Sebaceous Carcinoma Epidemiology and Genetics: Emerging Concepts and Clinical Implications for Screening, Prevention, and Treatment

Abstract

Sebaceous carcinoma is an aggressive skin cancer with a 5-year overall survival rate of 78% for localized/regional disease and 50% for metastatic disease. The incidence of this cancer has been increasing in the United States for several decades, but the underlying reasons for this increase are unclear. In this article, we review the epidemiology and genetics of sebaceous carcinoma, including recent population data and tumor genomic analyses that provide new insights into underlying tumor biology. We further discuss emerging evidence of a possible viral etiology for this cancer. Finally, we review the clinical implications of recent advances in sebaceous carcinoma research for screening, prevention, and treatment.

Figure 1.. Summary diagram of known and possible risk factors for sebaceous carcinoma.

Population data analyses have identified several risk factors for sebaceous carcinoma, which are listed in the above figure. The highly elevated risk for sebaceous carcinoma among immunosuppressed populations (solid organ transplant recipients; individuals with HIV infection), which is similar to the elevation observed for other virus-associated cancers (Kaposi sarcoma; Merkel cell carcinoma), suggests that sebaceous carcinoma may also be caused by a virus. In addition to causing cancer in immunosuppressed patients (pathway A), an oncogenic virus could also promote tumorigenesis in immunocompetent patients (pathway B). Among individuals with Muir-Torre syndrome, higher ambient ultraviolet radiation exposure is associated with an increased risk for sebaceous carcinoma in addition to being an independent risk factor for this cancer type.