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Basal Cell Nevus Syndrome

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

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Basal Cell Nevus Syndrome

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  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 40-79 • 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 P M D • Everyone has two copies of each gene • One each from Mum and Dad

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

  9. Patched P P Normal Cell Cell at Risk BCC Cell

  10. UVB Ultraviolet Light

  11. Spring Break - circa 1900

  12. BASAL CELL CARCINOMA • CLINICAL PRESENTATION • Nodular • Superficial • Morpheaform • Pigmented

  13. Nodular

  14. Superficial

  15. Pigmented

  16. Morpheaform

  17. Infiltrative

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

  19. 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

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

  21. Treatments • Radiation • Not in BCNS • Other • PDT

  22. 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

  23. ED&C Initial Lesion (BCC) Curettage (after biopsy)

  24. ED&C Desiccation Repeat X 3

  25. Final Defect ED&C Typical Scar

  26. 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

  27. 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)

  28. PDT PDT Pathway PDT Selectivity

  29. 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

  30. 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

  31. 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.

  32. 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)

  33. WHERE TO CUT? 3 - 4mm margin

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

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

  36. Prepare Tissue (Patient Waits)

  37. Taking residual Tumor - Stage II Map Stage 1 Positive

  38. Repairing Defect Clear Margins

  39. 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

  40. The End

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