Basal cell carcinoma (BCC) is the most common form of human cancer, with a continually increasing annual incidence in the United States. When diagnosed early, the majority of BCCs are readily treated with office-based therapy, which is highly curative. In these evidence-based guidelines of care, we provide recommendations for the management of patients with BCC, as well as an in-depth review of the best available literature in support of these recommendations. We discuss biopsy techniques for a clinically suspicious lesion and offer recommendations for the histopathologic interpretation of BCC. In the absence of a formal staging system, the best available stratification based on risk for recurrence is reviewed. With regard to treatment, we provide recommendations on treatment modalities along a broad therapeutic spectrum, ranging from topical agents and superficially destructive modalities to surgical techniques and systemic therapy. Finally, we review the available literature and provide recommendations on prevention and the most appropriate follow-up for patients in whom BCC has been diagnosed. Basal cell carcinoma (BCC) is the most common form of human cancer, with a continually increasing annual incidence in the United States. When diagnosed early, the majority of BCCs are readily treated with office-based therapy, which is highly curative. In these evidence-based guidelines of care, we provide recommendations for the management of patients with BCC, as well as an in-depth review of the best available literature in support of these recommendations. We discuss biopsy techniques for a clinically suspicious lesion and offer recommendations for the histopathologic interpretation of BCC. In the absence of a formal staging system, the best available stratification based on risk for recurrence is reviewed. With regard to treatment, we provide recommendations on treatment modalities along a broad therapeutic spectrum, ranging from topical agents and superficially destructive modalities to surgical techniques and systemic therapy. Finally, we review the available literature and provide recommendations on prevention and the most appropriate follow-up for patients in whom BCC has been diagnosed. Adherence to these guidelines will not ensure successful treatment in every situation. Furthermore, these guidelines should not be interpreted as setting a standard of care, or be deemed inclusive of all proper methods of care, nor exclusive of other methods of care reasonably directed to obtaining the same results. The ultimate judgment regarding the propriety of any specific therapy must be made by the physician and the patient in light of all the circumstances presented by the individual patient, and the known variability and biologic behavior of the disease. This guideline reflects the best available data at the time the guideline was prepared. The results of future studies may require revisions to the recommendations in this guideline to reflect new data. This guideline addresses the management of patients with basal cell carcinoma (BCC) from the perspective of a US dermatologist. The main focus of the guideline is on the most commonly considered and utilized approaches for the surgical and medical treatment of primary BCC, but it also includes recommendations on the treatment of recurrent tumors when applicable, appropriate biopsy techniques, staging, follow-up, and prevention of BCC. A detailed discussion of specific chemotherapeutic or radiotherapeutic approaches for distant metastatic BCC falls outside the scope of this guideline. However, general recommendations on the management of patients with advanced or metastatic BCC are included to provide guidance and facilitate consultation with a physician or multidisciplinary group with specific expertise in BCC, such as a surgical, medical, or radiation oncologist, head and neck surgeon, plastic surgeon, or dermatologist specializing in BCC. An expert work group was convened to determine the audience and scope of the guideline and to identify important clinical questions in the biopsy, staging, treatment, and follow-up of BCC (Table I). Work group members completed a disclosure of interests that was updated and reviewed for potential relevant conflicts of interest throughout the guideline development. If a potential conflict was noted, the work group member recused himself or herself from discussion and drafting of recommendations pertinent to the topic area of the disclosed interest.Table IClinical questions used to structure the evidence review•What is the standard grading system for BCC and cSCC?•What are the standard biopsy techniques for BCC and cSCC?•What pathologic and clinical information is useful in the pathology report for BCC and cSCC?•What are the benefits, harm, and effectiveness/efficacy of available treatments for BCC and cSCC?○Surgical treatment•Standard excision•Mohs micrographic surgery•Curettage and electrodessication•Cryosurgery○Topical therapy•Fluorouracil•Imiquimod•Other○Energy devices•Laser•Photodynamic therapy∗BCC only.•Radiation therapy•What are effective treatment options for the management of advanced BCC and cSCC?○Hedgehog inhibitors∗BCC only.•What are the effective methods for follow-up and prevention of recurrence and new primary keratinocyte cancer formation?○Oral and topical retinoids○Celecoxib○α-Difluoromethylornithine○Selenium○β-CaroteneBCC, Basal cell carcinoma; cSCC, cutaneous squamous cell carcinoma.