Sebaceous carcinoma usually occurs in adults older than 60 years, on the eyelid, head and neck, and trunk. In this Review, we present clinical care recommendations for sebaceous carcinoma, which were developed as a result of an expert panel evaluation of the findings of a systematic review. Key conclusions were drawn and recommendations made for diagnosis, first-line treatment, radiotherapy, and post-treatment care. For diagnosis, we concluded that deep biopsy is often required; furthermore, differential diagnoses that mimic the condition can be excluded with special histological stains. For treatment, the recommended first-line therapy is surgical removal, followed by margin assessment of the peripheral and deep tissue edges; conjunctival mapping biopsies can facilitate surgical planning. Radiotherapy can be considered for cases with nerve or lymph node involvement, and as the primary treatment in patients who are ineligible for surgery. Post-treatment clinical examination should occur every 6 months for at least 3 years. No specific systemic therapies for advanced disease can be recommended, but targeted therapies and immunotherapies are being developed.
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We searched MEDLINE, Web of Science, Embase, and Cochrane CENTRAL database for articles published between Jan 1, 1990, and June 30, 2019, for “sebaceous carcinoma”, “Meibomian gland carcinoma”, “tarsal gland carcinoma”, and “Zeis gland carcinoma”. We chose the year 1990 as the earliest date because historical approaches from before this time might differ from modern practice. We narrowed our search results by title, abstract, and full-text review, and we evaluated the reference lists of review articles for additional eligible studies. We extracted studies related to sebaceous carcinoma diagnosis, investigations, treatment, or follow-up. Articles that described fewer than three patients, management related to Muir-Torre syndrome, or those that contained no original data were excluded. 61 articles met the search criteria for extraocular sebaceous carcinoma, and 95 articles met the search criteria for periocular sebaceous carcinoma ( figure 1 ). During data extraction, when possible, case-level outcomes were preserved. All included studies were systematically assessed for bias using the ROBINS-I tool ( appendix pp 2–11 ). In accordance with ROBINS-I methods, non-randomised studies were classified as those with at least a moderate risk of bias. Contributors JLO, NK, and BW contributed equally to this manuscript and are the lead authors. JLO, NK, BW, and RCK were on the data team. MA was the Guidelines Chair. JLO, NK, BW, and MA had full access to the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis. MA, JLO, NK, BW, and EP were responsible for the concept and study design. All authors collected, analysed, and interpreted the data. MA, JLO, NK, BW, and RCK drafted the manuscript. All authors critically revised the manuscript for important intellectual content. TR did the statistical analysis. JLO, NK, BW, and EP provided administrative, technical, or material support. MA supervised the study. All authors had access to the information and certify its accuracy. Declaration of interests NCZ has received personal fees from Genetech, Sanofi/Regeneron, Biofrontera, and Sun Pharma; and grants from Sun Pharmagrants and Biofrontera, outside the submitted work. DB has received personal fees from Abbvie and Pelle Pharm, outside the submitted work. MA has received personal fees from Pulse Biosciences, outside the submitted work. CAB has received grants from Elekta, University of California San Francisco, Australia and New Zealand Melanoma Trials Group, Merck, and Amgen; personal fees from National Comprehensive Cancer Network, American College of Radiation Oncology, University of Rochester, University of Florida, Elsevier, University of Colorado, Charlotte Area Health Education Center, American Academy of Dermatology, University of Florida Health Center Orlando, Driver Group, Weill Cornell Medical Center, Patient Resource, and Pfizer; and non-financial support from National Comprehensive Cancer Network, American College of Radiation Oncology, University of Rochester, University of Florida, University of Colorado, Charlotte Area Health Education Center, American Academy of Dermatology, University of Florida Health Center Orlando, Driver Group, Pfizer, American Head and Neck Society, and Alpha Tau Medical, outside the submitted work. TMK has received grants from Tempus, outside the submitted work. MLC has received personal fees from Allergan, MD Outlook, Medline Industries, and Sanofi Genzyme/Regeneron, outside the submitted work. HD reports personal fees from Castle Biosciences and Immunocore, outside the submitted work. EHL has received personal fees from UpToDate, outside the submitted work. TMS has received grants from Regeneron, outside the submitted work. SYL is a health affairs coordinator for Cardinal Health, outside the submitted work. The remaining authors declare no competing interests. Acknowledgments This study was partially supported by unrestricted research funding from the Section of Cutaneous Surgery in the Northwestern University Department of Dermatology (IL, USA). We thank Ellen Burke, a patient representative who contributed her perspective based on personal experiences with sebaceous carcinoma and helped shape the summary statement.