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Woman experiences foreign body sensation, like 'double eyelids'

Issue: July 25, 2012

ByJay S. Duker, MD

ByMitesh K. Kapadia, MD, PhD

ByHelen K. Wu, MD

ByCatherine A. Cox, MD

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A 94-year-old woman presented to the New England Eye Center complaining of irritation in her left eye. She noted that this irritation was accompanied by a foreign body sensation when blinking, which she described as feeling like “double eyelids.” She also noted the appearance of a left upper lid lump within the past month. She denied any pain or decrease in vision in the left eye.

The patient’s ocular history was significant for a previous left upper and lower lid lesion excision, as well as bilateral cataract extraction. Her medical history included a left upper lip lesion excision but otherwise was noncontributory. Outpatient medications included hydrochlorothiazide for systemic hypertension. Review of systems was negative. She denied any tobacco use and reported moderate sun exposure.

On examination, the patient’s uncorrected visual acuity was 20/200 in the right eye and 20/70 in the left eye, with pinhole improvement to 20/50 in both eyes. Pupillary examination, extraocular movements and IOP were within normal limits. External examination of the left eye revealed 3+ injection of the temporal bulbar conjunctiva with large vessels feeding into a small, darkly pigmented conjunctival lesion at 1 o’clock. An additional small, pigmented conjunctival lesion was present, which was seen on adduction. Madarosis was present along the lateral aspect of the upper and lower left eyelids due to prior surgical resection (Figure 1). With eversion of the left upper eyelid, a darkly pigmented, 5-mm thick nodular lesion was seen on the palpebral conjunctiva (Figure 2).

 

 

Figure 1. Photograph, left eye. Two pigmented lesions were present with significant injection of the surrounding temporal conjunctiva. Madarosis of the upper and lower lids was also present due to prior surgical resection.

Images: Cox CA, Wu HK, Duker JS and Kapadia MK

 

Figure 2. Photograph, left eye. A darkly pigmented, nodular lesion was seen with eversion of the upper lid.

What is your diagnosis?

Palpebral conjunctival lesion

The differential diagnosis for a pigmented bulbar and/or palpebral conjunctival lesion includes a variety of etiologies.

Common pigmented lesions found on the conjunctival surface include racial melanosis and primary acquired melanosis (PAM), both of which present as flat pigmented lesions and are benign, although PAM is considered a risk factor for melanoma. Racial melanosis can be found anywhere on the conjunctiva and at any age, whereas PAM more commonly presents as an interpalpebral lesion in midlife. Malignant melanoma has been reported to arise from either of these two lesions and should be considered if a lesion has grown in size or changed in appearance over time. A nevus can present as a pigmented lesion but typically presents in childhood, occurs at the nasal or temporal limbus, and has characteristic clear overlying cysts. Ocular melanocytosis is also a possibility, particularly if accompanied by pigmented periorbital tissue associated with a nevus of Ota.

Other less common etiologies caused by deposition of pigment include ochronosis, argyrosis and adrenochrome deposits. Ochronosis occurs in association with alkaptonuria and is caused by deposition of homogentisic acid. It can be found on the bulbar conjunctiva, the cornea and, rarely, the tarsal plate. Argyrosis is caused by exposure to silver, which has been reported in patients using silver eye drops or even in patients with frequent exposure to silver metal, such as jewelry industry workers. Adrenochrome deposits were previously seen in association with epinephrine eye drops but are rare because these eye drops are no longer commonly prescribed. Finally, one must always consider the possibility of a foreign body, such as bits of mascara mimicking the appearance of a pigmented lesion.

Follow-up

After the patient’s initial examination, additional history was obtained regarding her previous left eyelid lesion. Four years prior, she had undergone excision and reconstruction by an outside plastic surgeon of both her upper and lower left eyelids for an extensive pigmented lesion. The pathology report returned as melanoma in situ with a small positive margin of the left upper lid at the conjunctival margin. Wide excision vs. “watchful waiting” was offered, and the patient chose the latter. No recurrence was noted at a clinic visit 7 months later, and the patient never returned for additional follow-up.

