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Pigmented Corneal Mass in a 59-Year-Old Man With Cutaneous Melanoma

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 59-year-old man with history of cutaneous melanoma of the trunk was referred for evaluation of a pigmented angle lesion in the right eye. He denied any changes in vision, flashes, floaters, eye pain, or eye redness. He denied a history of trauma, intraocular surgery, or inflammation in the eyes. He had a history of stage 3B melanoma treated with ipilimumab complicated by grade 3 colitis refractory to high-dose corticosteroids and infliximab requiring colectomy and stomal hernia after hernia repair. Serial positron emission tomography/computed tomography scans showed no evidence of metabolically active disease. His medical history was notable for hypertension controlled with metoprolol. His mother had a history of breast cancer but otherwise family history was unremarkable.

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Peripheral pigmented placoid corneal endotheliopathy

C. Observation with close follow-up

We present a case of a 59-year-old man, with history of stage 3B melanoma of the trunk but no trauma, intraocular surgery, or intraocular inflammation, who had a flat, pigmented, well-demarcated corneal endothelial lesion with scalloped edges. On gonioscopy, the pigmented lesion was located in the cornea without extension past the Schwalbe line and did not involve other angle structures such as the trabecular meshwork or scleral spur (Figure, B).

The patient most likely has a benign condition called peripheral pigmented placoid corneal endotheliopathy (PPPCE) given its morphology and location in the cornea without involvement of other ocular structures, and the most appropriate next step would be observation with close follow-up (choice C). The differential diagnosis includes uveal melanoma, pigment dispersion syndrome, trauma, or inflammatory etiology. Given the medical history of metastatic melanoma, uveal melanoma should be considered. However, there was no extension of the lesion into the angle on gonioscopy and no masses were identified on UBM. There have been prior reports of corneal melanoma secondary to infiltration from the limbus or in the setting of prior ocular surgery or trauma.13 Management of corneal melanoma consists of surgical resection, with or without cryotherapy, biopsy, and postoperative topical chemotherapy.3 The patient denied a history of surgery or trauma and there were no masses visualized on examination or imaging. Therefore, corneal biopsy (choice A) or surgical excision (choice B) was not recommended as the next step as these would be invasive and inappropriate at this stage. Corneal endothelial pigmentation could be due to trauma or inflammatory causes. However, the patient denied a history of trauma, he was asymptomatic, and there was no evidence of intraocular inflammation on examination. Given this history and examination findings, prednisolone drops (choice D) would not be indicated. Pigment dispersion syndrome is unlikely given the appearance of the trabecular meshwork, normal intraocular pressure, and lack of symptoms associated with this disease.

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CME Disclosure Statement: Unless noted, all individuals in control of content reported no relevant financial relationships. If applicable, all relevant financial relationships have been mitigated.

Article Information

Corresponding Author: Miguel A. Materin, MD, Duke Eye Center, Department of Ophthalmology and Visual Sciences, Duke University, 2351 Erwin Rd, Durham, NC 27705 (miguel.materin@duke.edu).

Published Online: June 23, 2022. doi:10.1001/jamaophthalmol.2022.1537

Conflict of Interest Disclosures: Dr Materin is on the advisory board for Castle Biosciences and AstraZeneca and was a speaker for Carl Zeiss Meditec. No other disclosures were reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
1.
Jukić  T , Katusic  D , Kordić  R , Cacić  M , Braunschweig  T , Thumann  G .  Malignant melanoma of the cornea after blunt trauma.   Ophthalmologe. 2009;106(7):625-627. In German. doi:10.1007/s00347-008-1872-2PubMedGoogle Scholar
2.
Romaniuk  W , Koziol  H , Muskalski  K , Dorecka  M , Tarnawska  D , Sabat  D .  A unique case of primary corneal melanoma.   Jpn J Ophthalmol. 2002;46(1):114-116. doi:10.1016/S0021-5155(01)00462-2PubMedGoogle ScholarCrossref
3.
Panagiotou  DZ , Chranioti  AA , Tzorakoleftheraki  SE , Ziakas  NG , Oikonomidis  PK .  Primary melanoma of the cornea.   GMS Ophthalmol Cases. 2020;10:Doc12. doi:10.3205/oc000139PubMedGoogle Scholar
4.
Chen  LG , Finger  PT , Dhrami-Gavazi  E .  Peripheral pigmented placoid corneal endotheliopathy.   Cornea. 2013;32(11):1483-1487. doi:10.1097/ICO.0b013e3182a64833PubMedGoogle ScholarCrossref
5.
Deeley  M . Peripheral pigmented placoid corneal endotheliopathy: a rare finding in a Caucasian male and its distinguishing features. Accessed November 28, 2021. https://www.aaopt.org/detail/knowledge-base-article/peripheral-pigmented-placoid-corneal-endotheliopathy-a-rare-finding-in-a-caucasian-male-and-its-distinguishing-features-339195-3238447
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