Nevoid Basal Cell Carcinomas in Gorlin Syndrome

Study Overview
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Brief Summary
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The overall aim of the project is to gain knowledge about the expected course of nevoid basal cell carcinomas and the usefulness of dermatoscopy in young individuals with Gorlin syndrome. Clinical experience and some case-reports suggest that nevoid basal cell carcinomas have an indolent course with slow growth and that they can sometimes regress. However, no systematic research has previously been performed. Increased knowledge about nevoid basal cell carcinomas and the use of dermatoscopy could be of great clinical value in the follow-up and treatment of individuals with nevoid basal cell carcinoma syndrome.

Official Title
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Nevoid Basal Cell Carcinomas in Young People With Gorlin Syndrome

Conditions
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Basal Cell Carcinoma, Gorlin Syndrome

Intervention / Treatment
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* Other: Active surveillance


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: The individual has Gorlin syndrome (clinically and/or genetically diagnosed) The individual is minimum 6 years and maximum 17 years The individual has at least three skin lesions clinically compatible with nevoid basal cell carcinomas Exclusion Criteria: The patient has a psychiatric disorder not compatible with completion of the study examinations The patient has received topical treatment (imiquimod, 5-fluorouracil, photodynamic therapy and/or cryotherapy) of nevoid basal cell carcinomas during the last three months

Ages Eligible for Study
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Minimum Age: 6 Years
Maximum Age: 17 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Gorlin syndrome<br>Children aged six to seventeen years old with Gorlin syndrome and nevoid basal cell carcinomas. | Other: Active surveillance<br>* Skin lesions clinically compatible with nevoid basal cell carcinomas are documented with measurements, clinical description, clinical photos and dermatoscopy photos every eight months for two years. Three representative skin lesions are selected at the first visit to be removed by skin biopsy at the end of the observation period (two years) for histopathological analyses. If a nevoid basal cell carcinoma shows a certain increases in size (according to pre-specified criteria of the study protocol), ulcerates, gets unevenly demarcated or give functional complaints during the observation period, the skin lesion will be excised.<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Nevoid basal cell carcinoma size increase | Largest diameter (measured in millimeter) of nevoid basal cell carcinomas after two years surveillance compared to at baseline. | Two years | 
| Dermatoscopy findings | Dermatoscopy findings of nevoid basal cell carcinomas compared to nevoid basal cell carcinoma size increase. | Two years | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Nevoid basal cell carcinoma histology | Histological analysis, including immune histochemistry, of skin biopsies obtained from representative nevoid basal cell carcinomas. | Two years | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Dermatoscopy