PERSPECTIVE AND PATTERNS OF SPREAD
The eyelid of the eye consists of three layers: skin, glands, and conjunctiva.
PERSPECTIVE AND PATTERNS OF SPREAD
Benign eyelid tumors, such as papillomas, nevi, hemangiomas, and xanthomas, occur with great frequency. Carcinoma (epithelioma) of the eyelids is the most common malignant tumor in ophthalmology. Basal cell carcinomas (BCCs) in the eyelids outnumber squamous cell carcinomas (SCCs) 40 to 1. In the conjunctiva, SCC occurs 10 times more often than BCC. The general behavior of both of these tumors is similar to that of skin carcinomas of other sites. They show a predilection for the junction of skin and conjunctiva at the margin of the eyelid. BCC is often only locally invasive and does not metastasize. SCCs, like the cancers elsewhere, do metastasize, particularly when they occur or invade regional lymph nodes.
Pigmented tumors of the lids, and especially of the conjunctiva, are rare. They are often difficult to manage because the onset or degree of malignancy is not always apparent on clinical examination. In some cases, because of extensive involvement of the ocular surface, surgical excision is impractical. In these cases, controlled cryotherapy of the involved epithelial surfaces is effective in eradicating malignant cells with minimal destruction of ocular tissues. Pterygium is a common benign condition characterized by an elevated fleshy tissue medial or lateral to the cornea. It has a neovascularization that can spread to the cornea.
There are numerous causes of eyelid swellings and inflammations of the conjunctiva, which can be recognized as follows:
• Episcleritis is localized inflammation of the conjunctiva and is movable over the scleral surface.
• Conjunctivitis and conjunctival hemorrhages appear more suddenly and can be due to infection, allergy, or trauma.
• Sty is an infection of the eyelash in the eyelid (Fig. 56.1A).
• Chalazion tends to be a diffuse swelling in the eyelid margin due to blockage of a meibomian gland and tends to point inside of the lid (Fig. 56.1B).
• Xanthelasmas are lipid deposits that are well circumscribed and yellowish in color.
A special type of cancer involving the eyelids or caruncle is sebaceous carcinoma; it is one of the “great masqueraders” and can resemble chronic inflammation or a benign mass lesion, that is, the chalazion.
For most of the cancers of the eyelid, the patterns of spread are presented (Fig. 56.1C) and the SIMLAP table (Table 56.1A) indicates the advancement of local invasion patterns. The most ominous is posterior with invasion of conjunctiva along the palpebral fold, then access to eye globe and orbit. Unlike the eye globe, the region is rich in lymphatic and regional lymph nodes. Treatment escalates from local excision to enucleation and exenteration.
Cancers of the conjunctiva can mimic pterygium and invade the cornea medially but can spread more easily than eyelid cancers into the eye globe and orbit the patterns of spread are presented (Fig. 56.1D; Table 56.1B). Histopathologically, basal cell and squamous cell carcinomas predominate, but a variety of adenocarcinomas can occur.
Figure 56.1 | A. Sty. A painful, tender red infection in a gland at the margin of the eyelid. A cancer nodule has a similar appearance but is painless and whitish in appearance. B. Chalazion. A subacute nontender and usually painless nodule involving a meibomian gland. May become acutely inflamed but, unlike a sty, usually points inside the lid rather than on the lid margins. Patterns of spread. C. Eyelid. Primary cancers of eyelids are essentially skin cancers and are coded for progression: Tis, yellow; T1, green; T2, blue; T3, purple; T4, red. D. Conjunctiva. Primary cancers are coded for progression: Tis, yellow; T1, green; T2, blue; T3, purple; T4a, red; and T4b,c,d, black. The concept of visualizing patterns of spread to appreciate the surrounding anatomy is well demonstrated by the six-directional pattern (SIMLAP; Tables 56.1A,1B).
