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Disease

Basal Cell Carcinoma (BCC) is a malignant epidermal carcinoma. BCC is the most common eyelid malignancy, accounting for over 90% of malignant eyelid neoplasms.

Etiology

Ultraviolet light (particularly UVB) induced damage to the epidermis.

Risk Factors

BCC tends to occur in lightly pigmented individuals at sites of sun exposure, although it may occur with any skin tone.

Other risk factors include:

Radiation
Immunosuppression, including solid organ transplant
Previous scar
Inherited syndromes, e.g., xeroderma pigmentosum

General Pathology

Figure 1. Basal cell carcinoma of the eyelid. Image courtesy of Marcus M. Marcet, MD FACS.

Figure 2. Basal cell carcinoma of the eyelid, morpheaform type. Image courtesy of Marcus M. Marcet, MD FACS.

As its names implies, BCC derives from cells of the epithelial basal cell layer. Histologically the tumor has an appearance similar to the normal epithelial basal cell layer (Figure 1). BCC forms strands, cords, and islands of tumor. Palisading of the nuclei at the periphery of the islands of tumor is characteristic (Figure 1). An additional distinguishing feature of the tumor is the clefts or separation artifact, which results from tissue processing. Both nodular and morpheaform tumor growth types are seen in the periocular region.

The morpheaform type, also known as sclerosing or fibrosing type, characteristically has small islands of tumor within dense fibrous tissue (Figure 2). The nests and strands of tumor tend to invade the underlying tissue more deeply. The more aggressive pattern of the morpheaform type presents a challenge in determining the extent of the tumor; the variant carries a higher rate of recurrence.

Pathophysiology

Defects of the PTCH gene located on chromosome 9q22.3 have been associated with the development of BCC.

Primary prevention

Minimize sun exposure by use of sun block products, as well as hats, sunglasses, and appropriate clothing.

Diagnosis

The diagnosis of BCC can be suspected clinically and is confirmed histologically following incisional biopsy.

History

The history for any suspicious periocular mass lesion should include assessment for any of the risk factors for BCC. The location of the lesion is noteworthy. Due to a relatively greater amount of sun exposure, BCC has a predilection for the lower eyelid, followed by the medial canthus.^[1]

Physical examination

Complete eye examination, including ocular motility and assessment of proptosis to assess for orbital extension. This is especially relevant for medial canthal lesions, as they are more likely to extend into the orbit.

Assessment of facial sensation

Examination of the lesion includes assessment for:

General appearance and extent of the lesion and periocular skin
Distortion of eyelid architecture or eyelid malposition
Presence of skin ulceration
Madarosis (loss of eyelashes)
Telangiectasias

Symptoms

May be asymptomatic
Ulceration and bleeding
Non-healing skin lesion
Skin crusting
Paresthesia or anesthesia
Eyelash loss (madarosis)
Distortion of normal eyelid margin or tissue architecture

Diagnostic procedures

Biopsy (incisional or excisional, depending on the size and location).

Differential diagnosis

The differential diagnosis includes any benign or malignant condition of the eyelid skin, including:

Benign

Seborrheic keratosis
Actinic keratosis
Chalazion
Cyst
Squamous papilloma
Blepharitis
Xanthelasma
Nevus
Verruca
Sebaceous hyperplasia

Malignant

Squamous cell carcinoma
Sebaceous gland carcinoma
Malignant melanoma
Lymphoma
Merkel cell tumor
Metastasis

Management

BCC is nearly always a locally invasive disease. Treatment is recommended to prevent damage to neighboring tissues.

Medical therapy

Treatment with topical imiquimod 5% cream has been shown to be effective, although not as effective as surgical excision, in several case series.^[2] More aggressive BCC has also been successfully treated with medication that targets the sonic hedgehog pathway, including vismodegib. Further studies are needed to evaluate the extent of histopathologic resolution or changes from vismodegib. ^[3]

Surgery

Complete surgical excision with margin control. Assuring that the surgical margins are without cancerous cells may be accomplished by frozen sectioning or Mohs surgery.

Complications

Recurrence of the tumor.

Prognosis

Five-year cure rates of up to 98% have been reported for BCC. There is a worse prognosis with:

Lesions greater than 3 cm
Long-standing lesions
Deeply invasive tumors
Inadequate treatment

Additional Resources

References

↑ Cook BE Jr., Bartley GB. Epidemiologic characteristics and clinical course of patients with malignant eyelid tumors in an incidence cohort in Olmsted County, Minnesota. Ophthalmology. 1999 Apr;106(4):746-50.
↑ Beutner KR1, Geisse JK, Helman D, Fox TL, Ginkel A, Owens ML. Therapeutic response of basal cell carcinoma to the immune response modifier imiquimod 5% cream. J Am Acad Dermatol. 1999 Dec;41(6):1002-7.
↑ Gill HS, Moscato EE, Chang AL, Soon S, Silkiss RZ. Vismodegib for periocular and orbital basal cell carcinoma. JAMA Ophthalmol. 2013 Dec;131(12):1591-4. doi: 10.1001/jamaophthalmol.2013.5018.

Ophthalmic Pathology and Intraocular Tumors, Section 4. Basic and Clinical Science Course. San Francisco: American Academy of Ophthalmology; 2009.
Orbit, Eyelids, and Lacrimal System, Section 7. Basic and Clinical Science Course. San Francisco: American Academy of Ophthalmology; 2009.
Sehu KW, Lee WR. Ophthalmic Pathology. Oxford: Blackwell Publishing, 2005: 25, 27-30.
Srivastava M, Kelley L. Mohs' Surgery for Eyelid Malignancies. In: Albert DM, Jakobiec FA, eds. Principles and Practice of Ophthalmology, 3rd ed. Philadelphia: W.B. Saunders, 2008;3:248.
Valenzuela AA, Sullivan TJ. Basal Cell Carcinoma in the Eyelid. In: Albert DM, Jakobiec FA, eds. Principles and Practice of Ophthalmology, 3rd ed. Philadelphia: W.B. Saunders, 2008;3:249.
Yanoff M, Sassani JW. Ocular Pathology, 6th ed. Philadelphia: Mosby Elsevier, 2009: 197-199.

[1] Cook BE Jr., Bartley GB. Epidemiologic characteristics and clinical course of patients with malignant eyelid tumors in an incidence cohort in Olmsted County, Minnesota. Ophthalmology. 1999 Apr;106(4):746-50.

[2] Beutner KR1, Geisse JK, Helman D, Fox TL, Ginkel A, Owens ML. Therapeutic response of basal cell carcinoma to the immune response modifier imiquimod 5% cream. J Am Acad Dermatol. 1999 Dec;41(6):1002-7.

[3] Gill HS, Moscato EE, Chang AL, Soon S, Silkiss RZ. Vismodegib for periocular and orbital basal cell carcinoma. JAMA Ophthalmol. 2013 Dec;131(12):1591-4. doi: 10.1001/jamaophthalmol.2013.5018.

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