You are in: eMedicine Specialties > Ophthalmology > LID Sebaceous Gland CarcinomaArticle Last Updated: Aug 16, 2006AUTHOR AND EDITOR INFORMATIONSection 1 of 11
  Author: Michael L Glassman, MD, Ophthalmic Plastic, Orbital and Reconstructive Surgery, Department of Ophthalmology, Oculoplastic and Orbital Surgery, New York Eye and Ear Infirmary, Manhattan Eye Ear and Throat Hospital Michael L Glassman is a member of the following medical societies: American Academy of Ophthalmology Coauthor(s): Mounir Bashour, MD, CM, FRCS(C), PhD, FACS, Assistant Professor of Ophthalmology, McGill University; Clinical Assistant Professor of Ophthalmology, Sherbrooke University; Medical Director, Cornea Laser and Lasik MD; Mirelle Benchimol, MD, Consulting Staff, Benchimol Eye Clinic Editors: Jorge G Camara, MD, Chairman, Department of Ophthalmology and Otorhinolaryngology, Director of Fellowship Training Program, St Francis Medical Center; Associate Professor, Department of Surgery, University of Hawaii School of Medicine; Simon K Law, MD, PharmD, Assistant Professor of Ophthalmology, Jules Stein Eye Institute; Chief of Section of Ophthalmology Surgical Services, Department of Veterans Affairs Healthcare Center, West Los Angeles; Mark T Duffy, MD, PhD, Consulting Staff, Division of Oculoplastic, Orbito-facial, Lacrimal, and Reconstructive Surgery, Green Bay Eye Clinic, BayCare Clinic; Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri; Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences Author and Editor Disclosure Synonyms and related keywords: meibomian gland carcinoma, sebaceous carcinoma, sebaceous cell carcinoma, carcinoma of meibomian and Zeis glands, sebaceous hyperplasia, sebaceous gland adenomas, eyelid malignancy INTRODUCTIONSection 2 of 11
  Sebaceous glands are part of epidermal appendages. Neoplasms of the sebaceous glands may be benign, such as sebaceous hyperplasia or sebaceous gland adenomas. The malignant sebaceous gland carcinoma most commonly arises in the periocular area. Fewer than 120 cases of sebaceous cell carcinoma have been reported at extraocular sites. The most common site of origin is the meibomian glands of the eyelids, leading to the term meibomian gland carcinoma. However, this neoplasm can occur in other sebaceous glands, such as in the caruncle, the glands of Zeis, and in the eyebrow. Sebaceous cell carcinoma is a lethal eyelid malignancy and can masquerade as benign conditions. Error or delay in diagnosis is common, and this tumor carries a significant mortality rate with metastasis. History of the ProcedureThis type of tumor has been confused with other tumors both clinically and histologically. FrequencyIncidence of sebaceous cell carcinoma is 3.2% among malignant tumors and 0.8% of all lid tumors. The mortality rate is 22%. Sebaceous cell carcinomas are typically found in women, more often in the seventh decade of life, and they usually are on the upper lid margin. Although sebaceous cell carcinomas are more common in elderly patients, they may be seen in younger patients with a history of radiation to the face. EtiologyCarcinoma of the meibomian and Zeis glands PathophysiologySebaceous cell carcinoma may mimic either a chalazion or chronic blepharitis. It tends to invade locally, as well as spreading to regional lymph nodes. Sebaceous cell carcinomas may grow in nests with central necrosis. The intraepithelial spread may lead to the erroneous histologic diagnosis of epithelial dysplasia or carcinoma in situ. Foamy cytoplasm is seen only in sebaceous carcinoma, but it is absent in conjunctival or cutaneous squamous cell carcinoma. It also can histologically mimic basal cell carcinoma, squamous carcinoma, or Merkel cell tumor. Either fresh tissue or formalin-fixed tissue not exposed to alcohol can be frozen, and positive fat stains such as oil red O can confirm the diagnosis of sebaceous carcinoma. ClinicalThe clinical appearance of sebaceous gland carcinoma is highly variable. They simulate such benign conditions as chalazion, blepharoconjunctivitis, keratitis, and other malignant or benign skin lesions. Many of the skin tumors have a predilection for the upper lid and have a yellowish