcoma: The most common type of bone sarcoma, typically affecting the long bones of the arms and legs.
  • Chondrosarcoma: Develops from cartilage cells and can occur in various locations, including the pelvis and ribs.
  • Ewing Sarcoma: Also classified as a bone sarcoma, it primarily affects children and young adults.

Symptoms

The symptoms of sarcoma can vary significantly based on the tumour’s location and size.

Common signs include:

  • A noticeable lump or swelling in the affected area.
  • Pain that may worsen over time, particularly if the tumour is pressing on nerves or muscles.
  • Limited range of motion if the tumour is near a joint.
  • Fractures or persistent bone pain in cases of bone sarcomas.

Risk factors

Sarcoma is a complex type of cancer with various risk factors that may increase an individual’s likelihood of developing the disease. While having one or more of these risk factors does not guarantee that a person will develop sarcoma, they can contribute to the overall risk. Here are some key risk factors associated with sarcoma:

  • Genetic Factors
    Inherited Syndromes: Certain genetic conditions can significantly increase the risk of developing sarcomas. These include neurofibromatosis type 1, Li-Fraumeni syndrome, retinoblastoma and tuberous sclerosis.
  • Previous Radiation Exposure
    Individuals who have undergone radiation therapy for other cancers, such as retinoblastoma, lymphoma, or breast cancer, may have an increased risk of developing sarcomas in the treated areas. This is particularly relevant for soft tissue sarcomas.
  • Chemical Exposure
    Exposure to certain toxic chemicals, such as herbicides, pesticides, and specifically Agent Orange, has been linked to an increased risk of developing sarcomas. Occupational exposure to these substances may also play a role.
  • Age and Gender
    Sarcomas can occur at any age, but certain types are more common in specific age groups. For example, osteosarcoma is often diagnosed in children and young adults, while other types, like liposarcoma, are more frequently seen in older adults. Additionally, some studies suggest that certain sarcomas may be more prevalent in one gender over the other.
  • Chronic Conditions
    Individuals with chronic conditions, such as lymphoedema (swelling due to lymphatic system blockage), may have a higher risk of developing soft tissue sarcomas.

Diagnosis

Diagnosing sarcoma involves a combination of imaging tests and biopsies. Key diagnostic methods include:

Imaging Tests

Techniques such as MRI and CT scans are used to visualise the tumour and assess its size and location.

Biopsy

A sample of the tumour is taken to determine whether it is cancerous and to identify the specific type of sarcoma.

Treatment

Treatment for sarcoma is tailored to the individual based on the type, location, and stage of the cancer. Common treatment modalities include:

Surgery

The primary treatment for many sarcomas, aiming to remove the tumour and surrounding healthy tissue. The complexity of the surgery can vary based on the tumour’s size and location.

Radiotherapy

Often used in conjunction with surgery to eliminate remaining cancer cells.

Chemotherapy

May be employed, particularly for certain types of sarcomas, to target cancer cells throughout the body.

Prevention

There is no definitive way to prevent sarcoma; adopting a healthy lifestyle by quitting smoking and minimising exposure to harmful chemicals may help reduce risk.

For individuals with a family history of sarcoma or genetic disorders that increase the risk (such as Li-Fraumeni syndrome or neurofibromatosis), genetic counselling may be beneficial. This can help assess personal risk and discuss potential monitoring strategies.

While there are no specific screening techniques for sarcoma, being aware of any unusual lumps or swelling and discussing them with a healthcare provider can lead to earlier detection and treatment. Regular check-ups can help monitor any changes in health.

Prognosis

The prognosis for sarcoma varies significantly based on several factors, including the type of sarcoma, its stage at diagnosis, the location of the tumour, and the overall health of the patient. Patients should engage in discussions with their healthcare providers to understand their unique situation and treatment options.

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