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Improving outcomes for people with skin tumours including melanoma

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Genetic predisposition to MM may also occur, <strong>with</strong> approximately<br />

1–5% of patients <strong>with</strong> MM having a family history. Rare high-risk<br />

genes occur that are inherited as autosomal dominants, and these may<br />

manifest as multiple primary <strong>tumours</strong> in an individual and/or<br />

clustering in families. Most families <strong>with</strong> these genes in the UK are at<br />

increased risk of MM alone. In other countries there also appears to<br />

be an increased risk of pancreatic cancer.<br />

<strong>Improving</strong> Outcomes <strong>for</strong><br />

People <strong>with</strong> Skin Tumours<br />

<strong>including</strong> Melanoma<br />

Management of special<br />

groups<br />

The incidence of MM in the UK is around 10 per 100,000 per annum.<br />

Allowing <strong>for</strong> 80% survival and a mean age of 51 at diagnosis, an<br />

estimated population prevalence is around 1 in 10,000. The majority<br />

of these will have high-penetrance genes resulting in a lifetime risk of<br />

developing MM of between 60% and 80%.<br />

In the general UK population, individuals <strong>with</strong> multiple moles (the<br />

atypical mole syndrome [AMS]) are at increased risk of MM and this is<br />

thought to be genetic, probably due to low-penetrance susceptibility<br />

genes. The phenotype is, however, common and patients <strong>with</strong> the<br />

AMS require education about prevention, both primary (sun<br />

avoidance) and secondary (signs and symptoms). Patients <strong>with</strong> AMS<br />

have a relative risk of MM of around 10 compared <strong>with</strong> those who<br />

have very few moles. The lifetime risk of MM in the UK is<br />

approximately 1 in 150; patients <strong>with</strong> AMS have an estimated 1 in 20<br />

lifetime risk compared <strong>with</strong> a person <strong>with</strong> an average number of<br />

moles. Their risk is lower when compared, <strong>for</strong> example, <strong>with</strong> those<br />

<strong>with</strong> XP, but as 2% of the general population have the AMS these<br />

patients ‘explain’ a significant proportion of the disease.<br />

6<br />

Organ transplant patients<br />

There is epidemiological evidence that patients who have had an<br />

organ transplant are at high risk of developing all types of <strong>skin</strong><br />

cancers as a result of long-term immunosuppression. The risk<br />

increases <strong>with</strong> time following the transplant and is higher in older<br />

patients and white-<strong>skin</strong>ned <strong>people</strong> who have had excessive sun<br />

exposure. These patients are especially at risk of developing SCC of<br />

the <strong>skin</strong> and often have multiple and fast-growing <strong>tumours</strong>, which<br />

may pose difficulties in their management. As organ transplant<br />

recipients live longer, so the prevalence of <strong>skin</strong> <strong>tumours</strong> <strong>with</strong><br />

metastases in this population will increase.<br />

Guidance on cancer services: <strong>skin</strong> <strong>tumours</strong> <strong>including</strong> <strong>melanoma</strong><br />

113

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