∗ BCC only. Open table in a new tab BCC, Basal cell carcinoma; cSCC, cutaneous squamous cell carcinoma. An evidence-based approach was used, and available evidence was obtained by using a systematic search and review of published studies from PubMed and the Cochrane Library databases from January 1960 through April 2015 for all identified clinical questions. A secondary search was subsequently undertaken to identify and review published studies from April 2015 to August 2016 to provide the most current information. Searches were prospectively limited to publications in the English language. As BCC is traditionally known as a form of nonmelanoma skin cancer (NMSC), which also includes cutaneous squamous cell carcinoma (cSCC), searches were collectively undertaken for literature on BCC and cSCC simultaneously, using a set of search terms applicable to both BCC and cSCC. A parallel American Academy of Dermatology (AAD) guideline on cSCC has also been developed.1Alam M. Armstrong A. Baum C. et al.Guidelines of care for the management of squamous cell carcinoma.J Am Acad Dermatol. 2018; 78: 560-578Abstract Full Text Full Text PDF Scopus (0) Google Scholar MeSH (Medical Subject Headings) terms used in various combinations in the literature search included carcinoma, basal cell carcinoma, squamous cell carcinoma, skin neoplasms, stage(ing), grade(ing), score(ing), biopsy, pathology, prognosis, signs and symptoms, risk factors, curettage, electrodessication, excision, incomplete, cryosurgery, Mohs (micrographic) surgery, topical, fluorouracil, imiquimod, laser, radiotherapy, radiation, photochemotherapy, phototherapy, metastasis, vismodegib, sonidegib, prevention, prevention and control, and recurrence. A total of 1120 articles were systematically reviewed for possible inclusion; 188 were retained on the basis of relevancy and the highest level of available evidence for the outlined clinical questions. Evidence tables were generated for these 188 studies and utilized by the work group in developing recommendations. Other current available guidelines on BCC were also evaluated.2National Comprehensive Cancer Center. NCCN Clinical practice guidelines in oncology; basal cell skin cancer. Available at: www.nccn.org. Accessed April 1, 2015.Google Scholar, 3National Comprehensive Cancer Center. NCCN clinical practice guidelines in oncology; basal cell carcinoma (V1.2017). Available at: www.nccn.org. Accessed October 3, 2016.Google Scholar, 4Telfer N.R. Colver G.B. Morton C.A. British Association of Dermatology. Guidelines for the management of basal cell carcinoma.Br J Dermatol. 2008; 159: 35-48Crossref PubMed Scopus (0) Google Scholar The available evidence was evaluated using a unified system called the Strength of Recommendation Taxonomy (SORT), which was developed by editors of the US family medicine and primary care journals (ie, American Family Physician, Family Medicine, Journal of Family Practice, and BMJ USA).5Ebell M.H. Siwek J. Weiss B.D. et al.Strength of recommendation taxonomy (SORT): a patient-centered approach to grading evidence in the medical literature.J Am Board Fam Pract. 2004; 17: 59-67Crossref PubMed Google Scholar Evidence was graded using a 3-point scale based on the quality of study methodology (eg, randomized controlled trial [RCT], case-control, prospective/retrospective cohort, case series, etc) and the overall focus of the study (ie, diagnosis, treatment/prevention/screening, or prognosis) as follows:I.Good-quality patient-oriented evidence (ie, evidence measuring outcomes that matter to patients: morbidity, mortality, symptom improvement, cost reduction, and quality of life).II.Limited-quality patient-oriented evidence.III.Other evidence, including consensus guidelines, opinion, case studies, and disease-oriented evidence (ie, evidence measuring intermediate, physiologic, or surrogate end points that may or may not reflect improvements in patient outcomes). Clinical recommendations were developed on the basis of the best available evidence tabled in the guideline. These are ranked as follows:A.Recommendation based on consistent and good-quality patient-oriented evidence.B.Recommendation based on inconsistent or limited-quality patient-oriented evidence.C.Recommendation based on consensus, opinion, case studies, or disease-oriented evidence. In situations in which documented evidence-based data were not available, expert opinion of the authors was utilized to generate clinical recommendations. This guideline has been developed in accordance with the AAD/AAD Association Administrative Regulations for Evidence-Based Clinical Practice Guidelines, which includes the opportunity for review and comment by the entire AAD membership and final review and approval by the AAD Board of Directors.6American Academy of Dermatology. Administrative regulations; evidence based clinical practice guidelines. Available at: www.aad.org/Forms/Policies/Uploads/AR/AR%20-%20Evidence-Based%20Clinical%20Guideline.pdf. Accessed December 1, 2014.Google Scholar An additional multidisciplinary panel of invited reviewers was utilized to provide cross-specialty comments on the draft guideline. This guideline will be considered current for a period of 5 years from the date of publication, unless reaffirmed, updated, or retired at or before that time.