Basal Cell Nevus Syndrome

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Basal Cell Nevus Syndrome

• Daniel Berg M.D., FRCPC
• Director, Dermatologic Surgery
• University of Washington

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Thank Goodness.. Shade at Last!
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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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Spring Break - circa 1900
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BASAL CELL CARCINOMA

• CLINICAL PRESENTATION
• Nodular
• Superficial
• Morpheaform
• Pigmented

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Nodular
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Superficial
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Pigmented
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Morpheaform
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Infiltrative
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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
• EDC (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 gt90
• ADVANTAGES
• Inexpensive
• Outpatient Office Procedure
• Quick
• DISADVANTAGES
• High Recurrence Rate for Difficult Tumors
• Location, recurrent, deep

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EDC
Initial Lesion (BCC)
Curettage (after biopsy)
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EDC
Desiccation
Repeat X 3
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Final Defect
EDC
Typical Scar
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SURGICAL EXCISION

• CURE FOR PRIMARY TUMORS gt 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 lt1cm 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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1. Debulk
3. Prepare Tissue
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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The End