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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

Basal Cell Nevus Syndrome

Daniel Berg M.D., FRCPC

Director, Dermatologic Surgery

University of Washington

basal cell nevus syndrome3
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
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
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
BCNS Time of Onset BCC
  • Before puberty: 15%
  • By age 22: 50%
  • By age 35: 90%
  • None over age 30: 10%
remember this
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
slide8

Tumor Suppressors

Proteins that normally act as brake on cell growth.

P

Patched

Smo

Inhibits

Downstream

Target Genes

Growth

Induces

patched
Patched

P

P

Normal

Cell

Cell at Risk

BCC Cell

slide10

UVB

Ultraviolet Light

basal cell carcinoma
BASAL CELL CARCINOMA
  • CLINICAL PRESENTATION
  • Nodular
  • Superficial
  • Morpheaform
  • Pigmented
nonmelanoma skin cancer
NonMelanoma Skin Cancer

Choice of Treatment

Balance:

CURE RATE

FUNCTIONAL RESULT

COSMETIC RESULT

choice of treatment
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
Treatments
  • Topical
    • 5FU (Effudex)
      • Superficial only
    • Imiquimod (Aldara)
      • Just approved by FDA 2004
  • Surgery
    • ED&C (scrape and burn)
    • Excision
      • Mohs
      • Regular
treatments22
Treatments
  • Radiation
    • Not in BCNS
  • Other
    • PDT
ed c scrape burn
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
slide24

ED&C

Initial Lesion (BCC)

Curettage (after biopsy)

slide25

ED&C

Desiccation

Repeat X 3

slide26

Final Defect

ED&C

Typical Scar

surgical excision
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
slide28
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)
slide29
PDT

PDT Pathway

PDT Selectivity

topical imiquimod aldara
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
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
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 surgery33
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)
slide34

WHERE TO

CUT?

3 - 4mm margin

slide35

2. Excise Stage 1

1. “Debulk”

Mohs Micrographic Surgery

2. Excise Stage 1

Initial Defect

slide36

3. Prepare Tissue

1. “Debulk”

Initial Defect

slide37

Prepare Tissue

(Patient Waits)

slide39

Repairing Defect

Clear Margins

slide40

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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