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TUMORS OF THE EYELIDS

BENIGN LID TUMORS

Benign tumors of the lids are very common and increase in frequency with age. Most are readily distinguished clinically, and excision is done for cosmetic reasons. However, it is often impossible to recognize malignant lesions clinically, and biopsy should always be performed if there is any doubt about the diagnosis.

Nevus

Melanocytic nevi of the eyelids are common benign tumors with the same pathologic structure as nevi found elsewhere. They initially may be relatively unpigmented, enlarging and darkening during adolescence. Many never acquire visible pigment, and many resemble benign papillomas. Nevi rarely become malignant.

Nevi may be removed by shave excision if desired for cosmetic reasons.

Papillomas

Papillomas are the most common benign eyelid tumors. Two types occur: squamous cell papillomas and seborrheic keratoses (basal cell papillomas, senile verrucae). In both, fibrovascular cores permeate thickened (acanthotic and hyperkeratotic) surface epithelium, giving it a papillomatous appearance. Seborrheic keratoses occur in middle-aged and older individuals. They have a friable, verrucous surface and are often pigmented because melanin accumulates in the keratocytes.

Verruca Vulgaris

Another papillomatous, hyperkeratotic nodule that may occur on the skin of the face and eyelids is due to the wart virus, a deoxyribonucleic acid (DNA) virus in the papovavirus group.

Molluscum Contagiosum (Figure 4-9)

This tumor, due to a poxvirus, is a small, dome- shaped nodule, often umbilicated centrally. Lid margin lesions may be minute and partially hidden in the cilia but may produce conjunctivitis and even keratitis if the lesion sheds into the conjunctival space.

Cure can usually be achieved by curettement, cautery, or excision.

Keratoacanthoma

Keratoacanthomas are benign inflammatory tumors occurring in sun-exposed skin of adults. Occasionally they are associated with immunodeficiency, xeroderma pigmentosum, or the Muir-Torre syndrome. Many will undergo spontaneous involution, but excisional biopsy is often indicated for cosmetic reasons or to rule out the possibility of squamous cell carcinoma, which they may mimic both clinically and histologically.

Xanthelasma (Figure 4-10)

Xanthelasma is a common disorder that occurs on the anterior surface of the eyelid, usually bilaterally near the inner angle of the eye. The lesions appear as yellow, wrinkled patches on the skin and occur most commonly in elderly people. Xanthelasma represents lipid deposits in histiocytes in the dermis of the lid. They occur in patients with hereditary hyperlipidemia and in diabetics and other patients with secondary hyperlipidemia, but approximately two-thirds of patients with xanthelasma have normal serum lipids.

Treatment is indicated for cosmetic reasons. Lesions can be excised, cauterized, or treated with laser surgery. Recurrence following removal is not unusual.

Cysts

Cysts in the eyelids are common. Keratinous cysts, lined by epithelium and filled with cheesy-looking keratin and debris, are the result of obstruction of pilosebaceous structures (milia and pilar cysts), or congenital and traumatic subepithelial implantation of surface epithelium (epidermal inclusion cysts).

Dermoid cysts, with adnexal structures such as hair follicles and sebaceous glands in the walls and with hair as well as keratin in the lumen, are congenital but may not become apparent until later in life, when they increase in size or rupture, eliciting a granulomatous inflammatory response. Most are located near the orbital rim superotemporally, and many are associated with a defect in the bone that may communicate with the intracranial cavity.

Hidrocystomas (sudoriferous cysts) arise from the sweat ducts and are filled with a watery material.

Hemangioma(Figure 4-11)

The most common congenital vascular tumor of the eyelids is the capillary hemangioma (strawberry nevus) composed of proliferating capillaries and endothelial cells. They arise at or shortly after birth, often grow rapidly, and usually involute spontaneously by age 7 years. If superficial, they may be bright red (strawberry nevus); deeper lesions may be bluish or violet. Secondary anisometropia, refractive amblyopia, and strabismus are common and must be appropriately treated. Treatment of the tumor is rarely indicated unless it blocks the pupil. If it does, intralesional injection of steroids may produce rapid resolution; if this fails, partial surgical excision is indicated.

Capillary hemangiomas should be differentiated from the much rarer nevus flammeus (port wine stain), which is more purple in hue than the bright red of capillary hemangiomas. The nevus flammeus is composed of dilated, cavernous vascular channels. It is always present at birth, does not grow or regress as does a capillary hemangioma, and is often associated with Sturge-Weber syndrome. The cosmetic defect can be treated with laser surgery.

