•Poor prognosis occurs when lesions are recurrent, greater than 10 mm in size, longer than 6 months of duration, involving both lids, involving canthus, and having aggressive histologic features.

26.1Introduction

Sebaceous carcinoma (SC) is a rare adnexal skin cancer that challenges the abilities of all physicians and surgeons. Although it comprises about 4% of ocular skin malignancies, SC is one of the most malignant primary tumors of the eye with a 5-year death rate of 15% [1]. Most tumors originate from the meibomian gland, and about 10% arise from the glands of Zeis. SC typically presents in Caucasian women between the ages of 60 and 80 years. It is rare under 40 years of age unless there is a history of previous irradiation. It is usually unilateral and involves the upper lid twice as frequently as the lower. It has been reported to occur disproportionately in Asians and East Indians [2]. It mimics more common entities like a sty, chalazion, or unilateral conjunctivitis. After a well-defined therapeutic trial period, refractory lesions should be biopsied to rule out an incipient SC. In one study, about 1 in 100 clinical chalazion was a SC on biopsy [3]. Every effort should be made to diagnose and treat SC before 6 months duration. Mohs surgeons and dermatologists have an opportunity for early diagnosis because about 25% of the cases have a contemporaneous history of skin cancer. Lesions that are greater than 10 mm in size, present for more than 6 months, and involve both lids and intervening canthus have a poor prognosis.

26.1.1 Origin

Sebaceous carcinoma is a malignant neoplasm derived from cells that comprise sebaceous glands. In the orbital region, these are the meibomian glands of the tarsus and the Zeis glands associated with the lid margin cilia, caruncle, and eyebrow. Sebaceous glands are usually associated with hair follicles and the hairy parts of the body [4]. Sebaceous glands not associated with hair follicles are found also in the mucosa of the mouth (Fordyce granule) [5] and vulva regions [6] where sebaceous carcinoma is known to occur [7, 8].

26.1.2 History

Mohs micrographic surgery (MMS) for eyelid cancers was first performed by Frederic Mohs using a local anesthetic in 1953 [9]. Two decades later, two dermatologists and trainees of Dr. Mohs, Ted Tromovitch and Sam Stegeman, presented their successful series of 102 basal and squamous cell carcinomas using frozen sections (including periorbital cancers) managed by MMS [10]. The first reported case of sebaceous carcinoma of the lid treated by MMS was presented by Dixon, Mikhail, and Slater in 1980 in the Journal of the American Academy of Dermatology [11, 12]. They reported a case in an 84-year-old woman with a primary sebaceous carcinoma of the upper lid of 6 months duration. The patient was followed for 2.5 years without a recurrence. Other early Mohs surgery pioneers include Harvey and Anderson [13], Dzubow [14], Folberg et al. [15], and Ratz et al. [16]. In 1994, Yount et al. [17] presented their favorable series of 8 cases using mainly paraffin sections in close collaboration with pathology and oculoplastic service.

26.1.3 Extraorbital Sites

Sebaceous carcinoma occurs in other sites of the body. Recently, the National Cancer Institute‘s database known as the Surveillance, Epidemiology, and End Results (SEER) reported a total of 1349 cases of sebaceous carcinoma in the USA accumulated from 1974 to 2004. This database represented 26% of the population from 12 states. There were 522 cases of the eyelids; 549 cases involving the scalp, ear, lip, neck and other facial sites; and 278 cases of the trunk, extremity, genitalia, and other sites. The clinical course, pathology, management, and prognosis are similar for extraorbital and periorbital sebaceous carcinoma. Survival analysis for orbital and periorbital versus nonorbital sites supports a similar prognosis with a 5-year survival of 75.2% versus 68% [7]. Sawyer et al. [18] accumulated 130 cases of extraorbital SC from the medical literature. The metastatic rate was 18% (23 of 130). The death rate from visceral metastasis was 7% (9 of 130).

26.1.4 Incidence

Sebaceous carcinoma accounts for about 4% of malignant tumors of the periorbital region. Basal cell carcinoma accounts for about 90%, squamous cell carcinoma