Anterior Segment Tumors

Learn how certain eye tumors may cause signs and symptoms similar to more common conditions.

BY JERRY A. SHIELDS, MD

FORTUNATELY, IT IS RARE to find that a patient’s diagnosis is associated with ocular cancer, but it does happen quite often in the practice of ocular oncology. I advise you to keep a high index of suspicion. Overlooking a life-threatening condition has both clinical and medicolegal significance, so be sure to read about eye tumors so you can play a role in diagnosing unsuspected cancers.

When something about a patient’s diagnosis doesn’t add up, it might require a closer look.

Astigmatism: If a patient has astigmatism and you can’t quite correct it, what should you do? In our ocular oncology practice, we dilate the pupil widely and look at the ciliary body, and we might see the tip of a malignant melanoma. Early-stage melanoma in the ciliary body tends to push on the lens equator and tilt it a little, producing a lenticular astigmatism.

Strabismus: When a child presents with esotropia, my colleagues and I try to take a good look at the pupil. There may be a white pupillary reflex because retinoblastomas that develop in the macular area tend to disrupt fixation and cause strabismus — either esotropia or exotropia.

Chalazia: It’s important to submit all tissue from chalazia because a number of cancers can simulate a chalazion, including sebaceous carcinoma, basal cell carcinoma, Merkel cell carcinoma and metastasis. You need the histopathology.

We frequently see what appears to be chalazion but is actually the first sign of metastasis from breast cancer, lung cancer or melanoma. Sebaceous carcinoma initially produces a chalazion-like eyelid nodule that is frequently misdiagnosed. Also, a sausage-shaped chalazion in the upper eyelid could be a serious Merkel cell carcinoma.

Blepharitis: Several cancers simulate blepharitis, including sebaceous carcinoma, morpheaform basal cell carcinoma, squamous cell carcinoma, lymphoma and others.

A patient was referred to us with chronic, severe blepharitis and was diagnosed with sebaceous carcinoma that had invaded the epidermis from the tarsus. Another patient’s blepharitis was actually a morpheaform basal cell carcinoma that produced a diffuse thickening of the lid.

Conjunctivitis: You manage typical conjunctivitis every week, but sebaceous carcinoma can produce conjunctivitis appearance because it can invade the conjunctiva and grow in a flat pagetoid pattern (Figure 1).

FIGURE 1. Conjunctivitis is a very common diagnosis, but in rare cases, the signs and symptoms are the result of the conjunctival epithelial involvement of sebaceous carcinoma as shown in this case.

Episcleritis: What looks like scleritis associated vessels can in fact be sentinel vessels due to ciliary body melanoma. Apparent episcleritis also may be a conjunctival metastasis from cancer that produces a diffuse lesion rather than a nodule.

We had a patient whose doctor had treated him with steroids for years for a vessel deemed a “cosmetic blemish.” He eventually was referred to us with vitreous hemorrhage. A melanoma filled half of his eye, so he underwent enucleation but eventually died of metastatic disease.

Pterygium: Squamous cell carcinoma and melanoma can simulate a pinguecula, or pterygium. Don’t just perform a biopsy — consider complete removal of any lesion that is atypical.

A good example is a young man whose conjunctival redness was treated with steroid drops for 2 years before it got darker and he was referred to us. By that point, he already had melanoma that had metastasized to his lymph nodes.

Conjunctival hemorrhage: Kaposi’s sarcoma is a malignant vascular tumor with red lesions that simulate a hemorrhage. It is almost exclusively found in AIDS patients, so we see it less frequently now than we did in the past.

Cataract: Cataract surgeons call us several times a year because something doesn’t look right in the ciliary body, and sure enough there’s an occult ciliary body melanoma pushing on the lens and causing the cataract. Sometimes it isn’t discovered until the surgeon finds it pressing on the intraocular lens after surgery. Methods of detecting the tumor before surgery include careful ophthalmoscopy, ultrasound and transillumination.

Iris tumors simulating benign conditions: We often are referred a patient with unilateral acquired hyperchromic heterochromia and glaucoma that has been misdiagnosed as pigmentary glaucoma. It proves to be a diffuse iris melanoma that presents as slight thickening of the iris but with no distinct mass. In addition, pediatric cases with iritis and neovascular glaucoma may be retinoblastoma. When we see a child of any age with hypopyon in a quiet eye, with no signs of inflammation, it may be the diffuse infiltrating type of retinoblastoma, a serious cancer in children in which a delay in diagnosis could be fatal. Iris metastasis is rare, but is often misdiagnosed as inflammation.

If this list of potential tumor diagnoses leaves you feeling scared, that could be a good thing. Read about eye tumors, be vigilant and make an early diagnosis of a potentially life-threatening disease.