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Basal Cell Nevus Syndrome Daniel Berg M.D., FRCPC Director, Dermatologic Surgery University of Washington.

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Presentation on theme: "Basal Cell Nevus Syndrome Daniel Berg M.D., FRCPC Director, Dermatologic Surgery University of Washington."— Presentation transcript:

1 Basal Cell Nevus Syndrome Daniel Berg M.D., FRCPC Director, Dermatologic Surgery University of Washington

2 Thank Goodness….. Shade at Last!

3 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

4 BASAL CELL CARCINOMA (BCC) Commonest Cancer U.S. 800,000/yr –99% in Caucasians –95% between age –85% on Head & Neck –Risk of Metastasis: Very Very Low –Main potential problem: Local Invasion

5 EPIDEMIOLOGY LIFETIME RISK OF BCC AND SCC MEN:18.6% WOMEN:18% (based on B.C. data - lifespan 75 yrs.)

6 BCNS Time of Onset BCC Before puberty: 15% By age 22: 50% By age 35: 90% None over age 30: 10%

7 Remember this? DNA molecules make up genes Genes are blueprints for Proteins Proteins are the building blocks of body functions Some proteins control cell growth Everyone has two copies of each gene One each from Mum and Dad P M D

8 Inhibits Induces Smo Downstream Target Genes Growth Patched Tumor Suppressors Proteins that normally act as brake on cell growth. P

9 Patched Normal Cell BCC Cell Cell at Risk P P

10 UVB Ultraviolet Light

11 Spring Break - circa 1900

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13 BASAL CELL CARCINOMA CLINICAL PRESENTATION Nodular Superficial Morpheaform Pigmented

14 Nodular

15 Superficial

16 Pigmented

17 Morpheaform

18 Infiltrative

19 NonMelanoma Skin Cancer Choice of Treatment Balance: CURE RATE FUNCTIONAL RESULT COSMETIC RESULT

20 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

21 Treatments Topical –5FU (Effudex) Superficial only –Imiquimod (Aldara) Just approved by FDA 2004 Surgery –ED&C (scrape and burn) –Excision Mohs Regular

22 Treatments Radiation –Not in BCNS Other –PDT

23 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

24 ED&C Initial Lesion (BCC) Curettage (after biopsy)

25 ED&C Desiccation Repeat X 3

26 Final Defect Typical Scar ED&C

27 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

28 PDT Not approved for BCC in USA Combination of Drug + LightEffect –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)

29 PDT PDT Pathway PDT Selectivity

30 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

31 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

32 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.

33 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)

34 WHERE TO CUT? 3 - 4mm margin

35 1. “Debulk” 2. Excise Stage 1 Initial Defect Mohs Micrographic Surgery 2. Excise Stage 1

36 1. “Debulk” Initial Defect 3. Prepare Tissue

37 Prepare Tissue (Patient Waits)

38 Map Stage 1 Positive Taking residual Tumor - Stage II

39 Clear Margins Repairing Defect

40 Hierarchy of Options 2 nd Intention Primary Closure Skin Graft -FTSG -STSG Local Flap -Advancement -Rotation -Transposition -Pedicle 2-Stage Local Flap Combination Repair Other -Free Flap -Tissue Expansion

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