American Academy of Ophthalmology Web Site: www.aao.org
Will It Go or Will It Grow Now?
George Sorbit* was a pleasant fellow who owned a furniture store—and had a red and extremely painful eye. Mr. Sorbit had been experiencing photophobia and pain in his left eye for four days and redness for one day. He was in a significant amount of pain, and described it as a “7” on a scale of 1 to 10. At age 68, Mr. Sorbit had no history of ocular problems, and his eye examination two years previously was normal. He had seen an ophthalmologist when his symptoms began, and was prescribed erythromycin drops and referred to the University of Iowa for further evaluation.
We Get a Look
Mr. Sorbit’s past medical history was significant for rheumatoid arthritis, osteoarthritis and hypertension. While he neither smoked nor drank, he had acid reflux. He also had uncomplicated surgery on his foot three weeks before we saw him.
In addition to the erythromycin, his medications consisted of low-dose prednisone, Zestoretic (lisinopril/hydrochlorothiazide), iron, calcium, a multivitamin, aspirin and Tylenol #3. He reported that he was allergic to sulfa. His family history was significant for cataracts, arthritis and diabetes mellitus.
On examination without correction, the visual acuity in his right eye was 20/25; in his left eye, it was 20/40 without improvement with pinhole. He had a 0.3 log unit relative afferent pupillary defect in his left eye. His ocular motility was normal. IOP was 21 mmHg in the right eye and 16 mmHg in the left.
Our slit-lamp exam of the right eye revealed quiet anterior structures; in the left, it revealed mild eyelid erythema, 2+ conjunctival injection most prominent in the inferior temporal quadrant, a clear cornea, a clear lens and a shallow anterior chamber with 1+ cell. On gonioscopy, his trabecular meshwork was easily visible without mass.
Our dilated fundus exam of his right eye revealed a cup/disc ratio of 0.2, macular retinal pigment changes and drusen with no subretinal fluid or subretinal hemorrhage and a normal peripheral retina. In his left eye, we observed 2+ vitreous cells, a cup/disc ratio of 0.2 and, most remarkable, an amelanotic temporal mass with either choroidal thickening or extension of the mass nasally and inferiorly for a maximum diameter of 20 millimeters. He had an inferior retinal detachment associated with this lesion (Fig. 1).
At this point, we knew that Mr. Sorbit harbored drusen in his right eye and a large choroidal amelanotic mass in his left. Our differential diagnosis consisted of posterior scleritis (secondary to the rheumatoid arthritis), infection, degenerative changes (eccentric disciform) and neoplasia (ocular melanoma vs. metastasis).
Echography of the left orbit revealed a 4.5-mm elevated, low-medium reflective, moderately irregular, 1-2+ vascular lesion at 3 o’clock near the equator (Fig. 2). The choroid was thick with low reflectivity superior and anterior to the lesion. A low reflective infiltration of Tenon’s was noted behind the mass. As noted on our indirect exam, an inferior serous retinal detachment was evident on echography.
It appeared as though an inflammatory process was occurring adjacent to a mass lesion. We considered the options of observation, a steroid trial, antibiotics, biopsy, brachytherapy and enucleation.
We performed a complete physical examination and ordered a chest X-ray, a tuberculin skin test, syphilis serologies, liver function tests and a complete blood count. We also prescribed oxycodone (Percocet and others) and oral prednisone (80 milligrams daily) and recommended follow-up in one week. The high-dose prednisone was initiated to quiet the scleritis adjacent to the mass, to allow us to assess the true size and character of a potential tumor.
One Week Out
Mr. Sorbit reported his pain had resolved within 24 hours of initiating both prednisone and oxycodone. A repeat slit-lamp exam of the left eye revealed mild inferior temporal conjunctival injection. The fundus exam was significant for a persistent amelanotic lesion, which was less elevated and had less subretinal fluid and less contiguous choroidal thickening than had been observed the previous week.
A repeat echo showed a smaller (3.5-mm), elevated, solid, low-reflective vascular lesion with much less scleral and episcleral infiltration. The maximal diameter of the mass had decreased from 20 mm to 16 mm.
