eMedicine Specialties > Dermatology > Malignant Neoplasms

Sebaceous Carcinoma

Author: James M Spencer, MD, Professor of Clinical Dermatology, Mount Sinai School of Medicine, New York; Private Practice, Spencer Dermatology, St Petersburg, Florida
Coauthor(s): Amy Lynn Basile, DO, MPH, Sun Coast Hospital/Largo Medical Center, Largo, Florida
Contributor Information and Disclosures

Updated: Feb 19, 2009

Introduction

Background

Sebaceous gland carcinoma is an aggressive, uncommon, cutaneous tumor first well-described by Allaire in 1891.1 This tumor is thought to arise from sebaceous glands in the skin and, thus, may arise anywhere on the body where these glands exist, including the genitalia.2,3,4 Approximately 75% of these tumors arise in the periocular region, an area rich in a variety of types of sebaceous glands.5,6 This tumor exhibits an aggressive clinical course, with a significant tendency for both local recurrence and distant metastasis.

Diagnosis and therapy tend to be delayed because sebaceous carcinoma is frequently mistaken for more common benign entities, further complicating treatment of this aggressive malignancy.7,8,9,10 In addition to its varied clinical appearance, a varied histologic appearance may occur, and delayed diagnosis or misdiagnosis following a biopsy is not uncommon.7,8,9

When arising in the periocular region, the clinical presentation is often variable, and sebaceous gland carcinoma is often not initially suspected. Instead, patients may receive multiple courses of incision and drainage for chalazion before a definitive biopsy is performed.7,11

Most sebaceous gland carcinomas have no obvious etiology. Only a few are associated with Muir-Torre syndrome. Although sebaceous adenoma and epithelioma are more specific markers for Muir-Torre syndrome, an evaluation for this syndrome is advisable once sebaceous gland carcinoma is diagnosed.12 In approximately 40% of cases, patients with Muir-Torre syndrome develop some type of sebaceous tumor before or concurrent with visceral malignancy.13

Pathophysiology

Modern tumor classification schemes name tumors for the type of normal adult tissue toward which the tumor appears to be differentiating. The cell of origin is often not known. Sebaceous gland carcinoma clearly resembles normal sebaceous glands.14 One may reasonably speculate that sebaceous gland carcinoma arises from mature sebaceous glands. Histologic studies have suggested that periocular sebaceous gland carcinomas arise from the sebaceous glands in this region. The following 5 types of sebaceous glands are seen in the periocular region5,6 :

  • Meibomian glands of the tarsal plate
  • Glands of Zeis of the cilia
  • Sebaceous glands of the eyebrows
  • Glands of the caruncle
  • Glands of the fine hair follicles of the eyelid surface
In one histologic series, 51% of cases reportedly arose from a specialized sebaceous gland of the eyelid, the meibomian gland. Indeed, sebaceous gland carcinoma is sometimes referred to as meibomian gland carcinoma. In the same series, 10% of cases arose from the glands of Zeis, less than 10% of cases arose from the caruncle and the eyebrow each, and 12% were multicentric with no obvious source of origin.15

Isolated case reports describe sebaceous gland carcinoma limited to the epithelium, with no obvious connection to the underlying sebaceous glands.16 In these rare cases, the sebaceous gland carcinoma may fill the conjunctival epithelium and create the appearance of squamous cell carcinoma in situ. Whether these tumors truly have an epithelial origin or whether the dermal connection has been lost or is simply unappreciated is unknown.

Approximately 20 case reports have described the development of sebaceous carcinoma in a sebaceous nevus of Jadassohn.17,18,19,20,21 Sebaceous carcinoma arising from a nevus sebaceous is more common in women and elderly persons, described as a nodule or ulcerated tumor that usually demonstrates rapid growth prior to diagnosis.19 Controversy exists regarding the prophylactic removal of a nevus sebaceous, but when malignant neoplasms are suspected, removal is warranted.22

Frequency

United States

Sebaceous gland carcinoma is a rare tumor. Approximately 75% of sebaceous gland carcinomas occur in the periocular region.5 In this region, sebaceous gland carcinoma represents 1-5.5% of eyelid malignancies, fourth after basal cell carcinoma, squamous cell carcinoma, and melanoma.15,23,24

International

Sebaceous gland carcinoma seems to occur with greater frequency relative to other skin cancers in Asian populations. In a large retrospective series from China, sebaceous gland carcinoma was the second most common periocular tumor after basal cell carcinoma, reported to represent 33% of eyelid malignancies.24

Mortality/Morbidity

Sebaceous gland carcinoma is an aggressive tumor, with a tendency for both local recurrence and distant metastasis.

