A 92-year-old man is referred for evaluation and treatment of a nonhealing growth on his nose. The lesion was first noticed approximately 1 year prior to presentation and has been steadily increasing in size. He denies bleeding or pain and gives no personal or family history of skin cancer. Examination reveals a 1.5cm firm, flesh-colored nodule with noticeable pearliness.
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Sebaceous carcinoma (SC) is a rare, aggressive malignant neoplasm that often presents as a painless, slow-growing, solitary nodule on the face or neck. It is typically a discrete lesion that may be pink, yellow, or white in color and may be accompanied by an inflammatory response. SC is categorized as ocular (orbit, conjunctiva, eyelid) or extraocular (head, neck).1
Ocular SC arises from sebaceous glands of the eyelids (meibomian or Zeis). This type of SC is both more common and more likely to metastasize.2 Extraocular SC is associated with Muir-Torre syndrome, an inherited cancer syndrome, caused by mutations that induce dysfunctional DNA repair.3 Histopathology reveals poorly differentiated basaloid cells with atypia. Differentiation from basal cell and squamous cell carcinoma may be difficult and some pathologies may resemble metastatic renal or prostate carcinoma as well as melanoma.4
Wide excision or Mohs micrographic surgery are treatments of choice.5 Radiation therapy is an option when surgery is contraindicated or as follow-up after surgery.6 A poor prognosis is associated with delayed diagnosis, metastases, lesion size greater than 1cm, and pagetoid spread.2-7 One retrospective study reported a mortality rate exceeding 30% at 5 years; this increased to 54% at 10 years.7
Stephen Schleicher, MD, is director of the DermDox Center for Dermatology in Pennsylvania, associate professor of medicine at Commonwealth Medical College, and clinical instructor of dermatology at Arcadia University and Kings College. Sarah Jacobs, PA-C, is a recent graduate of Kings College.
1. Foreman RK, Duncan L. Appendage tumors of the skin. In: Kang S, Amagai M, Bruckner AL, et al, eds. Fitzpatrick’s Dermatology, 9 ed. McGraw-Hill; 2019.
2. Wolfe JT, Yeatts RP, Wick MR, et al. Sebaceous carcinoma of the eyelid: errors in clinical and pathologic diagnosis. Am J Surg Pathol. 1984;8:597-606. doi: 10.1097/00000478-198408000-00003
3. Tetzlaff MT. Immunohistochemical markers informing the diagnosis of sebaceous carcinoma and its distinction from its mimics: adipophilin and factor XIIIa to the rescue? J Cutan Pathol. 2018;45(1):29-32. doi: 10.1111/cup.13073
4. Nelson BR, Hamlet KR, Gillard M, Railan D, Johnson TM. Sebaceous carcinoma. J Am Acad Dermatol. 1995;33(1):1-15; quiz 16-18. doi:10.1016/0190-9622(95)90001-2
5. Knackstedt T, Samie FH. Sebaceous carcinoma: a review of the scientific literature. Curr Treat Options Oncol. 2017;18(8):47. doi: 10.1007/s11864-017-0490-0
6. Yen MT, Tse DT, Wu X, et al. Radiation therapy for local control of eyelid sebaceous cell carcinoma: report of two cases and review of the literature. Ophthalmic Plast Reconstr Surg. 2000;16(3):211-215. doi: 10.1097/00002341-200005000-00008
7. Dasgupta T, Wilson LD, Yu JB. A retrospective review of 1349 cases of sebaceous carcinoma. Cancer. 2009;115(1):158-165. doi: 10.1002/cncr.2395