Choroidal melanoma with pigment dispersion in vitreous and melanomalytic glaucoma

A 39-year-old black man underwent enucleation of the left eye because of poor vision, ocular pain, and intractable glaucoma secondary to a choroidal tumor. Two diagnostic vitrectomies, performed 11 and 7 months before enucleation, had failed to disclose the proper diagnosis. Histologic diagnosis was necrotic malignant melanoma of the choroid with melanocytoma cells, extensive pigment dispersion throughout the eye, and melanomalytic glaucoma. Possible mechanisms of tumor necrosis are reviewed.     

Uveal melanoma presenting as acute angle-closure glaucoma: report of two cases

PURPOSE: To present two cases of uveal melanoma presenting as acute angle-closure glaucoma. DESIGN: Observational case reports.Assessment and management of two patients with uveal melanoma presenting with acute angle-closure glaucoma. RESULTS: One patient was initially treated with antiglaucoma medications, and a laser peripheral iridotomy was performed. The second patient had opaque media preventing visualization of the posterior segment, but the presence of an occult mass was suspected as the cause of angle closure. In both cases, B-scan ultrasonography revealed the presence of a tumor causing angle-closure glaucoma. The eyes were enucleated, and the diagnosis of uveal malignant melanoma was confirmed by histopathology. CONCLUSION: Although acute angle-closure as the initial presentation of uveal melanoma is rare, the presence of a uveal melanoma should be suspected in eyes unresponsive to medical therapy, especially in unilateral cases in which the view of the posterior segment is obscured.     

Nd-YAG laser iridotomy in pigment dispersion syndrome and pigment dispersion glaucoma

Pigment dispersion syndrome is characterized by iris transillumination defects, Krukenberg spindels, and dense trabecular pigmentation. Additional features are bilaterality, myopia, concavity of the peripheral iris, a higher incidence in men than in woman,and young age of onset. A mechanism of reverse pupillary block causes iridozonular friction. Laser iridotomy is recommended as treatment to prevent further pigment dispersion and pigmentary glaucoma.     

Uveal melanoma

The paper present a clinic and histological study, including all cases of uveal malignant tumours, hospitalized in Ophthalmological Clinic of Craiova in the last 15 years. The follow-up period of cases were 4 years. The diagnosis was established by clinic and paraclinic examination (imaging methods-- MRI, CT, Echo) and histologic examination. The histologic examination has been done for all cases. There were investigated several parametres: tumour cytology, the level of pigmentation, intravascular embolus, sclera infiltration, vascular invasion, vascular pattern, tumoral necrosis, mitotic activity. The histologic aspects of tumoral progression were: the high level mitotic activity, high surface area of intratumoral necrosis, the infiltrative process to the vessels, sclera, and ciliary body. The arcuat and network vascular patterns were involved in the systemic metastasis and local invasion. The most predominant type of cellularity related with a severe evolution of cases were the epithelioid cells and the mixed type cells.     

Conjunctival mycosis masquerading as melanoma

PURPOSE: To report a patient with a melanotic lesion of the conjunctiva that mimicked melanoma but proved to be a fungal infection on biopsy. DESIGN: Interventional case report. METHODS: A 75-year-old white man was examined for a 3-month history of a deeply pigmented lesion of the conjunctiva at the limbus of the right eye that closely resembled conjunctival melanoma. The lesion was completely excised. RESULTS: Histologic examination revealed fungal elements without evidence of tumor. CONCLUSION: Although rare, fungi should be included in the differential diagnosis of pigmented lesions of the conjunctiva.     

Conjunctival mascaroma masquerading as melanoma

PURPOSE: To describe a conjunctival mass composed of keratin with mascara deposition that clinically simulated a melanoma. METHODS: A 50-year-old woman, who used mascara for 20 years, was referred for a conjunctival mass that was suspected to be melanoma. Excisional biopsy was performed, and the lesion was studied histopathologically. RESULTS: The lesion consisted of conjunctival epithelium with a keratin plaque that contained multiple dark particles that exhibited birefringence with polarized light. The final diagnosis was conjunctival hyperkeratosis containing foreign bodies compatible with mascara deposition. The patient continues to use mascara, and there is no recurrence after 12 months. CONCLUSIONS: Deposition of mascara can assume tumorous proportions and simulate a conjunctival melanoma.     

Uveal melanoma presenting as cataract and staphyloma

Blind eyes can harbor a choroidal melanoma. We report a case of uveal melanoma presenting as staphyloma and complicated cataract in a 45-year-old female. The left eye was blind for six months. She underwent comprehensive ocular examination but fundus examination was precluded due to total cataract. The ultrasound of the eye showed a large mass filling the superior, nasal and inferonasal vitreous cavity with high surface reflectivity and low to moderate internal reflectivity. Magnetic resonance imaging (MRI) confirmed the diagnosis of choroidal melanoma. The patient underwent extended enucleation and histopathology was consistent with uveal melanoma.     

Dysthyroid eye disease masquerading as glaucoma

Intraocular pressure (IOP) screening by optometrists is increasingly prevalent. Although a raised IOP usually signifies glaucoma there can be other causes, notably dysthyroid eye disease, which those screening should be aware of. The patients in the four cases presented were presumed to have glaucoma on the basis of raised IOPs, and three of them actually received medication. Because their optic nerves and visual fields showed no abnormalities this diagnosis was questioned and the cause shown to be dysthyroid eye disease. Thyroid eye disease causes raised IOP on upgaze due to a thickened inferior rectus identing the globe; however, in the primary position the IOP is normal. A misleading raised IOP can thus be found if tonometry is inadvertently done in upgaze, either because of poor patient positioning or Bell's phenomenon. Although some patients will have a past medical history of thyrotoxicosis, dysthyroid eye disease can occur in the absence of systemic or biochemical abnormalities. The important clinical signs are lid lag, lid retraction, raised IOP on upgaze and proptosis, the first three being the most common. Tonometry should thus always be done in the primary position and if raised IOPs are found without any of the other features of glaucoma, other causes particularly dysthyroid eye disease, should be considered.     

