Download Basal Cell Nevus Syndrome - Dr. Daniel Berg

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
• Sensitivity to Radiation Treatment
–Palmar Pits
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
LIFETIME RISK OF BCC AND SCC
MEN:
18.6%
WOMEN:
18%
(based on B.C. data - lifespan 75 yrs.)
BCNS Time of Onset BCC
• Before puberty:
• By age 22:
15%
50%
• By age 35:
90%
• None over age 30:
10%
Remember this?
P
M
•DNA molecules make up genes
•Genes are blueprints for Proteins
•Proteins are the building blocks of
body functions
•Some proteins control cell growth
D
•Everyone has two copies of each gene
•One each from Mum and Dad
Tumor Suppressors
Proteins that normally act
as brake on cell growth.
P
Patched
Inhibits
Induces
Smo
Downstream
Target Genes
Growth
Patched
P
Normal
Cell
P
Cell at Risk
BCC Cell
UVB
Ultraviolet Light
Spring Break - circa 1900
BASAL CELL CARCINOMA
• CLINICAL PRESENTATION
•
•
•
•
Nodular
Superficial
Morpheaform
Pigmented
Nodular
Superficial
Pigmented
Morpheaform
Infiltrative
NonMelanoma Skin Cancer
Choice of Treatment
Balance:
CURE RATE
FUNCTIONAL RESULT
COSMETIC RESULT
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
• Topical
– 5FU (Effudex)
• Superficial only
– Imiquimod (Aldara)
• Just approved by FDA 2004
• Surgery
– ED&C (scrape and burn)
– Excision
• Mohs
• Regular
Treatments
• Radiation
– Not in BCNS
• Other
– PDT
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
ED&C
Initial Lesion (BCC)
Curettage (after biopsy)
ED&C
Desiccation
Repeat X 3
Final Defect
ED&C
Typical Scar
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
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)
PDT
PDT Pathway
PDT Selectivity
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
• 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
• 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
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)
3 - 4mm margin
WHERE TO
CUT?
2. Excise Stage 1
1. “Debulk”
Mohs
Micrographic
Surgery
2. Excise Stage 1
Initial Defect
3. Prepare
1. “Debulk”
Tissue
Initial Defect
Prepare Tissue
(Patient Waits)
Taking residual Tumor - Stage II
Map Stage 1
Positive
Repairing Defect
Clear Margins
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