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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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Gorlin syndrome | Children aged six to seventeen years old with Gorlin syndrome and nevoid basal cell carcinomas. |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Active surveillance | Other | 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. |
| 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 |
| 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 |
| Measure | Description | Time Frame |
|---|---|---|
| Clinical manifestations associated with nevoid basal cell carcinomas | Presence of congenital anomalities and benign neoplasias (odontogenic keratocysts, other cysts, fibromas) in patients with nevoid basal cell carcinomas. | Two years |
| Mutation burden in nevoid basal cell carcinomas analyzed by next-generation sequencing |
Inclusion Criteria:
Exclusion Criteria:
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Children aged six to seventeen years with Gorlin syndrome and nevoid basal cell carcinomas living in Norway.
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| Name | Affiliation | Role |
|---|---|---|
| Kristin Hortemo, MD, PhD | Oslo University Hospital | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Oslo University Hospital | Oslo | 0424 | Norway |
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| ID | Term |
|---|---|
| D002280 | Carcinoma, Basal Cell |
| D001478 | Basal Cell Nevus Syndrome |
| ID | Term |
|---|---|
| D002277 | Carcinoma |
| D009375 | Neoplasms, Glandular and Epithelial |
| D009370 | Neoplasms by Histologic Type |
| D009369 | Neoplasms |
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| ID | Term |
|---|---|
| D057832 | Watchful Waiting |
| ID | Term |
|---|---|
| D017063 | Outcome Assessment, Health Care |
| D010043 | Outcome and Process Assessment, Health Care |
| D011787 | Quality of Health Care |
| D006298 | Health Services Administration |
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Skin biopsies.
|
Next-generation sequencing of DNA extracted from skin biopsies obtained from representative nevoid basal cell carcinomas, including genes described in skin carcinomas. |
| Two years |
| D018295 |
| Neoplasms, Basal Cell |
| D009807 | Odontogenic Cysts |
| D007570 | Jaw Cysts |
| D001845 | Bone Cysts |
| D003560 | Cysts |
| D009386 | Neoplastic Syndromes, Hereditary |
| D001848 | Bone Diseases, Developmental |
| D001847 | Bone Diseases |
| D009140 | Musculoskeletal Diseases |
| D007571 | Jaw Diseases |
| D009057 | Stomatognathic Diseases |
| D000015 | Abnormalities, Multiple |
| D000013 | Congenital Abnormalities |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D030342 | Genetic Diseases, Inborn |