Nevoid Basal Cell Carcinoma Syndrome Medication

  • Author: Daniel Berg, MD, FRCP(C); Chief Editor: Dirk M Elston, MD   more...
 
Updated: Sep 14, 2011
 

Medication Summary

Topical agents such as imiquimod and 5-fluorouracil are available and have been approved for treatment of basal cell carcinoma (BCC) in the United States. These medications have a lower cure rate than surgical therapy but may be useful adjuncts in patients with multiple lesions.

Investigations and clinical trials are evaluating the use of these and other novel agents such as ALA and methyl-ALA in topical photodynamic therapy, tazarotene (Tazorac), and newly developed HH pathway inhibitors both topical and systemic.[2] Although tazarotene and photodynamic therapy are available (off-label), hedgehog inhibitors, which appear very promising,[19] are not available for use outside of clinical trials. Approval by the US Food and Drug Administration (FDA) and availability await further testing and results. Interested readers can visit ClinicalTrials.gov for more information on clinical studies.

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Topical Skin Product

Class Summary

Agents such as imiquimod and 5-fluorouracil are approved in the United States for use topically for BCC. Despite the lower cure rate (than with surgery), imiquimod and 5-fluorouracil may play a useful role in allowing patients to treat their own selected smaller lesions, especially superficial BCCs on the trunk and extremities. In general, such medications should be considered best for use in smaller or more superficial lesions, away from critical anatomic sites.

Imiquimod (Aldara)

 

The precise mechanism of imiquimod on superficial BCC is unknown. It may increase tumor infiltration of lymphocytes, dendritic cells, and macrophages. It is indicated for biopsy-confirmed, primary superficial BCC in adults with normal immune systems. Additionally, tumors generally should not exceed 2 cm in diameter on certain areas of the body. It is indicated primarily when surgical methods are not appropriate, including in situations in which, because of multiple lesions, scarring from surgical methods may be a consideration.

5-Fluorouracil (Efudex, Carac, Fluoroplex)

 

5-Fluorouracil is a cycle-specific agent that has activity as single agent and, for many years, has been combined with the biochemical modulator leucovorin. It is shown to be effective in an adjuvant setting. 5-Fluorouracil inhibits tumor cell growth through at least 3 different mechanisms that ultimately disrupt DNA synthesis or cellular viability. It is used topically for the management of superficial BCC.

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Contributor Information and Disclosures
Author

Daniel Berg, MD, FRCP(C)  Professor of Dermatology, Director of Dermatologic Surgery, University of Washington School of Medicine

Daniel Berg, MD, FRCP(C) is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Mohs Micrographic Surgery and Cutaneous Oncology, and American Society for Dermatologic Surgery

Disclosure: Nothing to disclose.

Specialty Editor Board

R Stan Taylor, MD  The JB Howell Professor in Melanoma Education and Detection, Departments of Dermatology and Plastic Surgery, Director, Skin Surgery and Oncology Clinic, University of Texas Southwestern Medical Center

R Stan Taylor, MD is a member of the following medical societies: American Academy of Dermatology, American College of Mohs Surgery, American Dermatological Association, American Medical Association, American Society for Dermatologic Surgery, Christian Medical & Dental Society, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Michael J Wells, MD  Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association

Disclosure: Nothing to disclose.

Edward F Chan, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania School of Medicine

Edward F Chan, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

References
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  5. Hahn H, Wicking C, Zaphiropoulous PG, Gailani MR, Shanley S, Chidambaram A, et al. Mutations of the human homolog of Drosophila patched in the nevoid basal cell carcinoma syndrome. Cell. Jun 14 1996;85(6):841-51. [Medline].

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  15. Kimonis VE, Mehta SG, Digiovanna JJ, Bale SJ, Pastakia B. Radiological features in 82 patients with nevoid basal cell carcinoma (NBCC or Gorlin) syndrome. Genet Med. Nov-Dec 2004;6(6):495-502. [Medline].

  16. Ferreres JR, Macaya A, Jucglà A, Muniesa C, Prats C, Peyrí J. Hundreds of basal cell carcinomas in a Gorlin-Goltz syndrome patient cured with imiquimod 5% cream. J Eur Acad Dermatol Venereol. Aug 2006;20(7):877-8. [Medline].

  17. Itkin A, Gilchrest BA. delta-Aminolevulinic acid and blue light photodynamic therapy for treatment of multiple basal cell carcinomas in two patients with nevoid basal cell carcinoma syndrome. Dermatol Surg. Jul 2004;30(7):1054-61. [Medline].

  18. Oseroff AR, Shieh S, Frawley NP, Cheney R, Blumenson LE, Pivnick EK, et al. Treatment of diffuse basal cell carcinomas and basaloid follicular hamartomas in nevoid basal cell carcinoma syndrome by wide-area 5-aminolevulinic acid photodynamic therapy. Arch Dermatol. Jan 2005;141(1):60-7. [Medline].

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Multiple basal cell carcinomas on the back of a patient with nevoid basal cell carcinoma syndrome.
Multiple small papules on the neck and upper trunk in a 10-year-old patient. Biopsy confirmed basal cell carcinoma.
Palmar pits in an adult.
Syndactyly is noted in some affected persons such as this 8-year-old boy.
 
 
 
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