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Chapter 9

Management of Primary Eyelid Cancers

Aaron Savar and Bita Esmaeli

Abstract A number of different cancers can affect the eyelid. Basal cell carcinoma is the most common; squamous cell carcinoma, melanoma, and sebaceous cell carcinoma are less common. Management of these tumors is most commonly surgical, although chemotherapy and radiation therapy play a role in some cases. Complex tumors require a multidisciplinary approach and long-term follow-up.

9.1 Introduction

The spectrum of cancers affecting the eyelids is wide. Primary eyelid malignancies can arise from virtually any cell type, including epidermal tissues, glandular tissues, connective tissues, or lymphocytes. The management of such lesions depends on the type, location, and size of the tumor.

9.2 Types of Eyelid Malignancies

9.2.1 Basal Cell Carcinoma

Basal cell carcinoma (BCC) is the most common type of cancer and accounts for over 90% of eyelid cancers. A large cohort study of eyelid malignancies found an incidence of 14.35 per 100,000 individuals per year [1]. Sun exposure, increased age, immunosuppression, and decreased skin pigmentation are known risk factors. BCC can also be seen in association with xeroderma pigmentosum and Gorlin–Goltz syndrome.

A. Savar (B)

Section of Ophthalmology, Department of Head and Neck Surgery, The University of Texas M.D. Anderson Cancer Center, Houston, TX, USA

e-mail: asavar@gmail.com

B. Esmaeli (ed.), Ophthalmic Oncology, M.D. Anderson Solid Tumor

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Oncology Series 6, DOI 10.1007/978-1-4419-0374-7_9,

C Springer Science+Business Media, LLC 2011

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Fig. 9.1 (a) Basal cell carcinoma (BCC) of lower eyelid. (b) Histologic section of nodular BCC. Note nests of cells with peripheral palisading

BCC lesions can present in a number of ways. Common clinical findings include pearly papules, telangiectatic vessels, and skin ulceration (Fig. 9.1a). The most common periocular locations of BCC are the lower eyelid, the medial canthus, the upper eyelid, and the lateral canthus, in that order. Lesions of the medial canthus have a higher risk of orbital involvement.

Local invasive growth is common if lesions are left untreated. Metastasis is rare and usually occurs only in tumors greater than 3 cm in diameter. In a review of 238 BCCs that metastasized, 12% were in the periocular region [2].

BCCs arise from cells in the stratum basale of the epidermis. Histopathologically, they are characterized by nests of cells with peripheral palisading. There are several pathologic variants described, with nodular BCC being the most common (Fig. 9.1b). Morpheaform BCCs can present with indistinct borders and have more aggressive behavior. Perineural invasion can be observed with BCC and should be treated with postoperative adjuvant radiation therapy.

9.2.2 Squamous Cell Carcinoma

Squamous cell carcinoma (SCC) is the second most common eyelid cancer, accounting for between 5 and 10% of eyelid malignancies in some large studies [3]. As with BCC, sun exposure, increased age, decreased skin pigmentation, and immunosuppression are known risk factors, as is exposure to certain chemicals. SCC is seen more commonly in men than women. SCC is known to arise from actinic keratoses.

SCC can present as an ulcer, a plaque, or a nodule (Fig. 9.2a). Lesions are more common in the lower eyelid. Perineural invasion can be seen with these tumors and can result in pain, paresthesia, or numbness.

The risk of lymph node and distant metastasis is higher than that with BCC. The rate of nodal metastasis has been reported to be as high as 24% with large and

9 Management of Primary Eyelid Cancers

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Fig. 9.2 (a) Squamous cell carcinoma (SCC) of the lower eyelid. (b) Histologic section of invasive SCC

advanced lesions [4]. The risk of nodal metastasis is higher with lesions that are greater than 1 cm in diameter.

SCCs arise from keratinocytes and are characterized by strands and nests of abnormal squamous cells (Fig. 9.2b).

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9.2.3 Melanoma

 

Primary melanomas of the eyelid are rare but potentially lethal. They account for fewer than 1% of eyelid malignancies. They usually present as pigmented lesions with irregular borders and color.

Melanomas arise from melanocytes. In patients with eyelid melanomas, as in patients with melanomas located elsewhere in the body, increased Clark level and Breslow thickness have been shown to be associated with decreased survival [5]. Pathologic subtypes include nodular, superficial spreading, lentigo maligna, and acral lentiginous melanoma and also amelanotic melanoma, a rare variant.

The risk of nodal and systemic metastasis is high and is correlated with Breslow thickness, Clark level, and presence of ulceration, as is the case for cutaneous melanoma in other anatomic sites. A systemic workup for metastasis is recommended for melanomas of intermediate thickness or greater.

9.2.4 Sebaceous Gland Carcinoma

Sebaceous gland carcinoma is a rare, potentially lethal eyelid tumor with a high rate of recurrence and a propensity for metastasis. Prior radiation exposure is a known risk factor. Sebaceous gland carcinoma is also seen in patients with Muir–Torre syndrome.

Diagnosis can often be delayed in sebaceous gland carcinoma as these lesions can be confused with benign lesions. In a series of 60 sebaceous gland carcinomas, over two-thirds were thought initially to be another less serious lesion [6]. Early diagnosis and treatment is critical. In a study of 31 patients, the median time from onset of symptoms to diagnosis was 24 months (range, 1–300 months) [7].

These lesions typically present as a nodule or as diffuse eyelid thickening, and unlike BCC, SCC, and melanoma, sebaceous gland carcinoma is more common in the upper eyelid than the lower eyelid (Fig. 9.3a). Sebaceous gland carcinomas can occur on the bulbar conjunctiva, although this is very rare.

Sebaceous gland carcinoma arises in the eyelids from the meibomian glands or glands of Zeis, or on the caruncle from sebaceous glands present there. Foamy cytoplasm is noted upon histopathologic examination. Special stains, such as

Fig. 9.3 (a) Clinical photograph of sebaceous carcinoma of the upper eyelid. (b) Histologic section of the same lesion. (c) Oil Red O stain confirms the diagnosis of sebaceous carcinoma