appearance. Tumors at the lid margin commonly cause loss of eyelashes. Classically, this lesion is a firm, painless, indurated mass or ulceration associated with the loss of cilia, in an area that has been treated for recurrent chalazia. Both the history and the presentation of sebaceous cell carcinoma are variable. Typically, there is an insidious onset of a painless firm eyelid mass. This mass easily can appear clinically as a recurrent or chronic chalazion. Sebaceous cell carcinoma also can mimic unilateral blepharoconjunctivitis, meibomitis, basal or squamous cell carcinoma, conjunctival or corneal carcinoma in situ, orbital inflammation, or superior limbic keratoconjunctivitis. INDICATIONSSection 3 of 11
Perform a biopsy on all recurrent or resistant chalazia, or new nonresolving lesions of the eyelid. RELEVANT ANATOMYSection 4 of 11
Sebaceous tumors of the eyelid may arise from meibomian glands, Zeis glands, or glands associated with the caruncle. The meibomian glands originate in the tarsus and number approximately 25 in the upper eyelid and 20 in the lower eyelid. They are oil-producing sebaceous glands. A chalazion is an inflammation caused by obstruction of the meibomian glands and occasionally can be confused with sebaceous cell carcinoma. Zeis glands are small, modified, sebaceous glands that open into the hair follicles at the base of the eyelashes. CONTRAINDICATIONSSection 5 of 11
There are no contraindications. This tumor can masquerade as other tumors both benign and malignant. WORKUPSection 6 of 11
Diagnostic Procedures
Histologic FindingsThe histopathology of sebaceous gland cell carcinoma is dominated by large anaplastic cells with open vesicular nuclei and prominent nuclei set in foamy or frothy cytoplasm. The appearance of the cytoplasm is a result of the presence of lipid vacuoles. A useful histopathologic feature is large nuclear forms, including multinucleated tumor cells and bizarre monstrous cell forms. The spread of the tumor in the form of infiltrating lobules, nests, and cords, as well as superficially within the epithelium is highly characteristic. TREATMENTSection 7 of 11
Surgical therapyTreatment aims to remove the malignant lesion to prevent local or systemic spread. The treatment of sebaceous gland carcinoma is adequate surgical excision, with wide surgical margins and fresh frozen section controls to delineate the tumor edges. Lymph node evaluation is necessary to evaluate metastasis. If diffuse involvement of both the upper and lower lids is present, exenteration is required. Perform a biopsy on areas of reddening of the conjunctiva that are suggestive of sebaceous gland carcinoma at the time of surgery. Follow-upMonitoring for additional malignancies or metastatic sites is warranted. A marked increase in head and neck basal cell lesions is found in patients with previous eyelid malignancies. As many as 40% of patients also may have had or may develop other visceral malignancies. COMPLICATIONSSection 8 of 11
Orbital invasion and lymph node metastases have occurred. OUTCOME AND PROGNOSISSection 9 of 11
With wide excision and no evidence of metastasis, surgery results in a cure for the malignancies. However, sebaceous lesions have a high incidence of recurrence and metastasis. The Mohs chemosurgery method commonly is used in facial and periocular nonmelanocytic malignancies. This method allows the focal and complete removal of a tumor with histologically verified margins. However, in diffuse tumors with high recurrence rates, such as sclerosing sweat duct carcinoma, recurrence rates may be as high with the Mohs chemosurgery method as with the traditional methods. In histologically verified sebaceous gland carcinoma, wide complete (ie, full thickness) resection is necessary. Sending additional margins for permanent sections as well as frozen section margins may increase success. Since multiple widespread adnexal biopsies need to be performed, the Mohs chemosurgery method probably is not warranted in most situations of sebaceous cell carcinoma. MULTIMEDIASection 10 of 11
REFERENCESSection 11 of 11
Sebaceous Gland Carcinoma excerpt Article Last Updated: Aug 16, 2006 | ||||||||||||||||||||||||||
