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