Survey
* Your assessment is very important for improving the workof artificial intelligence, which forms the content of this project
* Your assessment is very important for improving the workof artificial intelligence, which forms the content of this project
Basal Cell Nevus Syndrome Daniel Berg M.D., FRCPC Director, Dermatologic Surgery University of Washington Thank Goodness….. Shade at Last! Basal Cell Nevus Syndrome • Autosomal Dominant –50% risk of passing on • In the skin: –Numerous Basal Cell Carcinomas • Beginning at young age • Sensitivity to Radiation Treatment –Palmar Pits BASAL CELL CARCINOMA (BCC) • Commonest Cancer U.S. 800,000/yr – 99% in Caucasians – 95% between age 40-79 – 85% on Head & Neck – Risk of Metastasis: Very Very Low – Main potential problem: Local Invasion EPIDEMIOLOGY LIFETIME RISK OF BCC AND SCC MEN: 18.6% WOMEN: 18% (based on B.C. data - lifespan 75 yrs.) BCNS Time of Onset BCC • Before puberty: • By age 22: 15% 50% • By age 35: 90% • None over age 30: 10% Remember this? P M •DNA molecules make up genes •Genes are blueprints for Proteins •Proteins are the building blocks of body functions •Some proteins control cell growth D •Everyone has two copies of each gene •One each from Mum and Dad Tumor Suppressors Proteins that normally act as brake on cell growth. P Patched Inhibits Induces Smo Downstream Target Genes Growth Patched P Normal Cell P Cell at Risk BCC Cell UVB Ultraviolet Light Spring Break - circa 1900 BASAL CELL CARCINOMA • CLINICAL PRESENTATION • • • • Nodular Superficial Morpheaform Pigmented Nodular Superficial Pigmented Morpheaform Infiltrative NonMelanoma Skin Cancer Choice of Treatment Balance: CURE RATE FUNCTIONAL RESULT COSMETIC RESULT Choice of Treatment • Special Features in BCNS Patients: – Numerous BCCs expected • Save more complicated surgery • Early detection more important – Size – Consequences if recurrence – Pathology – Patient Concerns Treatments • Topical – 5FU (Effudex) • Superficial only – Imiquimod (Aldara) • Just approved by FDA 2004 • Surgery – ED&C (scrape and burn) – Excision • Mohs • Regular Treatments • Radiation – Not in BCNS • Other – PDT ED & C (“scrape & burn”) CURE FOR SMALL PRIMARIES >90% • ADVANTAGES – Inexpensive – Outpatient Office Procedure – Quick • DISADVANTAGES – High Recurrence Rate for Difficult Tumors • Location, recurrent, deep ED&C Initial Lesion (BCC) Curettage (after biopsy) ED&C Desiccation Repeat X 3 Final Defect ED&C Typical Scar SURGICAL EXCISION CURE FOR PRIMARY TUMORS > 90% • ADVANTAGES – Inexpensive – Often office or outpatient procedure • DISADVANTAGES – More difficult with recurrent, indistinct tumors – Margin control difficult in some locations PDT • Not approved for BCC in USA • Combination of Drug + Light Effect – Drug can be given as cream, by mouth or iv. – Currently two topicals approved in USA (AK) • Levulan Kerastick • Metvix – Some studies in BCC exist • Metvix - 70% Cure at 2 years (Arch Derm 2004) PDT PDT Pathway PDT Selectivity Topical Imiquimod (Aldara) • Approved FDA 2004 for Superficial BCC – 5 nights per week – Total 6 week course – Cure 70-85% – Not tested in lesions <1cm from eyes, nose, mouth, ears – Largest diameter 2cm • Side Effects – Significant irritation at site common Topical Imiquimod • Possible role in nodular BCC – Cure Rates 12 weeks: • Once daily 5nights per week: 70% • Twice daily 7 nights per week: 76% • Once daily 3 nights/ week: 60% – Cure Rates 6 weeks • Similar MOHS MICROGRAPHIC SURGERY • Definition: – The multistage excision of (non-melanoma skin) cancer using meticulous histologic examination of horizontal sections of removed tissue to guide the excision. – Allows maximal preservation of normal tissue with the highest published cure rates for selected tumors. MOHS MICROGRAPHIC SURGERY • Useful for difficult tumors with lower cure rates with standard methods: – Recurrent – Large – Difficult Anatomic Locations on Face – Clinically indistinct (ie margins difficult to ascertain) – Aggressive Pathology (Sclerosing) 3 - 4mm margin WHERE TO CUT? 2. Excise Stage 1 1. “Debulk” Mohs Micrographic Surgery 2. Excise Stage 1 Initial Defect 3. Prepare 1. “Debulk” Tissue Initial Defect Prepare Tissue (Patient Waits) Taking residual Tumor - Stage II Map Stage 1 Positive Repairing Defect Clear Margins Hierarchy of Options •2nd Intention •Primary Closure •Skin Graft -FTSG -STSG •Local Flap -Advancement -Rotation -Transposition -Pedicle •2-Stage Local Flap •Combination Repair •Other -Free Flap -Tissue Expansion