Basal Cell Carcinomas

90% of individuals with the syndrome will develop basal cell carcinomas (BCC) at some stage in their life.

BCCs is the most common form of cancer in the general population. BCCs in members of the general population are associated with excess exposure to the sun, and therefore appear on the sun exposed parts of the body, usually the face. In Gorlin syndrome the BCCs can also occur on other areas of the body which have not been exposed to the sun.

Brownish/pink/orange basal cell nevi may occur in early childhood and may lie quiescent (dormant) without aggressive behaviour. The histologic appearance is that of a typical BCC which, when excised, can be the first unexpected finding of Gorlin syndrome in simplex cases (affected individuals with no family history of Gorlin syndrome, especially children.

Active BCCs may grow from existing basal cell nevi that may be numerous or typical BCCs may appear from virtually blemish free skin. BCCs may also crust, bleed, ulcerate or may present as a localised infection.

BCCs can occur in early childhood but in general do not present until the late teens or early adulthood. They occur more frequently with age, although 10% of individuals with Gorlin syndrome never develop a BCC. Individuals with type 1 skin (white skin that burns but never tans) and individuals with excessive ultraviolet light exposure seem especially prone to developing large numbers of BCCs.

The areas around the eyes, nose, cheek bones and the upper lip are the most frequently afected sites on the face. Usually only a few become aggressive, when they are locally invasive and behave like ordinary BCCs. Evidence of aggressive transformation of an individual lesion includes an increase in size, ulceration, bleeding, or crusting. Some patients can develop aggressive BCCs without first developing naevi.

Ref: Neviod Basal Cell Carcinoma Syndrome. Synonyms: Basal Cell Nevus syndrome (BCNS), Gorlin syndrome, NBCCS. D Gareth Evans, MD, FRCP and Peter A Farndon, MD, FRCP Gene Reviews 20301330 Mar 2013

To see full article see Gene Reviews

Further information about BCCs can be accessed – courtesy of British Association of Dermatologists

Remember, BCCs are curable – Recognise the early warning signs.

	BCCs frequently appear as an open sore or ulcer. It may bleed or crust, but does not heal.
	BCCs can look like a red patch on the skin which may be itchy, painful or crusty. Sometimes no symptoms are felt but the lesion does not heal or fade.
	A smooth raised growth can appear with an ulcer in the centre. These BCCs can be flesh coloured, pink, red or brown like a mole.
	BCCs can also look like a firm nodule in the skin. These too can appear flesh coloured, pink, shiny, red or pigmented like a mole.
	BCCs can take on the appearance of a flat scarred area in the skin. This area appears pale or white compared to the surrounding skin and may have an ulcer or indentation in the centre. This form of BCC can grow more quickly, making the affected skin look taught and shiny.