Identifying malignant eyelid tumors

Malignant eyelid tumors can lead to significant ocular and visual morbidity. They present in varied histologic types. An understanding of the clinical features of common malignant eyelid tumors can be helpful in earlier diagnosis and could lead to less ocular morbidity.

Malignant eyelid lesions often require a biopsy to confirm that diagnosis, and definitive treatment involves surgery and in rare cases adjuvant radiation therapy. Let’s review the typical clinical features of the most common eyelid tumors and briefly discuss their management.

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Clinical evaluation

When evaluating a patient with an eyelid lesion, it is crucial to obtain a detailed history and perform a thorough ophthalmic examination. Important background information from history-taking include any history of previous skin cancer, excessive sun exposure, smoking, previous exposure to radiation therapy, tanning salons, or chronic immunosuppression. The duration of symptoms and signs, whether the lesion is fast or slow growing, and if the lesion is associated with symptoms of bleeding, crusting, or loss of lashes may be relevant.

On examination, careful attention should be given to the size of the lesion and its location on the eyelid, as well as the presence of ulceration, irregular pigmentation, loss of lashes, or any other distortion or destruction of the normal eyelid margin architecture. Evert the upper eyelid to rule out tarsal or palpebral involvement in the upper eyelid-even for a primarily lower eyelid mass.

External photographs of the lesion should be taken during the first exam. Ocular motility should be tested for presence of restriction which may indicate orbital extension of the eyelid lesion. Preauricular, submandibular, and cervical lymph nodes should be carefully palpated in assessing for the presence of nodal metastases.

The most common malignant eyelid lesions include basal cell carcinoma, squamous cell carcinoma, sebaceous cell carcinoma, and melanoma.

Next: Basal cell carcinoma

Basal cell carcinoma

Basal cell carcinoma represents the most common type of malignant eyelid lesions and accounts for over 90 percent of eyelid malignancies.^1,2 The most common periorbital site for basal cell carcinoma is the lower eyelid margin, followed by the inner corner of the eyelid margin (the medial canthus), the upper eyelid, and the outer corner of the eyelid margin (the lateral canthus).

Basal cell carcinoma can manifest on the eyelid in different ways. Commonly it appears as a “pearly” firm nodule with telangiectasia and signs of ulceration (Figure 1). It can also present as a firm lesion with indistinct margins which may exhibit more aggressive growth, known as the morpheaform variant. During history-taking, it is important to identify patients who may be at higher risk for developing basal cell carcinoma, including individuals with fair skin, blue eyes, and red hair, or with Irish, Scottish, or Scandinavian ancestry.

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Squamous cell carcinoma

Squamous cell carcinoma of the eyelid is the second most common type of eyelid malignacy, albeit much less common in prevalence (five to 10 percent of all eyelid malignancies) when compared to basal cell carcinoma.^3,4 There is higher prevalence among men when compared to women.

Squamous cell lesions can arise from pre-cancerous skin lesions called actinic keratosis, which present with a characteristic sandpaper-like texture. Squamous cell carcinoma may appear as an area of skin ulceration, a flat-topped lesion (plaque) or a predominantly-elevated lesion (nodule) (Figure 2).

Similar to basal cell carcinoma, the initial work-up should involve asking the patient about sun exposure, skin changes, and any active diseases or medications that could cause immunosuppression. Because there is a significant risk for lymph node and distant metastasis (~24 percent) associated with squamous cell carcinoma lesions that are large or advanced, it is recommended to perform careful palpation of lymph nodes during the physical exam.

Next: Sebaceous carcinoma

Sebaceous carcinoma

Sebaceous carcinoma usually arises from the sebaceous (oil) meibomian glands around the eyelid margin, but can also arise from sebaceous glands of skin, the caruncle, or conjunctival surface. Sebaceous carcinoma of eyelid is rare but can be highly malignant, potentially lethal, and with the potential for nodal metastasis and recurrence. Sebaceous carcinoma has been reported to be more common in women than men.⁵

Unlike basal cell carcinoma, squamous cell carcinoma, or melanoma, sebaceous carcinoma occurs more commonly in the upper eyelid due to the presence of greater number of sebaceous glands in the upper eyelid. The sebaceous carcinoma can appear as a well circumscribed nodule (Figure 3) or diffuse thickening of the eyelid, sometimes yellowish in color due to the accumulation of lipid material.