  • Reported local recurrence rates range from 9-36%, with larger series reporting recurrence rates in the 30% range. Local recurrence tends to occur within 5 years.5,9
  • The rate of metastasis in extraocular and ocular sebaceous carcinoma is thought to be similar, occurring in 14-25% of cases, first to the draining lymph nodes and then to distant sites.7,15,25 Sites of distant metastasis include the liver, lungs, bones, and brain.7,15,26 Metastasis has been reported to occur as late as 5 years after the initial diagnosis, lending support to the continual surveillance of patients with sebaceous carcinoma.26

Sex

Women tend to be affected somewhat more often than men, with 57-77% of patients being women in several large series.7,14,15,27,28,29

Age

Most patients present in their sixth or seventh decade of life, although the range is from early childhood through the nineties.14,30 The youngest reported case arose in a 3-year-old child.31

Clinical

History

  • The presentation of sebaceous gland carcinoma is often nonspecific, and a noncancerous condition of the periocular area may be mistakenly assumed. A biopsy-confirmed diagnosis is typically delayed for months, and even years, after the patient becomes aware of the condition.9,28
  • Most often, a painless nodule develops on the eyelid, and the patient receives treatment for the far more common benign chalazion.5,7,11

Physical

Sebaceous gland carcinoma has a varied clinical presentation that often results in delayed diagnosis.

  • Tumors of the upper lid are 2-3 times more common than lesions of the lower lid.5,15,27,29
  • The most common presentation is a firm, slowly enlarging nodule of the upper eyelid, often mistaken for a chalazion.5,7,11 Patients are not uncommonly treated for many months for recurrent chalazia before a biopsy is performed.

In a variety of series, the delay in diagnosis following presentation ranges from 1-5 years.9,28

  • Loss of cilia is a clinical clue that the lesion is malignant.29
  • One study reported 7 of 31 patients presented with a characteristic “tigroid” pattern of the conjunctiva, described as yellow streaks (lipid material from the meibomian glands) within an area of papillary hypertrophy.9
  • Clinical presentation can mimic keratoconjunctivitis, aroconjunctivitis,7,11 squamous cell carcinoma, basal cell carcinoma, cutaneous horn,32 sarcoidosis, ocular pemphigoid, and a variety of benign and malignant ocular tumors.5,9
  • Extraocular sebaceous gland carcinoma represents 25% of reported tumors.33
  • The head and the neck are where most extraocular sebaceous gland carcinomas occur, with the parotid gland alone representing 30% of cases.33
  • Rare reports describe tumors arising in virtually every area of the body. Unusual anatomical locations reported in association with sebaceous carcinoma include the genitalia,3,5 the parotid gland,34 the ear canal,35 the breast,36,37 and the intraoral cavity2
  • The clinical presentation is nonspecific and is usually described as a nodule that is pink to yellow-red.5

Causes

The cause of sebaceous gland carcinoma remains unclear. No association with ultraviolet radiation has been documented, but a history of ionizing radiation has been reported. Reports of prior radiation therapy for a variety of benign and malignant conditions include radiation for cavernous hemangioma, barber's itch, and retinoblastoma.30,38,39

