The Gorlin Syndrome Group, campaigning to help people with a rare genetic skin cancer, today launched new research highlighting the significant impact of multiple surgeries on patients' wellbeing.
The survey, presented today at the European Association of Dermato Oncology (EADO) Congress, revealed that almost half (48%) of patients endure more than 20 surgical procedures although only 15% are offered counselling throughout diagnosis and treatment.1 Furthermore, 51% of people undergo surgery every few months to manage their condition, with 49% saying these surgeries have a significant emotional impact.1
The research, conducted in people with Gorlin syndrome, which causes highly visible skin tumours,2 was published by the Gorlin Syndrome Group to raise awareness of the need for greater patient support.
The findings are particularly significant as 97% of patients undergo surgery to remove tumours, with 86% of patients suffering from tumours on their face, 60% on their arms or legs, and 52% on their chest.1
"Quality of life is a real issue for people with this disease, not least because it requires such frequent surgery, as well as regular hospital check-ups" said Margaret Costello, Co-founder, Trustee and Secretary, Gorlin Syndrome Group. "While emotional wellbeing is a core part of cancer care elsewhere, it is being unfairly overlooked in this group of people and must be addressed. We hope this research further improves the understanding of this condition and leads to greater support for people with this life-long genetic condition."
Gorlin syndrome, also known as nevoid basal cell carcinoma (BCC), is a genetic disorder that affects 1 in 30,000 people in the UK, which causes multiple BCCs in all those with the condition, as well as nearly 100 other features including jaw cysts and skeletal abnormalities.2, 3
Dr John Lear, Consultant Dermatologist, Central Manchester University Hospitals and lead study author said: "This condition has a profound impact on patients' physical and emotional wellbeing, often causing hundreds of skin tumours across a person's lifetime. More support is needed to provide the treatment these people need; it is simply not enough to treat the physical symptoms alone."
About the survey
The survey was conducted from 20 March to 30 April 2013 and completed by 67 Gorlin syndrome patients and 6 on behalf of a patient by a family member or friend with Gorlin syndrome.1
About Gorlin Syndrome
The condition is characterised by the development of multiple jaw cysts (keratocysts) and/or basal cell carcinomas (BCCs).3
Most individuals have palmar and plantar pits (small despressions) on the palms of the hands and the soles of the feet and skeletal anomolies such as bifid ribs (ribs that are divided at the end), or wedge-shaped vertebrae.3
Other features occur in the syndrome, although most are rare but these can include ophthalmic problems, cleft lip and palate, ovarian fibromas (a fibroma is a benign tumour), cardiac fibromas which can affect heart function and medulloblastoma (type of brain tumour).3
There are nearly 100 features of the syndrome, some of which are described under specific headings within the website, these can be accessed via the main menu.3