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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

Daniel Berg M.D., FRCPC

Director, Dermatologic Surgery

University of Washington



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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


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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


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EPIDEMIOLOGY

LIFETIME RISK OF BCC AND SCC

MEN: 18.6%

WOMEN: 18%

(based on B.C. data - lifespan 75 yrs.)


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BCNS Time of Onset BCC

  • Before puberty: 15%

  • By age 22: 50%

  • By age 35: 90%

  • None over age 30: 10%


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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


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Tumor Suppressors

Proteins that normally act as brake on cell growth.

P

Patched

Smo

Inhibits

Downstream

Target Genes

Growth

Induces


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Patched

P

P

Normal

Cell

Cell at Risk

BCC Cell


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UVB

Ultraviolet Light



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BASAL CELL CARCINOMA

  • CLINICAL PRESENTATION

  • Nodular

  • Superficial

  • Morpheaform

  • Pigmented







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NonMelanoma Skin Cancer

Choice of Treatment

Balance:

CURE RATE

FUNCTIONAL RESULT

COSMETIC RESULT


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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


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Treatments

  • Topical

    • 5FU (Effudex)

      • Superficial only

    • Imiquimod (Aldara)

      • Just approved by FDA 2004

  • Surgery

    • ED&C (scrape and burn)

    • Excision

      • Mohs

      • Regular


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Treatments

  • Radiation

    • Not in BCNS

  • Other

    • PDT


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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


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ED&C

Initial Lesion (BCC)

Curettage (after biopsy)


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ED&C

Desiccation

Repeat X 3


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Final Defect

ED&C

Typical Scar


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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


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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)


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PDT

PDT Pathway

PDT Selectivity


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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


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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


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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.


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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)


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WHERE TO

CUT?

3 - 4mm margin


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2. Excise Stage 1

1. “Debulk”

Mohs Micrographic Surgery

2. Excise Stage 1

Initial Defect


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3. Prepare Tissue

1. “Debulk”

Initial Defect


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Prepare Tissue

(Patient Waits)


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Taking residual Tumor - Stage II

Map Stage 1

Positive


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Repairing Defect

Clear Margins


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  • 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



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