We performed a diagnostic biopsy of the patient’s palpebral conjunctival lesion, which returned as positive for MART-1, a melanoma marker, and positive for Ki-67, a marker for increased cell proliferation (Figure 3). PCR was negative for the BRAF mutation. Our patient was sent to both the cornea and retina services for further examination, as well as to radiation and hematology/oncology. The cornea service recommended wide local excision of the bulbar conjunctival lesions with cryotherapy, and the retina service noted no extension of melanoma into the fundus. An MRI was obtained, which confirmed these findings (Figure 4). A PET scan showed no evidence of metastatic disease.

 

Figure 3. Pathology slide, biopsy of palpebral lesion. Prominent nucleoli and two mitotic figures per high power field were present, consistent with epithelioid-type melanoma. MART-1 and Ki-67 stains were also positive (not shown).

 

Figure 4. T2-weighted axial MRI, non-contrast. A left preseptal soft tissue lesion is present with no evidence of extension into postseptal tissues.

 

Based on these findings, the patient was offered either orbital exenteration or external beam radiation with lesion debulking. The patient and her family opted for the latter, more conservative treatment.

Discussion and treatment

Conjunctival melanoma is relatively uncommon: Only 40 new cases are reported on average per year, and it accounts for roughly 2% of ocular malignancies. The most frequent patient population affected is Caucasian elderly women. Conjunctival melanoma is known to arise from PAM in 53% to 74% of cases, de novo in 37% of cases, from nevi in 4% of cases and from racial melanosis in a handful of cases. On examination, a melanoma is often expected to be pigmented, but it is not uncommon for melanomas to also be amelanotic. Poor prognostic signs include location in the fornix or tarsal conjunctiva, as well as thickness greater than 1 mm to 2 mm.

Initial treatment should include a diagnostic biopsy. Histologically, melanoma may present as four different types: epithelioid, spindle A, spindle B, or a mixture of the other types. Epithelioid type carries the least favorable prognosis. Once melanoma has been diagnosed, MRI and PET scans can be used to determine disease extent and possible metastasis. Baseline labs are also useful to evaluate immune status and liver function. PCR testing is available for the BRAF mutation, which is known to stimulate the MEK/ERK pathway, thus favoring cell growth over differentiation. Testing for BRAF helps determine if treatment with vemurafenib, a BRAF inhibitor, is likely to be effective. Of note, BRAF mutations are more common in cutaneous melanoma; therefore, the likelihood of using vemurafenib for conjunctival melanoma is low.

Due to its high recurrence rates, conjunctival melanoma has been described as “one of the most dreaded and unpredictable ocular tumors.” When metastasis occurs, possible sites include the preauricular or submandibular lymph nodes, brain, lungs and liver. Sentinel node screening for micrometastatic disease has been reported by some groups, but the long-term benefit of this procedure has not yet been proven. Local recurrence following excision is estimated to be 50% to 70% after 10 years, and metastatic risk after 10 years is 25%. Survival rates are expected to be less than 6 months if distant metastasis is present on initial diagnosis.

Treatment options vary by location of the melanoma. Conjunctival lesions can be successfully removed using a “no-touch” technique by leaving 4-mm margins and applying double freeze-thaw cryotherapy. Topical mitomycin C and interferon alpha-2b have been used as adjuvants for residual PAM after melanoma excision; however, neither is effective as an initial treatment. When a lesion is located in the eyelid or orbit, either wide local excision with external beam radiation or orbital exenteration can be performed. Orbital exenteration has not been shown to improve survival rates over excision/radiation, but it can be used for palliation when orbital involvement is present.

Once conjunctival melanoma has been diagnosed and treated, long-term follow-up should include clinical monitoring every 2 to 3 years. Careful examination of the conjunctival surface including double eversion of the eyelid, palpation of regional lymph nodes and photographic documentation are important. An annual physical by the patient’s primary care physician is also suggested, which may include basics labs, repeat MRI and follow-up chest X-ray.