Improving outcomes for people with skin tumours including melanoma

Improving Outcomes for
People with Skin Tumours
including Melanoma
Management of special
groups
Management of
special groups
Introduction
Patients who are immunocompromised or who have a genetic
predisposition are at increased risk of developing skin cancers. They
are likely to develop precancerous lesions and cancers at a younger
age and to develop multiple primaries. These patients and others with
rare skin tumours (Appendix 1) may need more investigations, more
intensive, earlier follow-up and specific, supportive care and
information. Another group with special additional needs are children
and young people with ‘adult-type’ skin cancers.
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Genetic predisposition
There are a number of genetic conditions associated with the
development of multiple skin cancers earlier in life than in the general
population. The most common are described below.
The commonest genetic condition that predisposes to BCC is the basal
cell naevus syndrome (Gorlin’s syndrome), which is estimated to
occur in 1 in 57,000 of the population. This is inherited as an
autosomal dominant condition with a high spontaneous mutation rate.
Affected individuals often have bony anomalies and dental cysts. In
adult life they are at increased risk of BCC although BCCs can
develop at very much younger ages. This risk is increased by
exposure to irradiation, which must therefore be avoided. Sun
avoidance throughout life should be promoted to prevent skin cancer.
Xeroderma pigmentosum (XP) is a rare autosomal recessive disorder.
The estimated frequency is one case per 250,000 population in the
United States and Europe. Within the first few years of life, BCC, SCC,
sarcomas and MM start to develop if sun protection does not occur.
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National Institute for Health and Clinical Excellence