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  • Sebaceous carcinoma (SC) is a malignant adnexal neoplasm arising from sebaceous glands.

  • Periocular SC and extraocular SC behave differently and require a different diagnostic approach and management.

  • SC can be associated with Muir-Torre syndrome (MTS).


  • SC can have a nonspecific appearance, which can delay diagnosis and treatment.

  • Consider a biopsy for all nonresolving eyelid lesions or unilateral blepharitis not responsive to treatment after 6 months in patients older than 60 years.

  • Screen for MTS in the appropriate population.


  • Margin-controlled surgical techniques such as Mohs micrographic surgery or complete circumferential peripheral and deep margin assessment are the preferred treatment modalities for both extraocular and periocular SC.


  • Patients with a history of SC should be counseled on the need for regular self-examination as well as in-office clinical examinations every 6 months for the first 3 years following diagnosis.

  • Patients with MTS will require a multidisciplinary approach with intensive follow-up and screening due to the high incidence of internal malignancies.


Sebaceous carcinoma (SC) is an uncommon malignant neoplasm arising from sebaceous glands.1,2 Though rare, they are associated with significant morbidity and mortality.2-6 Tumors can develop in any location with sebaceous glands but favor the head and neck region.1,4,7 Sebaceous carcinomas are divided into periocular SC and extraocular SC. This distinction is relevant as tumors in these locations display different genetic and molecular signatures and behavior.2,8-10 Consequently, treatment algorithms vary between these subtypes.8 SC can be associated with Muir-Torre syndrome (MTS), a variant of Lynch syndrome that presents with skin neoplasms (typically sebaceous neoplasms or keratoacanthomas) in addition to one or more Lynch syndrome–related cancers.1,11,12



SC accounts for 0.2% to 4.6% of all malignant cutaneous neoplasms in the United States.2,13 Data analysis of the National Cancer Institute Surveillance Epidemiology and End Results-18 Program (SEER-18) estimates the overall incidence of SC in the United States at 0.23 per 100,000 person-years.2 Over the last decade, this number has been found to be steadily increasing, specifically because of an increase in incidence in white men.2,13 This mimics trends seen in nonmelanoma and melanoma skin cancer. The likelihood of developing SC increases with age. The peak incidence for both SC subtypes is between the ages of 60 and 79 years, with a mean age at the time of diagnosis of 67 years.2,8 Rare cases of SC in younger adults and children have been reported, and these patients should be evaluated for underlying immunosuppression and MTS.12,14-16

When evaluating demographic data per SC subgroup, it is noticeable that incidence trends appear to be shifting. Historically, ...

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