OVERVIEW OF HISTOGENESIS AND HISTOPATHOLOGY
Overview of Eyelid
The eyelids are essential to protect the eye from pollutions—dust and debris—with skin on the outer surface and conjunctiva the inner surface. Between these two epithelial layers is a variety of glandular adnexa—sebaceous glands (Zeis), apocrine glands (Moll), and eccrine glands (meibomian)—each of which can transform into adenocarcinomas. Accessory lacrimal gland (Krause) and lacrimal gland form an inferior border in the upper eyelid. Eyelashes are hair follicles and are of importance in cosmesis. Table 56.2A lists the various cancers. The eyelid of the eye (Fig. 56.2A) consists of three layers: (i) skin with eyelashes; (ii) meibomian glands, sebaceous glands of Zeis, and apocrine glands of Moll; and (iii) tarsal muscle and conjunctiva cover. Tears consist of three elements: (i) The lacrimal gland secretions are watery, and the glands of the tarsal plate provide (ii) a waxy sebaceous secretion (meibomian) and (iii) an oily film (Zeis and Moll).
The conjunctiva consists of stratified columnar epithelium containing numerous goblet cells whose secretion bathes the eye. Tears drain from the eye through lacrimal puncta canaliculi located at a medial angle into a lacrimal sac lined by pseudostratified epithelium, as is the nasolacrimal duct. The conjunctiva has another list of histopathologic types (Table 56.2B; Fig. 56.2B). Note that conjunctiva can occur although this is the least common site.
Embryogenesis is an indication of histogenesis. The eyelid and conjunctiva are ectodermal structures giving rise to a variety of carcinomas.
Figure 56.2 | Eyelid histopath overview. A. Basal cell carcinoma, (eyelid). Buds of atypical basaloid keratinocytes extend from the overlying epidermis into the papillary dermis. The peripheral keratinocytes mimic the stratum basalis by palisading. The separation artifact (arrow) is present because of poorly formed basement membrane components and the hyaluronic acid–rich stroma that contains collagenase. B. Squamous cell carcinoma (conjunctiva).A microscopic view of the periphery of the lesion shows squamous cell carcinoma in situ. The entire epidermis is replaced by atypical keratinocytes. Mitoses and multinucleation of keratinocytes are apparent, as is apoptosis (straight arrows).
EYELID BASAL CELL SQUAMOUS CELL CANCER
SUMMARY OF CHANGES SEVENTH EDITION AJCC
Carcinoma of the Eyelid
• A section on Lymph Node Staging was added (Fig. 56.3A).
• T3 was redefined, and the lesions have been divided into T3a and T3b.
• T4 has been redefined.
• N0 was redefined and divided into cN0 (no regional lymph node metastasis, based upon clinical evaluation or imaging) and pN0 (no regional lymph node metastasis, based upon lymph node biopsy).
• Stage groupings have been defined and added.
EYELID
Figure 56.3 | A. Eyelid staging figure. TNM staging criteria are color coded bars for T advancement: Tis, yellow; T1, green; T2, blue; T3, purple; T4, red.
TNM STAGING CRITERIA
TNM STAGING CRITERIA
Because of the fineness and thin character of the layers of the eyelid, conjunctiva size is the key criterion for localized stages, measured in millimeters (Fig. 56.3B).
• Skin cancer of the eyelid: T1, 5 mm; T2, >5 to 10 mm; T3, >10 mm.
• Conjunctival carcinoma: T1, >5 mm; T2, >5 mm.
CONJUNCTIVA CANCER
Figure 56.3 | B. Conjunctival staging figure. TNM staging criteria are color coded bars for T advancement: Tis, yellow; T1, green; T2, blue; T3, purple; T4, red.
SUMMARY OF CHANGES SEVENTH EDITION AJCC
Carcinoma of Conjunctiva
• A listing of site-specific categories is included in T3.
• Sebaceous gland carcinoma with pagetoid conjunctival spread was added under histopathologic type.
The TNM staging matrix allows for identification of stage group once T and N stages are determined (Table 56.3).
SUMMARY OF CHANGES SEVENTH EDITION AJCC
Malignant Melanoma of the Conjunctiva
• Definitions of T classification have changed to describe location (bulbar, noncaruncular, caruncular) (Fig. 56.3C).
• Definitions of N category have changed to describe whether a biopsy was performed.
• Definitions of pT status have changed to describe local invasion and tumor thickness.
• Definition of T (is) or melanoma in situ when tumor is limited to the epithelium.
• Definitions of “Histologic Grade” were changed to describe cases of synchronous PAM with atypia and conjunctival melanoma (G3 and G4).
