IT is perhaps easier to grasp the concept of a cancer when it is preceded by the name of the organ in which it originates.

The terms lung cancer or breast malignancy are fairly unambiguous, and thanks to a vast media drive, the majority of us are aware not only of the symptoms, but that seeking urgent medical help is the utmost priority.

Yet a recent poll showed that only one in four individuals had an understanding of the word sarcoma, the umbrella term to describe primary, malignant tumours arising from the connective tissues of the body. The connective tissues support the organs in their respective positions allowing them to function in a coordinated manner.

The connective tissues can further be split into soft tissues, these being blood vessels, cartilage, fat, muscles, nerves, tendons and ligaments, and a separate group; bones.

As the 21st commonest tumour, it is easy to see why sarcomas have been relegated in pecking order. In the UK, there are on average just over 4,500 soft tissue sarcoma diagnoses per year, and just under 700 for bone sarcomas.

It is estimated that the average general practitioner will see one to two in an entire career.

Yet as primary and malignant, with the potential for local damage and spread to other organs, these are indeed serious conditions, which we should ideally be aware of.

Sarcomas are classified according to the tissue type they arise from, for example an osteosarcoma denotes that developing in a bone, whereas myosarcomas are muscle tumours. The perhaps well-known Kaposi’s sarcoma, associated with advanced HIV infection, is a cancer due to increased production of endothelial cells lining blood vessels, and shows as purple welts on the skin.

The biggest risk factor for soft tissue sarcoma is advancing years, whereas bone sarcomas are commoner in children and young adults, making up 4 per cent of childhood cancers up to age 14.

Both soft tissue and bone sarcomas primarily affect the limbs. Just under two thirds of all sarcomas occur in a leg or arm. They may also involve the abdomen. A Gastrointestinal Stromal Tumour (GIST) is that which affects the connective tissue of the stomach. Around 15 per cent of sarcomas are found in the head and neck region.

The classical description of a sarcoma is a slow growing lump. The size of a golf ball has been arbitrarily used as a yard stick for concern, yet I would encourage anyone with a new lump to get it checked out. Unlike benign (non-cancerous) lumps, sarcomas may not be easy to move around with your hand, having attached themselves to underlying structures. Pain may be caused by the tumour pressing on surrounding nerves. At this point it would be relevant to point out that the majority of lumps and bumps will not be cancerous, but it pays to know your own body, and to recognise any changes, hopefully without becoming fixated on the worst-case scenario.

Suspected sarcomas will be fast tracked to a specialist centre. It has long been acknowledged that the only way to ensure the best outcomes is if high volumes of cases are seen in dedicated centres. As sarcomas are rare, this may mean a significant journey for some individuals.

Management begins with a thorough history and examination and radiological imaging. The lump itself may be best seen with ultrasound, whereas CT will look for spread. A biopsy, usually done under local anaesthetic is vital to classify the cells of origin of the tumour. This is known as the grade. Stage describes spread both at the original site as well as to other parts of the body.

Multidisciplinary teams are honed in providing the absolute best care, tailored to each individual circumstance. Surgery is seen as the definitive treatment (I hasten to use the word curative) removing both the cancerous tissue and a rim of healthy tissue to ensure proper clearance. Radiotherapy and chemotherapy are used as an adjunct to surgery either pre procedure to shrink the tumour, or post operatively to reduce the chance of recurrence. They may be used individually or in combination where surgery is not an option, or less suitable.

One of the most difficult aspects of sarcoma treatment is that for some patients, treatment may result in appearance and life changing outcomes. That affecting the head and neck may be very visible, and the loss of a limb will always be a difficult hurdle, both emotionally as well as physically.

Psychological support is available throughout, with many sarcoma patients followed up for 10 years, compared to the usual 5 for other cancers.