  • In one series of 20 patients with sebaceous gland carcinoma, 8 patients had a history of diuretic use, and a possible association was suggested.8
  • Sebaceous gland carcinoma seems to be more common in Asian populations than in other populations, and involvement of human papillomavirus (HPV) has been suggested as a possible etiologic factor in these populations.
    • One paper from Japan reported the presence of HPV DNA in some sebaceous gland carcinomas, as well as an overexpression of TP53.40  
    • A study from the United States failed to detect HPV, but it did find overexpression of TP53.41
  • Experimental evidence in animals has implicated chalazia as a possible factor in the development of sebaceous gland carcinoma.
    • Chalazia are caused by inflammation of the meibomian glands or glands of Zeis that results in the formation of a hard, usually painless, nodule in the eyelid.
    • Histologically, chalazia contain granulomatous inflammation, areas of caseation necrosis, and an unsaturated 8-carbon fatty acid called oleic acid. Animal studies have suggested possible carcinogenicity with exposure to oleic acid, and prolonged exposure to this molecule within chalazia may induce dysplastic growth in glandular structures.
  • Genetic factors clearly play a role, because sebaceous gland carcinomas are part of the genodermatosis Muir-Torre syndrome.
    • Muir-Torre syndrome is a rare autosomal dominant condition with variable penetrance characterized by skin manifestations, including benign and malignant sebaceous neoplasms, keratoacanthomas, and internal manifestations (eg, colonic polyps, low-grade visceral malignancies).12
    • A diagnosis of Muir-Torre syndrome requires the presence of both an internal malignancy and a sebaceous neoplasm.12 The sebaceous neoplasms encompass a spectrum from well-differentiated sebaceous hyperplasia through undifferentiated sebaceous gland carcinoma.12
    • In one literature review of 120 patients with Muir-Torre syndrome, 29 had sebaceous gland carcinoma, 50% of which were ocular.13
    • Sebaceous neoplasms developed before or concurrent with visceral malignancies in 41% of patients in one series.13
    • Colorectal carcinoma is the most common internal malignancy in Muir-Torre syndrome, followed by genitourinary malignancy.13
    • A variety of internal malignancies, including head and neck, small bowel, and hematologic, occur less frequently in Muir-Torre syndrome.
    • Sebaceous gland carcinoma is clearly part of Muir-Torre syndrome. However, the percentage of patients with sebaceous gland carcinoma who will develop Muir-Torre syndrome is not clear.12
    • All patients with sebaceous gland carcinoma should be evaluated for Muir-Torre syndrome.

More on Sebaceous Carcinoma

Overview: Sebaceous Carcinoma
Differential Diagnoses & Workup: Sebaceous Carcinoma
Treatment & Medication: Sebaceous Carcinoma
Follow-up: Sebaceous Carcinoma
References
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Further Reading

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Keywords

sebaceous carcinoma, sebaceous gland carcinoma, meibomian gland carcinoma, sebaceous cell carcinoma

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Contributor Information and Disclosures

Author

James M Spencer, MD, Professor of Clinical Dermatology, Mount Sinai School of Medicine, New York; Private Practice, Spencer Dermatology, St Petersburg, Florida
James M Spencer, MD is a member of the following medical societies: American Academy of Cosmetic Surgery, American Academy of Dermatology, American College of Mohs Micrographic Surgery and Cutaneous Oncology, American Dermatological Association, American Medical Association, American Society for Dermatologic Surgery, American Society for Laser Medicine and Surgery, and International Society for Dermatologic Surgery
Disclosure: Graceway Pharmaceutical Honoraria Speaking and teaching; Sanofi Aventis Honoraria Consulting; Medicis Grant/research funds Other

Coauthor(s)

Amy Lynn Basile, DO, MPH, Sun Coast Hospital/Largo Medical Center, Largo, Florida
Amy Lynn Basile, DO, MPH is a member of the following medical societies: American Medical Association, American Osteopathic Association, and American Osteopathic College of Dermatology
Disclosure: Nothing to disclose.

Medical Editor

Kelly M Cordoro, MD, Fellow and Clinical Instructor, Department of Pediatric Dermatology, University of California at San Francisco; Assistant Professor (On Educational Leave), Assistant Program Director for Resident Medical Education, Department of Dermatology, University of Virginia School of Medicine
Kelly M Cordoro, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Association of Professors of Dermatology, Dermatology Foundation, Medical Society of Virginia, National Psoriasis Foundation, Society for Pediatric Dermatology, and Women's Dermatologic Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA
Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

John G Albertini, MD, Consulting Staff, Dermatologic Surgery, The Skin Surgery Center
John G Albertini, MD is a member of the following medical societies: American Academy of Dermatology and American College of Mohs Micrographic Surgery and Cutaneous Oncology
Disclosure: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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