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Sebaceous Carcinoma - Submitted on: Thursday May, 11, 2000
Contributed by:Steven Wang, MD, Artur Zembowicz, MD
Sebaceous carcinoma (SC) is an aggressive malignant neoplasm showing sebaceous differentiations. SC occurs predominantly in the eyelids, but can also be seen in extraocular sites. First report of this entity was credited to Allaire in 1891, but this disease was not firmly accepted after 1956 when Straatsma thoroughly studied the histological and clinical presentation of this disease.111

Definition:
SC is an aggressive malignant carcinoma showing sebaceous differentiation with propensity for locally invasive behavior and metastasis. It is a dermally based and non-capsulated tumor that composed of lobules of cells with variable sebaceous differentiation. Pagetoid spread of neoplastic cells are commonly present in ocular SC.

Clinical Features:
Ocular sebaceous carcinoma occurs commonly in patients 60 to 80 years of age, but this tumor can be seen in any ages and even in childhood. The incidence of ocular lesion is slightly higher in woman and in the Asian population. The tumors are derived from the meibomian and Zeiss glands, two types of sebaceous glands in the eyelids. The upper eyelid lesions are much more prevalent. Clinically, ocular sebaceous carcinoma has wide-range of presentation, and thus, diagnosis of the tumor is often delayed. The most common presentation is a small, firm, deep-seated nodule that enlarges slowly, and it is virtually indistinguishable from a chalazion, inflammation of the meibomian and Zeiss glands.

Extraocular sebaceous carcinoma accounts for approximately 25% of all the SC. The mean age of presentation is 63 years, and there is an equal sex distribution. Lesions can occur virtually in any parts of body that contain sebaceous glands, but the two most common sites are the head and neck. Other sites include external genitalia, the parotid and submandibular glads, the trunk, and upper extremities. Clinically, the lesion presents as a firm nodule that ranges in size from 6 mm to 20 cm.


Histology:
Figures
(Click on an image for a larger view)
Figure 1 Ulcerated sebaceous carcinoma
Figure 2 Cytological atypia and mitotic activity in sebaceous carcinoma.
Figure 3 Pagetoid spread in sebaceous carcinoma
SC is a dermally based and non-encapsulated basaloid tumor diagnosis rests on identification of sebaceous differentation within the tumor (Figure 1).

SC is associated with high degree of cytological atypia and mitotic activity (Figure 2).

Tumor necrosis is often present. Four typical growth patterns (i.e., lobular, comedo-carcinoma, papillary and mixed) have been described. In the lobular pattern, the tumor is usually composed of lobules or sheets of cells that extend and infiltrate to the deep subcutis and skeletal muscles. The cells show variable sebaceous differentiation where the cytoplasm has the foamy and vacuolated appearance, and the sebaceous differentiation is more prominent in the cells in the center of the lobules. Typically, the cells have large nuclei and nucleoli, and scattered mitosis can be seen. The comedo-carcinoma pattern exhibits large lobules with central foci of necrosis. The papillary pattern shows projection of cells resembling those in a squamous cell carcinoma with or without focal keratinization. The mixed type consists of any combination of the three mentioned patterns. Pagetoid intraepidermal spread of malignant cells with sebaceous differentiation is a common feature (Figure 3).


Treatment:
Surgical excision or Mohs micrographic surgery are standard treatments for primary SC. For metastatic disease, excision combined with radiation and/or chemotherapy is applicable.

Bibliography:
  • Nelson BR, Hamlet R, Gillard M et al. Sebaceous carcinoma. J. Am Acad Dermatol. 1995; 33: 1-15.
  • Wick MR, Goellner JR, Wolfe JT et al. Adnexal carcinoma of the skin, II Extraocular sebaceous carcinomas. Cancer. 1985; 56: 1163-1172.
  • Weadon, D, Skin Pathology. (1998) Churchill Livingstone, p727-728.
  • Elder D, Elenitsas R, Ragsdale BD. Tumors of the epidermal appendages. In: Elder D, Elenitsas R, Jaworsky C et al., eds Leverís Histopathology of the skin. 8th ed. Philadelphia: Lipppincott-Raven Publishers. P768-769
     

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