Upload
presta
View
97
Download
0
Tags:
Embed Size (px)
DESCRIPTION
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 - PowerPoint PPT Presentation
Citation preview
Basal Cell Nevus SyndromeDaniel Berg M.D., FRCPCDirector, 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
EPIDEMIOLOGYEPIDEMIOLOGY
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: 15%• By age 22: 50%
• By age 35: 90%
• None over age 30: 10%
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
•Everyone has two copies of each gene•One each from Mum and Dad
P
MD
Inhibits
Induces
Smo
DownstreamTarget Genes
Growth
Patched
Tumor SuppressorsProteins that normally act as brake on cell growth.
P
Patched
NormalCell
BCC CellCell at Risk
PP
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
Typical ScarED&C
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)
WHERE TO CUT?
3 - 4mm margin
1. “Debulk”2. Excise Stage 1
Initial Defect
Mohs Micrographic Surgery
2. Excise Stage 1
1. “Debulk”
Initial Defect
3. Prepare Tissue
Prepare Tissue(Patient Waits)
Map Stage 1Positive
Taking residual Tumor - Stage II
Clear Margins
Repairing Defect
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