- Patricia Tai, MB, BS, DABR, FRCR, FRCPC
Patricia Tai, MB, BS, DABR, FRCR, FRCPC
- Clinical Oncologist
- Clinical Professor
- University of Saskatchewan
- Canada Department of Radiation Oncology
- Allan Blair Cancer Center
- Section Editors
- Robert S Stern, MD
Robert S Stern, MD
- Section Editor — Nonmelanoma Skin Cancer
- Professor of Dermatology
- Harvard Medical School
- June K Robinson, MD
June K Robinson, MD
- Section Editor — Nonmelanoma Skin Cancer
- Professor of Clinical Dermatology
- Northwestern University Feinberg School of Medicine
Sebaceous carcinoma is a rare malignant tumor of the sebaceous glands . It can occur in any body site where sebaceous glands are present, but is most commonly found in the head and neck region, particularly in the periocular area . Sebaceous carcinoma of the eyelid (picture 1) may be mistaken for inflammatory lesions, such as chalazion or blepharoconjunctivitis, resulting in a delayed diagnosis and poorer prognosis .
Sebaceous carcinomas can occur sporadically or may be associated with Muir-Torre syndrome, a subset of the hereditary nonpolyposis colorectal cancer syndrome (HNCCS, Lynch syndrome) characterized by single or multiple sebaceous neoplasms, keratoacanthomas, and internal malignancy .
This topic will review the pathogenesis, clinical presentation, diagnosis, and treatment of sebaceous carcinoma. Muir-Torre syndrome and Lynch syndrome are discussed separately. (See "Muir-Torre syndrome" and "Lynch syndrome (hereditary nonpolyposis colorectal cancer): Clinical manifestations and diagnosis" and "Lynch syndrome (hereditary nonpolyposis colorectal cancer): Screening and management".)
Sebaceous carcinoma is a rare tumor, with an estimated incidence rate of approximately 1 to 2 per 1,000,000 per year [2,4,5]. However, sebaceous carcinoma is the most common eyelid malignancy after basal cell carcinoma and squamous cell carcinoma [6,7].
A review of the Surveillance, Epidemiology and End Results (SEER) database from 1973 to 2004 identified 1349 cases of sebaceous carcinoma . More than 90 percent of cases were seen in patients over 50 years old and the median age was 72 years. Sebaceous carcinoma is exceedingly rare in children [8,9].
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- CLINICAL PRESENTATION
- Eyelid sebaceous carcinoma
- Extra-ocular sebaceous carcinoma
- ASSOCIATION WITH MUIR-TORRE SYNDROME
- Screening for DNA mismatch repair defects
- DIFFERENTIAL DIAGNOSIS
- EVALUATION AND STAGING
- Local disease
- - Surgical treatment
- - Radiation therapy
- - Adjuvant therapies
- Metastatic disease
- SUMMARY AND RECOMMENDATIONS