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In ocular tumor cases, ophthalmologists need to know when to intervene, when to refer
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 Richard L. Lindstrom |
While life-threatening primary malignancies of the eye or adnexa are a rare diagnosis for the primary ophthalmologist, we all deal with “tumors” every day, and it is our responsibility to know when we might be dealing with something more serious.
A tumor can be defined as any abnormal new mass of tissue that serves no useful purpose. It is one of the classic signs of disease noted by ancient physicians, along with the heat associated with inflammation, redness and loss of function. Things as simple to diagnose and as common as pingueculae and pterygia are tumors, as are seborrheic and actinic keratoses.
As a clinician, I have found it helpful to think histopathologically as I do my differential diagnosis of ocular and periocular tumors. Every cell type can undergo benign enlargement to form a tumor through hypertrophy, an enlargement of cell size, or hyperplasia, an increase in the number of cells. In addition, infection as in a hordeolum or inflammation as in a chalazion can cause a tumor. Most of these tumors are benign, although they can interfere with normal function, and in many cases are undesirable cosmetically. Surgical excision or incision and drainage are often indicated to enhance function and/or cosmesis.
In today’s environment, where cost containment and malpractice risks collide every day in our decision making, it remains prudent and appropriate to send a specimen for pathologic evaluation in any case in which the diagnosis is at all in doubt. In particular, recurrent lesions deserve a higher index of suspicion and a pathology report.
While most ocular and adnexal tumors are benign, every cell type can undergo malignant transformation. Of most concern in the skin is basal cell carcinoma, which represents 90% of eyelid malignancies, the rarer but more invasive squamous cell carcinoma, sebaceous cell carcinoma and, last but not least, the most feared, malignant melanoma. On the surface of the eye, conjunctival and corneal intraepithelial neoplasia, usually originating near the limbus, is usually easily recognized. The more invasive squamous cell carcinoma usually has conjunctival and corneal intraepithelial neoplasia as a predecessor, and sebaceous cell carcinoma, while usually originating in the meibomian glands, can also present on the surface.
Once inside the eye, melanoma becomes a core concern, and the decision as to when and how to intervene in the face of a pigmented lesion on or in the eye is a great challenge. In addition, metastatic lesions to the eye can affect visual function and occasionally are the presenting sign of an occult malignancy, especially of the breast or lung.
Of concern is the fact that delayed diagnosis or improper initial surgical management of adnexal, ocular surface or intraocular malignancies can significantly affect treatment prognosis. For example, simply using a dry technique and avoiding irrigation in the excision of an ocular surface malignancy can significantly reduce the chance of tumor seeding and recurrence. The size of excisional biopsy clear margins required, the use of adjunct therapies such as cryotherapy, interferon, mitomycin C and the like, and the alternatives such as Mohs’ technique and radiation often require a knowledge and skill level difficult to obtain for most of us. For this reason, when faced with a potentially sight-threatening or life-threatening malignant tumor, timely referral to an experienced expert is usually prudent.