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Sarcoma

Dr Roger Henderson
Reviewed by Roger HendersonReviewed on 29.04.2024 | 5 minutes read
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Sarcoma is a rare cancer affecting any part of the body. The most common type are soft tissue sarcomas which develop in muscle, nerves, fatty tissue and blood vessels. The other types are bone sarcoma and gastrointestinal stromal tumours.

Around 3,000 people a year in the UK are diagnosed with sarcoma, making it a rare cancer, and it affects men and women equally.

Types of Sarcomas

Bone Sarcomas

Bone sarcomas are a diverse group of malignancies that originate from bone tissue, comprising several distinct subtypes with varying characteristics and treatment approaches. The main types of bone sarcomas include osteosarcoma, Ewing sarcoma, and chondrosarcoma.

Osteosarcoma is the most common type of primary bone cancer, typically arising in the long bones of the arms and legs. It predominantly affects children and young adults, with a peak incidence during adolescence. Osteosarcoma often presents as a painful swelling or mass near the affected bone and may be associated with pathological fractures. Diagnosis is confirmed through imaging studies, such as X-rays, CT scans, or MRI, followed by biopsy for histological examination. Treatment typically involves a combination of surgery, chemotherapy, and sometimes radiation therapy, aimed at achieving local control and preventing metastasis.

Ewing sarcoma is a rare type of bone cancer that primarily affects children and young adults. It commonly arises in the pelvis, femur, or ribs, although it can occur in any bone. Ewing sarcoma is characterised by aggressive growth and a propensity for metastasis to other bones or organs. Symptoms may include pain, swelling, and occasionally, fever. Diagnosis involves imaging studies and biopsy for histological analysis. Treatment usually consists of chemotherapy, surgery, and/or radiation therapy, depending on the extent of the disease and the presence of metastasis.

Chondrosarcoma is a malignant tumour that arises from cartilage cells within bones. It typically occurs in older adults and commonly affects the pelvis, femur, or shoulder. Chondrosarcoma is characterised by slow growth and a tendency to recur locally, although it has a lower risk of metastasis than osteosarcoma and Ewing sarcoma. Diagnosis is established through imaging studies and biopsy. Treatment involves surgical resection of the tumour, with adjuvant therapy such as chemotherapy or radiation therapy in some cases.

Soft Tissue Sarcomas

Soft tissue sarcomas are a diverse group of cancers that arise from various soft tissues in the body, including muscles, fat, nerves, blood vessels, and connective tissues. These tumours encompass a wide range of subtypes, each with unique histological, molecular, and clinical features.

Liposarcoma is the most common type of soft tissue sarcoma, arising from fat cells (adipocytes). It typically occurs in the extremities, retroperitoneum, or trunk. Liposarcoma is classified into several subtypes, including well-differentiated, dedifferentiated, myxoid, and pleomorphic variants, each with distinct characteristics and prognosis.

Leiomyosarcoma originates from smooth muscle cells and can arise in various locations, including the uterus, gastrointestinal tract, and soft tissues of the extremities. It is characterised by aggressive growth and a propensity for metastasis.

Fibrosarcoma arises from fibroblasts, which are cells that produce collagen and other structural proteins in connective tissue. It commonly affects the extremities, trunk, or head and neck region. Fibrosarcoma is characterised by a high recurrence rate and potential for metastasis.

Synovial sarcoma is a rare soft tissue sarcoma that typically arises near joints, especially in the extremities. Despite its name, it does not originate from synovial tissue. Synovial sarcoma is characterised by a unique chromosomal translocation and tends to affect young adults. It has a propensity for local recurrence and metastasis.

What symptoms should I look out for?

Sarcomas can cause a lump on the skin, which may or may not be painful, bone pain or an unexpected fracture with minimal injury. Symptoms can develop close to the site where the sarcoma is growing- for example abdominal pain near the abdomen, breathing problems and coughing near the lungs. Generalised symptoms like fatigue and unintentional weight loss can also occur.

What causes a sarcoma?

Like most cancers, sarcoma forms when there are mutations in the DNA of cells, causing abnormal cells to grow and divide uncontrollably. The reason this happens is unknown but there are certain things that increase your risk, such as genetic conditions, previous exposure to radiation from radiotherapy as well as exposure to chemicals such as industrial chemicals.

Angiosarcoma occurs often in places where the lymphatic system has been blocked.

Kaposi’s sarcoma originates from blood vessel walls and forms purple painless spots on the legs, feet or face. It has different causes, depending on the subtypes, including certain ethnicities, immune suppression including those with AIDS, and it’s more common in people with human herpes virus 8 (HHV-8), a sexually transmitted infection.

What can the doctor do?

Your doctor will ask you questions about your symptoms and other health conditions and examine you. They may arrange further investigations, such as a scan, or they may refer you urgently to a specialist.

Sarcomas are often difficult to spot - the presentation is not always clear-cut and can sometimes take some time to arrive at. The most appropriate imaging depends on the location and type of any suspected sarcoma. If there is a bone fracture, an X-ray is the usual place to start.

Soft tissue lumps may require ultrasound scans, and later an MRI, for a more detailed view.

At some point, a suspicious mass will require a sample (biopsy) to be taken for lab analysis of the cell type and confirm whether it is cancerous.

What is the treatment?

Treatment depends on the location, the type and how aggressive the sarcoma is, and also if it’s spread to other areas. Surgery is the mainstay of treatment to remove the bulk of the cancer cells, if not all. Then accompanying treatments like radiotherapy or chemotherapy can be used before or after to stop the cells from growing and dividing.

Almost 55% of people survive their cancer for 5 years or more, and 45% for ten years-plus.

Sarcoma UK website provides great resources for people suffering from and caring for those with sarcoma.

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Dr Roger Henderson
Reviewed by Roger Henderson
Reviewed on 29.04.2024
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