Pathophysiology of pigment dispersion syndrome and pigmentary glaucoma

Pigmentary glaucoma results from zonular-pigment dispersion, primarily in young, myopic, white individuals. The concavity of the midperipheral iris allows iridozonular contact. Released pigment is carried to the trabecular meshwork where it resides: (1) benignly, not affecting the intraocular pressure, as in pigment dispersion syndrome; or (2) malignantly, elevating the intraocular pressure, as in pigmentary glaucoma. Small amounts of pigment are quickly phagocytized. If the particulate load is heavy, the cells migrate further along the outflow pathway. The flattening of the iris in pigmentary glaucoma patients receiving iridotomies, along with the backward flow of pigment observed during treatment, demonstrates a greater pressure in the anterior than the posterior chamber. This reverse pupillary block may be due to temporary ocular deformations caused by blinking, as small aqueous aliquots are forced into the anterior chamber. Flattening of the concave iris is the key to current and future management of these patients.     

Histologic findings in pigment dispersion syndrome and pigmentary glaucoma

PURPOSE: To investigate the morphologic changes in the trabecular meshwork in a case series of eyes with pigment dispersion syndrome and pigmentary glaucoma, and surgical trabeculectomy specimens from eyes with pigmentary glaucoma. MATERIALS AND METHODS: Trabecular meshworks from 6 whole eyes from 3 donors and 7 trabeculectomy specimens were studied by light and electron microscopy. Axonal counts from the whole eyes were correlated with qualitative and quantitative data of meshwork changes. RESULTS: Changes in the meshwork varied around the circumference of the eyes, but in all 6 eyes in most regions of the circumference there were numerous pigment granules within trabecular cells; pigment was not found within intertrabecular or cribriform spaces. In some regions of the circumference there was trabecular cell loss, loss of intertrabecular spaces, fusion of lamellae, and an increase in extracellular material under the inner wall of the canal. Separation of the normal tendinous connection to the canal wall cells was noted in some regions of all eyes. This change could be associated with regions of pathologic separation of the inner wall from the cribriform region, associated with partial obliteration of the lumen of the canal with cells and cell processes. In eyes with pronounced axon loss, meshworks showed most pronounced loss of trabecular cells and increased extracellular material. Trabeculectomy specimens had similar changes and, in addition, showed damaged trabecular cells and collapse of intertrabecular spaces without fusion of lamellae, consistent with artifacts from manipulation during surgery. CONCLUSIONS: Loss of trabecular cells, fusion of trabecular lamellae with collapse of intertrabecular spaces, increase in extracellular material, and obliteration of the canal were found in various amounts around the circumference of eyes with pigment dispersion syndrome and elevated intraocular pressure, and pigmentary glaucoma. These probably all contribute to the development of increased intraocular pressure. Meshworks from trabeculectomy specimens showed these findings and also showed artifactual damage of cells and loss of intertrabecular spaces. This suggests that handling during surgery may cause single trabeculectomy specimens to give only an incomplete picture of the pathophysiology of pigmentary glaucoma.     

Subretinal pigment dispersion following transpupillary thermotherapy for choroidal melanoma

PURPOSE: To report the case of a patient who developed considerable subretinal pigment/debris dispersion following transpupillary thermotherapy (TTT). This type of tumour response is extremely rare with this relatively new therapeutic modality. METHODS: A 50-year-old man with a left juxtapapillary choroidal melanoma measuring 8 x 6 x 4.3 mm was treated with 810 nm diode laser TTT administered in two sessions. Spot size was 3 mm and the power setting was 450 mW. RESULTS: Four months after the first treatment session, a considerable amount of pigment/debris was seen to have dispersed in the subretinal space, accumulating mainly in the macular area. Over a 12-month follow-up, the tumour showed progressive shrinkage without any change in the amount or location of the shed pigment/debris. No new tumour formation, recurrence or systemic metastases were detected. CONCLUSION: Subretinal pigment/debris dispersion is an unusual complication after TTT and requires close follow-up. There has been no short-term compromise on the life or visual acuity of this patient.     

Prolapsed intraocular aspergilloma masquerading as malignant melanoma

We describe an aspergilloma that masqueraded as an intraocular malignant melanoma in an elderly male patient.     

Primary orbital melanoma masquerading as vascular anomalies

PURPOSE: To review two cases of primary orbital melanoma presenting like orbital vascular anomalies. METHODS: Retrospective review of clinical presentation, treatment, radiology and pathology for two patients under the care of the Orbital Clinic at Moorfields Eye Hospital. RESULTS: Both lesions presented with the appearance and behaviour of vascular anomalies. In one case, a spindle cell melanoma appeared to be a low flow vascular anomaly with a loculated secondary haemorrhage and, in the other case, a melanoma of soft parts was considered to be an arteriovenous malformation and responded partially to embolisation. CONCLUSION: Primary malignant melanoma may present as a secondary vascular lesion of the orbit and this very rare tumour should be considered in the differential diagnosis of any vascular anomaly.