The clinician needs to be aware that sebaceous carcinoma of the eyelid is a great masquerader and can mimic benign-appearing eyelid diseases such as chalazion, blepharitis, or keratoconjunctivitis. This frequently leads to delayed or erroneous diagnosis.

During the patient work-up, it is imperative to note suspicious signs and features that could indicate malignancy, such as chronic or recurrent chalazion despite surgical drainage; lesions associated with loss of lashes or destruction of the eyelid margin; or recurrent blepharitis or conjunctivitis that is segmental or unilateral.

The risk for metastasis is higher for larger lesions. The most common first site of metastasis is the regional lymph nodes thus careful palpation of the parotid and submandibular lymph nodes as well as a systemic work-up is recommended for newly-diagnosed cases of sebaceous carcinoma.⁶

Next: Melanoma

 Melanoma

The prevalence of primary eyelid melanoma is rare. It accounts for <0.1 percent of all eyelid malignancies.^7,8 However, due to its potentially lethal nature and potential for recurrence and metastasis, prompt recognition and diagnosis is very important.

As with the work-up of other malignant eyelid lesions, a detailed history will be helpful to determine risk factors such as sunlight exposure, genetic disposition, and environmental exposure. A patient with melanoma of the eyelid typically presents with a newly-acquired pigmented lesion of the eyelid skin or eyelid margin (Figure 4). On examination, careful attention to features such as irregular borders, size and extent of lesions, variability in color and pigmentation are appropriate. The thickness of the lesion should be carefully ascertained based on an excisional or incisional biopsy as it is associated with prognosis and survival.^7,9 In addition, double-eversion of the eyelids should be performed in every patient suspected of having eyelid melanoma, to look for evidence of pigmentation on the inner eyelid surface as well as the conjunctiva.

Conclusion

Malignant eyelid lesions account for important pathology that can be recognized and diagnosed mostly based on external and slit-lamp examination. Early detection can lead to less morbidity and better outcomes. A surgical biopsy can confirm the diagnosis and followed by appropriate surgical treatments can lead to a cure in the majority of patients. Careful palpation of the regional lymph nodes and a total body skin check should be done to identify metastatic lesions or additional skin lesions.

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References

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. Margo CE, Waltz K. Basal cell carcinoma of the eyelid and periocular skin. Surv Ophthalmol. 1993 Sep-Oct;38(2):169-92.

3. Reifler DM, Hornblass A. Squamous cell carcinoma of the eyelid. Surv Ophthalmol. 1986 May-Jun;30(6):349-65.

4. Faustina M, Diba R, Ahmadi MA, . Esmaeli B. Patterns of regional and distant metastasis in patients with eyelid and periocular squamous cell carcinoma. Ophthalmology. 2004 Oct;111(10):1930-2.

5. Shields JA, Demirci H, Marr BP, Eagle RC Jr, Shields CL. Sebaceous carcinoma of the eyelids: personal experience with 60 cases. Ophthalmology. 2004 Dec;111(12):2151-7.

6. Ho VH, Ross MI, Prieto VG, Khaleeq A, Kim S, Esmaeli B. Sentinel lymph node biopsy for sebaceous cell carcinoma and melanoma of the ocular adnexa. Arch Otolaryngol Head Neck Surg. 2007 Aug;133(8):820-6.

7. Esmaeli B, Wang B, Deavers M, Gillenwater A, Goepfert H, Diaz E, Eicher S. Prognostic factors for survival in malignant melanoma of the eyelid skin. Ophthal Plast Reconstr Surg. 2000 Jul;16(4):250-7.

8. Song A, Carter KD, Syed NA, et al. Sebaceous cell carcinoma of the ocular adnexa: clinical presentations, hisotpathology, and outcomes. Ophthal Plast Reconstr Surg 2008;24:194-200.

9. Boulos PR, Rubin PA. Cutaneous melanomas of the eyelid. Semin Ophthalmol. 2006 Jul-Sep;21(3):195-206.