Concurrent lacrimal gland melanocytoma and ocular melanocytosis in a dog

A 9-y-5-mo-old, spayed female, mixed-breed dog with buphthalmia and elevated intraocular pressure in the left eye, consistent with glaucoma, was evaluated. Black-pigmented, slightly elevated tissue with irregular margins was noted on the dorsolateral aspect of the left globe. Ultrasonography detected a mass, later identified as lacrimal gland, adjacent to the globe and the thickened uvea. The surgically removed lacrimal gland was effaced by dense sheets of melanin-laden cells. Within the enucleated globe, numerous melanin-laden cells infiltrated and expanded the rostral two-thirds thickness of the cornea, the entire anterior uvea (iris and ciliary body), and a rostral portion of the choroid. Melanin-laden cells in the left lacrimal gland and globe showed no nuclear atypia or mitotic figures, and reacted to anti-S100 and anti–melan A antibodies by immunohistochemistry. Our final diagnosis was concurrent lacrimal gland melanocytoma and ocular melanocytosis. The trabecular meshwork of the eye was obliterated by melanin-laden cells, which was the likely cause of glaucoma in this patient. To our knowledge, melanocytoma affecting the lacrimal gland has not been reported previously in a non-human mammalian species. Veterinary clinicians are encouraged to include melanocytoma in the differential list when examining an enlarged lacrimal gland.

Intra-Scleral Blue Nevus Arising within Oculodermal Melanocytosis: Melanoma or Not?

We report a 61-year-old female who presented to our service with recent growth of a thickened, pigmented, sub-conjunctival lesion within an area of ocular melanocytosis in her left eye. Lamellar sclerectomy was performed. Histopathological assessment revealed a localized melanocytic proliferation with features of blue nevus arising within the area of ocular melanocytosis. There was a small zone of cells showing cytological atypia and expansion of the scleral stroma. We believe this case to be the first report of intrascleral blue nevus arising within an area of ocular melanocytosis. Given the cytological atypia, the blue nevus may perhaps represent an intermediate stage within a progression from ocular melanocytosis towards melanoma. Patients with oculodermal melanocytosis merit regular long-term surveillance for early detection of melanoma, not only in the uvea, but also in the orbit and potentially the ocular surface.

Congenital choroidal melanocytoma in a child with ocular melanocytosis

A 1-day-old child was brought to the clinic for evaluation of enlarged right eye (OD). On examination, OD showed buphthalmos with diffuse scleral melanocytosis, fleshy blackish-brown extrascleral mass with corneal extension, and secondary glaucoma. Anterior segment evaluation revealed darkly pigmented iris and fundus evaluation OD revealed a darkly pigmented choroidal lesion. The left eye was within normal limits. A clinical diagnosis of choroidal melanocytoma with ocular melanocytosis was made. Enucleation OD followed by orbital implant was performed. Histopathology showed features of diffuse ocular melanocytosis involving limbus, iris, ciliary body, choroid, sclera, optic nerve head, optic nerve sheath, along with choroidal melanocytoma with extrascleral tumour extension. We presume that choroidal melanocytoma may have arisen from ocular melanocytosis.

Primary Episcleral Melanoma Consistent with Uveal Melanoma Mutations Treated by Excision and Gamma Knife Stereotactic Radiosurgery

Ocular melanocytosis has traditionally been associated with increased risk of developing uveal melanoma; however, rarely primary episcleral melanoma has been reported in the literature. Herein, we present the third case of primary episcleral melanoma treated by complete excision and cryotherapy. In contrast to previous cases, we obtained molecular genetic testing which revealed a GNA-11 mutation, and gene expression profiling resulted in a Class 2 PRAME positive tumor diagnosis. These two tests which have never been performed on previous cases, support lineage similar to uveal melanoma. In addition, we are the first case to report treatment of the surgical bed and surrounding orbital tissue with Gamma Knife stereotactic radiotherapy to both treat residual tissue and decrease risk of recurrence.

Bilateral Iris Mammillations in Amblyopic Eyes without Oculodermal Melanocytosis or Neurofibromatosis

Purpose. Iris mammillations are related to oculodermal melanosis and iris nevi. We report a rare case of bilateral simple iris mammillations without ocular melanosis or systemic neuronal disorders. Case Report. A healthy 10-year-old Japanese girl was found incidentally to have bilateral iris mammillations while being treated for amblyopia. The best-corrected visual acuity was 20/40 in both eyes. Ocular examination showed evenly spaced, uniform-size, iris protrusions completely covering the iris surface bilaterally. There were no other ocular or neurological abnormalities. Conclusion. To the best of our knowledge, this is the first report of bilateral iris mammillations in Japan. Our case emphasizes that iris mammillations can occur even without ocular melanocytosis or systemic diseases.