CONJUNCTIVA MELANOMA
Figure 56.3 | C. Conjunctival melanoma staging figure. TNM staging criteria are color coded bars for T advancement: Tis, yellow; T1, green; T2, blue; T3, purple; T4, red.
TNM STAGING
T-ONCOANATOMY
The eyelid is the anterior protective layer of skin and mucosa and can rapidly close when needed to avoid harm to the globe. The intricate anatomy is rarely appreciated until tumefaction occurs and benign conditions need to be distinguished from cancers. The three-planar views are most revealing if the oncoanatomy is related to specific tumors (Fig. 56.4).
• Coronal (Fig. 56.4A): The important features to note are at the inner canthus of the eye, where two fine lacrimal puncta and canaliculi drain into a hidden sac, which drains into the lacrimal duct and into the inferior meatus of the nose.
• Sagittal (Fig. 56.4B): The true complexity and the rich variety of tissues are noted as to lesion formation. The infected eyelash can give rise to a sty or hordeolum (a plugged tarsal gland to a chalazion), not to be confused with the variety of cancers that occur as adenocarcinomas of tarsal glands of Zeis or ciliary glands at the edge of the lids and squamous cell cancer of the conjunctiva. The magnified sagittal view provides the intricate organization of layers. Note the insertion of the levator palpebrae muscle in the upper lid. The conjunctiva covers the globe and lid, meeting at the fornix.
Figure 56.4 | Three-planar T-oncoanatomy. A. Coronal. B. Sagittal.
N-ONCOANATOMY AND M-ONCOANATOMY
N-ONCOANATOMY
The eyelids and conjunctiva drain predominantly into the preauricular nodes except for its medial margin, which follows medial lymphatics into the submandibular nodes (Figs. 56.5A, 56.5B; Table 56.4).
M-ONCOANATOMY
The pterygoid plexus of veins drains the fine veins of the eyelids into the internal jugular vein (Fig. 56.5C). Pulmonary metastases are the most common site of dissemination.
Figure 56.5 | A,B. N-oncoanatomy. Lateral view. The sentinel node is the preauricular node. C. M-oncoanatomy of the eye. Venous drainage of the eyelid. Retinal arteries and veins.
STAGING WORKUP
RULES FOR CLASSIFICATION AND STAGING
Clinical Staging
Clinical staging begins with histopathologic identification of cancer type and grade and then involves careful inspection, palpation, and slit-lamp biomicroscopy. The entire conjunctival surface needs viewing with upper-lid eversion. If the cancer is deeply invading T3, T4, imaging procedures are highly recommended to determine anatomic extent. Conjunctival melanoma needs to be distinguished from acquired melanosis, junctional and compound nevi, and melanoma in situ (Table 56.5; Fig. 55.6).
Pathologic Staging
Pathologic staging is appropriate for total excision of cancers and exenterations. Deeply invading malignancies require notation of margins of conjunctiva and, if globe is included, optic nerves. Depth of lesions suspected as melanomas need to be perpendicular to the skin. Sentinel node biopsy is encouraged using 99m-technicium.
For lacrimal gland cancer resections, the complete specimen should be studied for margins. Perineural and sentinel preauricular/parotid node evaluation needs to be recorded if positive.
PROGNOSIS AND CANCER SURVIVAL
PROGNOSTIC FACTORS
See Table 56.6.
CANCER STATISTICS AND SURVIVAL
The eye and orbit only account for 2,750 new diagnoses, excluding carcinomas of the eyelids. Deaths attributed to ocular malignancy are less than 10% of the entire group (240). Some of the most elegant proton and three-dimensional conformal radiation stereotactic techniques allow for the cure of choroidal melanomas and retinoblastoma with preservation of vision.
Survival remains impressive; 90% survive long term. Most skin cancers about the eyelids are detected early and are readily controlled by excision and/or radiation. Only recurrent cancers that deeply invade can lead to deformity, loss of eye, and death.
Figure 56.6 | Note the relationship of the eyelid to the eye globe and skull base. Correlates with Fig. 56.4B Sagittal View T-oncoanatomy. 1. Orbicularis oculi, 2. Eyeball, 3. Inferior Oblique Muscle, 4. Extracoronal Fat, 5. Interior Rectus Muscle, 6. Dural and Arachnoid Sheath, 7. Retrobulbar Fat, 8. Superior Rectus Muscle, M. Maxillary Sinus.