The complete physical examination revealed no evidence for metastatic neoplastic disease, infection or active rheumatoid arthritis. The tuberculosis skin test, liver function tests and syphilis serologies were normal. His CBC revealed only a long-standing anemia.
Though it was clear that the inflammation had decreased following his course of immunosuppression, his mass lesion persisted and did not decrease to an extent expected if the process were purely inflammatory. Thus, the odds were that the lesion was most likely a malignant neoplasm.
The decrease in size of the lesion in the context of neoplasia led us to conclude that the process was most likely a choroidal malignant melanoma with partial spontaneous involution. The management plan at this point consisted of continuing the prednisone and following up in one week.
Two Weeks Out
Mr. Sorbit returned with no complaints of pain. His visual acuity was stable and slit-lamp exam was quiet. The fundus exam revealed a persistent amelanotic lesion stable to slightly smaller with less subretinal fluid. A repeat echo showed a somewhat smaller (3-mm), elevated, solid, low-reflective vascular lesion with minimal episcleral infiltration. The greatest diameter, which had dropped from 20 mm to 16 mm in the first week, was now 14 mm.
We had several management options:
After weighing risks and benefits, Mr. Sorbit elected to proceed with continued, tapered prednisone over the next four weeks, followed by brachytherapy.
Six Weeks Out
After four more weeks of prednisone (for a total of six weeks), the lesion had further decreased in size to 2.9 mm (Fig. 3). The scleral injection had resolved and Mr. Sorbit reported no pain.
Brachytherapy was performed at this time. After detaching the left lateral rectus muscle, a 20-mm diameter iodine 125 plaque was placed on the postero-lateral sclera using indirect ophthalmoscopy, echography and transillumination to assess the margins of the tumor. This allowed for a 3-mm annulus of irradiated tissue surrounding the mass. The plaque remained in place for five days and was removed without difficulty. We anticipate that this tumor will begin to regress one to two months postoperatively and will continue to regress over several years.
An Abnormal Presentation
There are 4.9 to 7.6 cases per million of ocular melanoma per year. Risk factors include light skin, older age, presence of uveal nevi, exposure to ultraviolet light and genetics. Most commonly, there are no presenting signs and symptoms, and the abnormality is picked up on a routine examination.
Melanoma may present with prominent episcleral or scleral vessels. There may be vision loss secondary to a ciliary body tumor, or disruption of the retina by a choroidal tumor. There may be visual field defects, floaters, photopsias or metamorphopsias secondary to retinal detachments. Redness and pain is extremely rare as a presentation of ocular melanoma unless the tumor is very large with secondarily increased IOP, or a retinal detachment is extensive enough to cause angle-closure glaucoma.
Because Mr. Sorbit presented with an acute onset of redness and pain, we think that his ocular tumor had undergone either rapid growth or change secondary to inflammation or necrosis. Proposed mechanisms include immune responses to antigenic tumor cells, or inflammation secondary to necrosis following a rapid growth of tumor that outstripped the blood supply. Other ways that tumors can rapidly present themselves include hemorrhage into the tumor by a rapidly growing mass, hemorrhage into the subretinal space by a subretinal mass (mimicking a macroaneurysm or eccentric disciform) or hemorrhage into the vitreous.
This case raises an interesting question: Could certain melanomas, without treatment, totally involute? According to the literature, this is possible. The largest case series consisted of four cases of clinically diagnosed malignant melanoma that, by all clinical measures, spontaneously and completely involuted. There were no pathology specimens, however, to confirm the histologic composition of the masses. Unfortunately, two of these four cases have subsequently recurred and required treatment.
Mr. Sorbit’s unique presentation illustrates that ocular melanoma may occasionally present as ocular pain and inflammation. Partial to total involution of uveal melanoma is rare, but possible. Half of these eyes will eventually require treatment.
* Patient name is fictitious.
Ms. Kutzbach is a medical student and Dr. Boldt is professor of ophthalmology; both are at the University of Iowa.