A Phase II Single Arm Open-Label Clinical Trial of Chemotherapy of BCC's With Tazarotene 0.1% in Subjects With Basal Cell Nevus Syndrome

Inclusion Criteria

DISEASE CHARACTERISTICS:

- Diagnosis of ≥ 1 basal cell carcinoma (BCC) ≥ 3 mm (not impinging on vital sites) on
the face

- Meets 1 of the following criteria for diagnosis of basal cell nevus syndrome (BCNS)

- Has major criterion # 1 (first in the list of major criteria) AND 1 additional
major criterion OR 2 minor criteria

- Has a first degree relative with BCNS AND any 2 major criteria or any single
major plus 2 minor criteria

- Major criteria include:

- More than 2 BCCs total OR 1 BCC before the age of 20 years

- Odontogenic keratocysts of the jaw proven by histology

- Three or more palmar and/or plantar pits

- Bilamellar calcification of the falx cerebri (if less than 20 years old)

- Fused, bifid, or markedly splayed ribs

- First degree relative with BCNS

- PTCH1 gene mutation in normal tissue

- Minor criteria include:

- Macrocephaly determined after adjustment for height

- Congenital malformations (i.e., cleft lip or palate, frontal bossing, "coarse
face", moderate or severe hypertelorism)

- Skeletal abnormalities (i.e., Sprengel deformity, marked pectus deformity, or
marked syndactyly of the digits)

- Radiological abnormalities (i.e., bridging of the sella turcica or vertebral
anomalies, such as hemivertebrae, fusion or elongation of the vertebral bodies,
modeling defects of the hands and feet, or flame shaped lucencies of the hands
or feet)

- Ovarian fibroma

- Medulloblastoma

PATIENT CHARACTERISTICS:

- Not pregnant or nursing

- Negative pregnancy test

- Fertile patients must use effective contraception

- Willing to apply sunscreen (SPF ≥ 15) at least once daily on all exposed skin sites

- No history of hypersensitivity to any of the ingredients in the study medication
formulations

- No uncontrolled systemic disease

- No known HIV positivity

- No history of other skin conditions or significant illness that would interfere with
evaluation of the study medication

- No condition or situation that, in the investigator's opinion, may put the patient at
significant risk, could confound the study results, or could significantly interfere
with the patient's participation in the study

PRIOR CONCURRENT THERAPY:

- No prior topical or systemic therapies that would interfere with the evaluation of
the study medication

- At least 30 days since prior systemic investigational medication or topical
investigational medication to the face

- At least 6 months since prior systemic or topical retinoids (e.g., etretinate,
isotretinoin, tazarotene, tretinoin, or adapalene) or glucocorticoids (other than ≤
1% hydrocortisone applied no more than 6 times/month) to the face

- At least 6 months since prior systemic or topical alpha hydroxy acids (e.g., glycolic
acid or lactic acid) to the face

- At least 6 months since prior topical chemotherapeutic agents (e.g., fluorouracil,
retinoids, or imiquimod) to the face as chemoprevention prophylaxis

- At least 1 year since prior systemic chemotherapy

- No other concurrent topical medications to the face, including prescription and
over-the-counter preparations (e.g., topical preparations containing corticosteroids
[other than ≤ 1% hydrocortisone applied no more than 6 times/month]) or vitamin A
derivatives

- No concurrent enrollment in another clinical investigational drug or device trial
that involves systemic administration of the drug or use of the drug/device on the
face

- No other concurrent systemic retinoids, chemotherapeutic agents, or local treatment
to the face (e.g., laser resurfacing, imiquimod, fluorouracil, alpha hydroxy acids,
irradiation, or topical or systemic photodynamic therapy [except as localized
treatment of specific tumors])

- Concurrent moisturizers and emollients allowed