A third type of angioma is the cavernous hemangioma, composed of large, endothelium-lined vascular channels with smooth muscle in their walls. They are developmental rather than congenital and tend to arise after the first decade. Unlike capillary hemangiomas, they do not usually regress.

PRIMARY MALIGNANT TUMORS OF THE LIDS

Carcinoma (Figures 4-12 and 4-13)

Basal cell and squamous cell carcinomas of the lids are the most common malignant ocular tumors. These tumors occur most frequently in fair-complexioned individuals who have had chronic exposure to the sun. Ninety-five percent of lid carcinomas are of the basal cell type. The remaining 5% consist of squamous cell carcinomas, meibomian gland carcinomas, and other rare tumors such as Merkel cell carcinomas and carcinomas of the sweat glands.

Treatment of all these carcinomas is by complete excision, which is best achieved by controlling the surgical margins with frozen sections. Many of these malignant tumors and many benign ones as well can have the same appearance; biopsy is usually required to establish the correct diagnosis.

A. Basal Cell Carcinoma:

Basal cell carcinoma usually grows slowly and painlessly as a nodule that may or may not become ulcerated. It slowly invades adjacent tissues but rarely metastasizes. A less common type-sclerosing or morphea basal cell carcinoma-tends to extend insidiously and surreptitiously beneath the surface, sometimes producing ectropion, entropion, lid notching or retraction, dimpling of the overlying skin, or loss of eyelashes.

Frozen section study of the surgical margins is particularly important for sclerosing basal cell carcinomas, since the tumor margins are seldom clinically apparent. Microscopically controlled excision (a modified Mohs technique) is used by some dermatologists to achieve complete excision. Selected cases may be treated by other methods such as radiotherapy or cryotherapy with liquid nitrogen.

B. Squamous Cell Carcinoma:

Squamous cell carcinomas also grow slowly and painlessly, often starting as a hyperkeratotic nodule that may become ulcerated. Benign inflammatory tumors such as keratoacanthomas may closely resemble carcinomas. The correct diagnosis may depend on biopsy. Like basal cell carcinomas, these tumors can invade and erode through adjacent tissue; they can also spread to regional lymph nodes via the lymphatic system.

C. Sebaceous Gland Carcinoma:

Sebaceous gland carcinomas most often arise from the meibomian glands and the glands of Zeis but can also occur in the sebaceous glands of the eyebrow or caruncle. About half resemble benign inflammatory lesions and disorders such as chalazia and chronic blepharitis. They are more aggressive than squamous cell carcinomas, often extending into the orbit, invading lymphatics, and metastasizing.

Carcinoma Associated With Xeroderma Pigmentosum

This rare disease is characterized by the appearance of a large number of freckles in sun-exposed areas of the skin. These are followed by telangiectases, atrophic patches, and eventually a warty growth that may undergo carcinomatous degeneration. The eyelids are frequently affected and may be the first area to show degenerative changes, causing atrophy and ectropion with secondary inflammatory changes of the conjunctiva, symblepharon, corneal ulceration, and carcinoma of the lids. Malignant tumors include basal cell carcinomas, squamous cell carcinomas, and malignant melanomas. This condition is inherited as an autosomal recessive trait and is due to a defect in the repair of DNA damaged by ultraviolet light. Carriers can often be identified by excessive freckling.

The disease appears early in life and in most cases is fatal by adolescence as a result of metastasis. Life may be prolonged by carefully protecting the skin from actinic rays and treating carcinomatous tumors as rapidly as they appear.

Sarcoma

Soft tissue sarcomas of the lids are rare and usually are anterior extensions of orbital tumors. Rhabdomyosarcomas involving the lids and orbit are the most common primary malignant tumors found in these tissues in the first decade of life. The lid tumor may be the first sign. A combination of radiotherapy and chemotherapy is usually effective in preserving ocular function and preventing death.

Malignant Melanoma

Malignant melanomas of the eyelids are similar to those elsewhere in the skin and include three distinct varieties: superficial spreading melanoma, lentigo maligna melanoma, and nodular melanoma. Not all malignant melanomas are pigmented. Most pigmented lesions on the eyelid skin are not melanomas. Therefore, biopsy should be used to establish the diagnosis. The prognosis for melanomas of the skin depends upon the depth of invasion or the thickness of the lesion. Tumors less than 0.76 mm in thickness rarely metastasize.

METASTATIC TUMORS

Metastatic tumors to eyelids are rare. Because they frequently mimic benign lesions such as chalazions, cysts, and granulomas, the diagnosis is often not suspected. Not infrequently, the lid lesion appears before the primary tumor is discovered.

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